Oval-shaped, raised, scaly, hyperkeratotic lesion on the entensor surface of the leg of a young man with psoriasis courtesy of Dawn M. Davis, MD, Mayo Clinic 64 ; nail pitting in a child with psoriasis. In the third and fourth fingernails, characteristic pitting is noted which may precede the development of arthritis courtesy of Dawn M. Davis, MD, Mayo Clinic 65 ; toenail changes in the child shown in Note the asymmetry courtesy of Dawn M.
Davis, MD, Mayo Clinic Note the erythema and swelling over the medial aspects of the first metatarsophalangeal joint and interphalangeal joint of the great toe in this year-old boy who was seronegative. Note the diffuse swelling of the second toe in this boy with severe nail changes from PsA courtesy of C. As with JAS, complications of medical therapies such as gastritis associated with NSAIDs, infections associated with immunosuppressive medications, and bone disease related to corticosteroids may occur.
The etiology of IBD-associated arthropathy is unknown. It is not generally associated with HLA-B27 unless there is axial involvement. Note the aphthous changes of the intestinal mucosa of the small intestine of this year-old adolescent referred for intermittent loose stools and infrequent, mild abdominal pain courtesy of W. Faubion, MD, Mayo Clinic Note the erythematous borders and large areas of mucosal ulceration of the small bowel in this year-old girl referred for diarrhea, abdominal pain, weight loss, and an abnormal small bowel radiograph courtesy ofW.
Cobblestoning and stricture formation is apparent in the small bowel of this year-old girl referred for diarrhea, abdominal pain, growth retardation with anemia, and hypoalbuminemia courtesy of W. One of these is erythema nodosa EN. It is usually painful, subcutaneous, and frequently on the extensor surfaces of the legs Another painful ulcerative lesion occasionally seen with IBD is pyoderma gangrenosum Oral mucositis, which is painful, may precede the GI manifestations of IBD; a severe case of mucositis associated with Crohn disease is shown Mucositis may be seen with other forms of JSpA.
The axial disease seen in adults is very uncommon in children Burgos-Vargas et al. These slightly raised, painful subcutaneous lesions may be seen in patients with IBD courtesy of Dawn M. This ulcerating lesion, usually on the lower extremities may be seen in patients with IBD courtesy of Dawn M. Mucositis in Crohn disease. Large left ankle effusion.
Synovial overgrowth and synovial fluid are palpable and warm in this 8-year-old boy with seronegative oligoarthritis, who had an antalgic gait Evidence of occult blood is frequently found in stool samples. Since most patients with IBD-associated arthropathy have peripheral joint involvement, they share imaging features with the other forms of JSpA. One of the challenges of managing these patients is that many are not candidates for NSAIDs because of their potential for adverse actions to the GI system.
Patients may rarely develop sequelae of uveitis, such as cataracts. Complications of medical therapies such as infections associated with immunosuppressive medications may occur. Bone disease related to corticosteroids may be enhanced by vitamin and mineral deficiencies related to enteritis. Poorly controlled IBD can increase the risk of bowel perforation, fistulae formation, and growth retardation. Faubion, MD, Mayo Clinic. The precise mechanisms of how the infectious agents in ReA initiate a remote, inflammatory response are not known, but depend on both host and pathogen characteristics.
Post-enteric ReA tends to occur in younger children and the post-UTI form is seen more in adolescents and young adults. The pattern of joint involvement lower extremity, oligoarthritis, asymmetric, and so on is similar to the other forms of JSpA. Enthesitis may also be present. The involved joints are very painful.
Findings of inflammatory eye disease, such as uveitis, may also be seen. The classic form of ReA is characterized by urethritis, conjunctivitis, and a painful oligoarthritis frequently following a lower UTI. Two characteristic skin findings may be seen in older children with ReA. In males, circinate balanitis may be seen in a few cases of ReA Keratoderma blenorrhagicum is a pustlar rash, on the soles of feet and may also be seen in an occasional patient with ReA Since the time between the exposure and the development of arthritis could be several weeks, or the exposure may have generated minimal symptoms, a very high degree of suspicion is needed to look for potential GI or GU pathogens that could be provocative.
Stool culture and urine polymerase chain reaction for evidence of previous chlamydia infection may be needed. The use of antibiotics beyond treating the initial, precipitating infection to treat the arthritis of ReA is not felt to be helpful Kvien etal. This rash, which is usually painless, may be seen in a subset of males with ReA courtesy of Dawn M. This painless, pustular rash on the soles of the feet may be seen in Reiter syndrome, a form of ReA courtesy of Dawn M.
The precise mechanisms of how the infectious agents in ARF initiate a remote, inflammatory response are not known, but depend on both host and pathogen characteristics. This form of reactive arthropathy tends to have a migratory pattern, with primarily large joint involvement.
Insufficiency murmurs may be noted. The rash of erythema marginatum 79 is uncommon as are subcutaneous nodules.
Join Kobo & start eReading today
A late finding of rheumatic fever is chorea, which is also very uncommon. Alternatively, serologic studies may be preferred. An ECG is done to document cardiac conduction abnormalities or other markers of structural cardiac damage. IMAGING Like the other forms of reactive arthropathies, the risk of joint damage is very low, and imaging affected joints may show evidence of soft tissue swelling, but no joint space narrowing or erosion.
Imaging of the heart with echocardiography is very important in assessing potential cardiac disease in ARF. Table 10 Modified Jones criteria for the diagnosis of acute rheumatic fever Dajani etal. Higher levels of acute-phase reactants and more treatment- resistant arthropathy favor the diagnosis of ARF Barash etal. Other forms of ReA may have similar clinical features. The presence of valvular heart disease is the primary prognostic marker. The American Heart Association AHA has guidelines for antibiotic prophylaxis for the prevention of progression of valvular heart disease in patients with rheumatic fever Dajani et al.
The management of the arthropathy of post-streptococcal arthropathies, including ARF, is highlighted box 7. The primary potential extra-articular complication is valvular heart disease. The precise mechanisms of how the infectious agents in PSRA initiate a remote, inflammatory response are not known, but are similar to ARF.
- Editorial Reviews.
- American Bouquet, No. 5: Last Rose of Summer - Piano Score.
- Write a review!
As noted previously, higher levels of acute phase reactants and more treatment- resistant arthropathy favor the diagnosis of ARF Barash et al. Cases of carditis have been described following the diagnosis of PSRA, thus antibiotic prophylaxis is indicated see below. Post-streptococcal arthropathies 59 The duration of antibiotic prophylaxis in patients who have had PSRA is unclear, but one reference suggests prophylaxis for 1 year after diagnosis Dajani et al.
The management of the arthropathy of post-streptococcal arthropathies, including PSRA, is highlighted box 7. PSRA is not associated with cardiac involvement. Although its etiology, pathogenesis, clinical findings, and laboratory abnormalities are similar to adult-onset SLE, children affected with lupus have special considerations regarding physical and emotional growth and development, treatment toxicities, and lifelong burden of chronic disease. The estimated incidence of pediatric SLE is 0. Lupus occurs throughout the world, and may be more frequent in non-white populations.
SLE, like many autoimmune diseases, is more common in females than males. In a genetically susceptible individual, there is loss of normal homeostatic control of immunologic reactivity to antigens. The immune complexes may be ineffectively cleared, resulting in cytokine release, inappropriate inflammation, and tissue destruction Hormonal factors are also implicated, given the female-to-male predominance.
This illustrates the heritability of lupus, but the actual susceptibility to SLE is multifactorial and polygenic. However, many drugs have been implicated with this phenomenon. Drug-induced lupus is associated with anti-histone antibodies. Systemic lupus erythematosus 63 80 Pathogenesis of SLE. Apoptotic bodies 81 Pathogenesis of SLE. RBC, red blood cell; BM, basement membrane. In adults, SLE is diagnosed when 4 of the 11 criteria are met. Constitutional symptoms such as fevers, malaise, and weight loss are common at disease presentation and with flares of disease activity.
Musculoskeletal involvement may include arthralgias or arthritis that is usually non-erosive and non-deforming. Malar rash Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds 2. Discoid rash Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions 3.
Photosensitivity Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation 4. Oral ulcers Oral or nasopharyngeal ulceration, usually painless, observed by physician 5. Arthritis Non-erosive arthritis involving two or more peripheral joints, characterized by tenderness, swelling, or effusion 6. Serositis a Pleuritis - convincing history of pleuritic pain or rubbing heard by a physician or evidence of pleural effusion OR b Pericarditis - documented by ECG or rub or evidence of pericardial effusion 7.
Renal disorder a Persistent proteinuria greater than 0. Neurologic disorder a Seizures - in the absence of offending drugs or known metabolic derangements, e.
Customers who bought this item also bought
For the purpose of identifying patients in clinical studies, a person shall be said to have SLE if any 4 or more of the 11 criteria are present, serially or simultaneously, during any interval of observation. Reed, MD, Mayo Clinic. Lupus is an additional independent risk factor for atherosclerosis, and adult lupus patients can have myocardial infarctions earlier than the general population Deapen et ctl. The most common pulmonary lesions in SLE are pleural effusions which may present with pleuritic chest pain or shortness of breath Pulmonary function testing is followed and lupus patients most commonly develop a restrictive pattern.
Pulmonary emboli from antibodies against cardiolipin or a lupus anticoagulant can also cause shortness of breath and chest pain 87, Liver dysfunction in the form of acute autoimmune hepatitis is the most frequent liver pathology related to SLE; however, steatohep- atitis is common secondary to steroid use and obesity Systemic lupus erythematosus 67 Diffuse pulmonary emboli in a patient with anticardiolipin antibody 87 ; CT scan demonstrating pulmonary artery thrombosis 88 ; CT scan of the liver showing diffuse fatty infiltrate in a patient with lupus on high-dose steroids When present, nephritis in children is often more severe than in adults with SLE.
It can show at presentation or develop many years later. Patients with nephritis present with hematuria and proteinuria. Children commonly have a renal biopsy to evaluate the extent of lupus involvement if they present with or develop abnormalities in their urinalysis. Class 1 Minimal mesangial lupus nephritis Normal glomeruli by light microscopy, but mesangial immune deposits by immunofluorescence Class II Mesangial proliferative lupus nephritis Purely mesangial hypercellularity of any degree or mesangial matrix expansion by light microscopy, with mesangial immune deposits.
Segmental is defined as a glomerular lesion that involves less than half of the glomerular tuft. This class includes cases with diffuse wire loop deposits but with little or no glomerular proliferation. More serious symptoms can include seizures, strokes, bleeds, or coma Neuropsychiatric testing can be helpful to make the diagnosis and to follow progress. Systemic lupus erythematosus 71 developmental level. The malar rash , b is the most common cutaneous manifestation and is present over the nasal bridge, extends onto the cheeks, and spares the nasolabial folds.
It is often symmetric and well demarcated, slightly raised, and with texture. Petechiae and purpura can be related to vasculitis or underlying thrombocytopenia. Reed, MD, Mayo Clinic ; malar rash b. Ulcerations present in the oral or nasal mucosa can be painful, especially when present on the tongue.
These ulcerations are not as specific for SLE. Serositis may be detected on exam by the presence of pleural or pericardial rubs. Hematologic abnormalities are frequently seen in SLE patients at presentation and with flares of disease. Anemia in SLE patients can be either hemolytic or anemia of chronic disease. Pancytopenia can also be present. APL antibodies and the lupus anticoagulant are measured in a number of ways and must be present on at least two occasions 12 weeks apart for the ACR diagnostic criteria. SLE patients can have thrombosis and bleeding at the same time, making them difficult to treat.
C3 and C4 complements are consumed by the circulating immune complexes and are often low in SLE patients with active disease. C3 and C4 are followed clinically, because decreases in these components of the complement cascade correlate with systemic disease activity.
Pediatric Rheumatology : Thomas G. Mason :
Urinalysis is done regularly with a random urine protein-to-creatinine ratio to monitor for development of nephritis. Active renal disease is indicated by an increase in the first morning urine protein: For example, chest radiographs may show pleural effusion or an enlarged heart consistent with pericardial effusions characteristic of the serositis seen in lupus.
Joint radiographs and MR images may show soft-tissue swelling and joint fluid, but the arthritis of lupus is usually non- erosive and easily treated. A kidney ultrasound may be required if there is nephritis, or an abdominal ultrasound if there are liver function test abnormalities. For treatment-related toxicities, bone density scans help to evaluate the bone health of a patient on chronic corticosteroids. Table 14 Active and chronic glomerular lesions adapted from Weening et al.
Gottron papules in juvenile dermatomyositis Malignancy Fever, cytopenia, fatigue, pain, lymphadenopathy, hepatosplenomegaly Night pain, bone tenderness, normal complement, no urinary changes Systemic vasculitis Fever, fatigue, rash Nodules, calf pain, positive ANCA, bruits Juvenile idiopathic arthritis systemic Arthritis, fatigue, fever, rash, lymphadenopathy, marked anemia Lack of specific autoantibodies, normal complements, no major organ dysfunction Systemic viral infection Fever, lymphadenopathy, hepatosplenomegaly, cytopenias Lack of specific autoantibodies, normal complements 74 CHAPTER 4 Lupus erythematosus PROGNOSIS The natural history of lupus is characterized by a widely variable and unpredictable clinical course with periods of flare and quiescence.
Left untreated, SLE often results in progressive deterioration with a significant fatality rate. However, these numbers reflect all lupus patients and are not specific to lupus patients with a particular organ system disease. Other important lupus outcome measures include disease activity, cumulative organ damage, and functional health status. Several studies have shown that cumulative organ damage is common in patients with pediatric- onset SLE, with a frequency of damage of Most of the damage occurs in the musculoskeletal, renal, and neuropsychiatric systems, although the current damage index does not take into account pediatric-specific issues such as growth retardation, pubertal development, and the regenerative ability of children to recover and reverse some forms of damage.
Unfortunately, studies have also shown that patients with pediatric-onset SLE have poorer health-related quality of life and lower socio-economic status compared with their healthy peers Ravelli et al. Patients with childhood-onset SLE are living longer, but are subsequently dealing with the burden of chronic disease and treatment-associated morbidities, which greatly impact their physical, psychological, and financial wellbeing.
Treatment for pediatric SLE can be roughly divided into mild, moderate, and severe categories. Patients with childhood-onset SLE require treatment with high-dose corticosteroid Tucker et al. The management of SLE is outlined in the table Table Decreased cognitive function, seizures, and paralysis are examples of possible neurologic complications. Renal failure and hypertension may be the result of renal involvement.
The arthritis is not destructive. Bleeding can occur from severe thrombocytopenia. Opportunistic infections may occur from leukopenia. Complications of medical therapies such as gastritis associated with NSAIDs, infections associated with immunosuppressive medications, and bone disease related to corticosteroids may occur. Table 16 Management principles for systemic lupus erythematosus Characteristics Treatment Mild lupus No renal or other major organ system involvement.
Three sets of classification criteria for MCTD are widely used that include a variety of clinical features. Kasukawa criteria are used most frequently in the pediatric setting Table 1 7 Kasukawa Median age at onset is 11 years, ranging from 4 years to 16 years. However, given that MCTD is a disease with overlapping features of other autoimmune diseases, the following associations have also been reported: Table 17 Kasukawa's criteria for mixed connective tissue disease Patients must meet all three of the following criteria to be diagnosed with MCTD: Raynaud phenomenon or swollen fingers or hands or both Anti-RNP antibody positivity At least one abnormal finding from two or more of the following categories: Pericarditis, vasculitis, and hypertension have all been described.
Transverse myelopathy, seizures, and psychosis have also been reported. Inflammatory muscle disease with muscle weakness, mostly at disease onset. Digital ulcerations, subcutaneous nodules , calcinosis , telangiectasias, and photosensitivity can occur. Serositis has also been reported. Additional serologies that could be obtained include those more specific for particular rheumatologic diseases, such as: Children with MCTD may have more frequent and severe renal disease than adults, warranting periodic urine analyses.
A chest rediograph may be helpful in evaluating for possible cardiopulmonary disease. A high-resolution chest CT scan may be indicated if there is concern for pulmonary fibrosis. An upper GI series with small bowel follow-through or swallow study may be useful in evaluating for esophageal or GI dysmotility or strictures. The serologic profile of patients with MCTD may also be somewhat prognostic. Renal disease was uncommon but was the cause of the only two disease-related deaths in the study.
Pulmonary hypertension and CNS disease have been shown to be persistent, possibly relaying worse prognosis Kasukawa The annual disease-specific mortality in pediatric MCTD is reported to be per patients Mier et al. Calcium channel blockers such as amlodipine and nifedipine and topical nitropaste can be used for complaints of Raynaud phenomenon. Myocardial involvement can lead to cardiac dysfunction.
Pulmonary fibrosis may occur after interstitial lung disease. Digital ischemia may also occur. The arthritis is usually not destructive. These autoantibodies pass through the placenta and can cause neonatal organ damage including to the skin, liver, brain, heart, bloodvessels, and blood elements. Half of infants with NLS have mothers who are clinically well. NLS results from transplacental passage of autoantibodies which target fetal and neonatal tissues for immune destruction.
I I I Newborn with neonatal lupus rash. These connective tissue disorders are characterized by the presence of autoantibodies that could cross the placenta perinatally. The presence of bradycardia, cytopenias, or characteristic skin lesions in a newborn also prompts an evaluation for NLS.
The fetal bradycardia could be detected in utero prior to delivery. Annular erythema , bullous lesions on the soles and palms, and facial telangiectasias have also been described. Lesions typically involve the face and scalp, but are also found on the neck, trunk, extremities, and intertriginous areas. A raccoon-eye rash is common, whereas the malar rash is rarely seen. The rash may be be present at birth but more commonly develops within the first few months of life.
Lesions can develop at any time up to 6 months of age, and can be exacerbated by sunlight or phototherapy. Residual dyspigmentation and atrophy are rare. Neonatal lupus syndrome 81 Cardiac - the most severe and life- threatening complication is congenital heart block CHB. Incomplete heart block can progress to higher degrees of block years after the postnatal period Askanase et al. Ventricular endocardial fibroelastosis can present prenatally or postnatally in the first year of life.
Hematologic manifestations - thrombocytopenia and anemia are the most common hematologic manifestations and typically worsen in the first days after birth and resolve after disappearance of maternal autoantibodies. Cases of neutropenia, transient pancytopenia, aplastic anemia, hemolytic anemia, and thrombosis have also been reported.
Liver - the primary manifestations are transaminitis, cholestasis, and hepatosplenomegaly. The two most common presentations of NLS liver disease are: Most infants have spontaneous resolution of liver abnormalities by 6 months after clearance of maternal autoantibodies. Severe perinatal hepatic dysfunction, often with the phenotype of neonatal iron storage disease, suggests a poor prognosis and warrants treatment with corticosteroids Lrankovich et al.
Cerebral dysmaturation and vasculopathy affecting the blood vessels that supply the basal ganglia and germinal matrix are also reported Lrankovich et al. Rarely, infants with NLS can have hypotonia, seizures, developmental delay, and feeding difficulty Lrankovich et al. Pneumonitis, pulmonary capillaritis, glomerulonephritis, and nephrotic syndrome are rare manifestations of NLS and may be suggestive of infantile primary SLE. The initial screening can be done using maternal serum or cord blood. A complete evaluation for congenital infection should be pursued simultaneously since congenital infections mimic NLS and require prompt antibiotic therapy.
Blood counts, liver function tests, and thyroid hormone levels should be evaluated in all infants born to mothers with autoimmune diseases and asymptomatic mothers with implicated autoantibodies. Neonatal iron storage disease and giant cell hepatitis have similar liver pathology and presentation. Peroxisomal disorders can also present with rhizomelic chondrodysplasia. Permanent sequelae can occur when inflammation is extensive skin, liver, or bone marrow or when vulnerable tissues are involved cardiac conduction system and small end-organ blood vessels.
Maternal disease activity during pregnancy predicts worse outcomes. For cardiac involvement, maternal oral dexamethasone therapy and IVIG in utero have been used but efficacy and risks are still questionable. Sympathomimetics are used for severe intrauterine and postnatal bradycardia until a pacemaker can be placed. Corticosteroids are recommended for active carditis postnatally. Corticosteroids are also used for severe or persistent liver involvement.
In most cases, affected neonates present with an isolated manifestation of NLS, although multiorgan dysfunction can occur. The most common multiorgan presentation of NLS is a sick preterm infant who has anemia, thrombocytopenia, liver dysfunction, and respiratory failure. In addition to the standard evaluation for congenital infection, a timely evaluation for NLS, including ECG and maternal autoantibody profiles, may facilitate diagnosis. In addition, maternal thyroid and APL antibodies may be measured and a careful neurologic examination undertaken.
There is risk for bleeding and infection with the associated cytopenias. There exists a female: The etiology for CLE is thought to be multifactoral, with genetic predisposition, environmental factors, and host immune factors all contributing. This reaction can be delayed for up to months after the exposure. Alopecia can also develop. CCLE can present in a variety of ways: The most common type is DLE a, b , which has sharply demarcated scaly erythematous papules or plaques that can extend into the follicles. DLE lesions can also develop into squamous cell carcinoma.
There can be an associated superficial ulceration of nasal and oral mucosa. The most common localized ACLE rash is the classic malar or butterfly rash. The most generalized ACLE rash is photosensitive lupus dermatitis. It usually presents as a papulosquamous or annular polycyclic eruption. The lesions from SCLE can have a psoriasis-type or pityriasis look to them.
In addition, malar eruption, livedo reticularis, periungal telangiectasias, and generalized poikiloderma have been reported. However, association with SLE serology is neither diagnostic nor exclusionary. The following laboratory results have been reported in CLE: Localized CLE can be treated with varying potency topical steroids depending on the location of the lesions, with lower potency for facial lesions and higher potency for the palms and soles.
Intralesional steroids and lasers have been reported to treat localized CLE. Other non-steroidal topical agents used include: Systemic treatment of CLE includes the use of antimalarials such as hydroxychloroquine as a first-line agent. In more diffuse cases, systemic corticosteroids, antibiotics i. Skin cancers may also develop. Three general types commonly seen in pediatric rheumatology are juvenile dermatomyositis, juvenile polymyositis, and postinfectious myositis.
Classic findings are Gottron rash and papules, heliotrope rash, calcinosis cutis, and symmetric, proximal muscle weakness. Diagnosis is made by Bohan and Peter a: Skin findings with three other features are necessary to make the diagnosis Table Females are affected more often than males. The median age of onset is approximately 7 years with a median delay to diagnosis of months.
The etiology is not completely understood. The innate and adaptive immune systems and environmental factors may trigger JDM in a genetically susceptible host. Molecular mimicry may play a causative role. Non-infectious agents such as exposure to UV light and bone marrow transplantation as in a graft-versus-host paradigm are also hypothesized to be factors in JDM Table The pathogenesis of JDM involves the innate and adaptive immune system with the humoral and cell-mediated pathways causing vascular and muscle damage.
There is increased expression of major histocompatibility complex class I and II. These cells are arranged in a perivascular and perifascicular distribution There is perifascicular atrophy of type I and type II fibers. Infarcts and necrosis of muscle fibers can occur in the fascicle. Immune complex deposition with membrane attack complexes produces muscle inflammation. Juvenile dermatomyositis 89 Table 20 Environmental causes of myositis Agents Observations Infectious Coxsackie virus Antibodies to Coxsackie B viruses noted at onset of JDM compared with controls few in studies Influenza virus Seen with acute viral myositis children who subsequently developed JDM Hepatitis B and C Myositis development associated after acute illness Parvovirus Myositis development associated after acute illness Echovirus Myositis seen mainly in children with X-linked hypogammaglobulinemia Streptococcus spp.
The child may have difficulty getting out of bed or tire easily in physical education classes or sporting events. Other symptoms include dysphonia, hoarseness, and melena as a consequence of vasculopathy involving the GI tract. The eyelids may be swollen with a heliotrope rash and an overlying scale , Also, patches of erythema with poikilodermatous changes may occur in various areas of the face and the anterior and posterior neck I Eyelid vasculitis and heliotrope rash with periorbital edema in a child with JDM I 16 ; a heliotrope rash with periorbital edema in a child with JDM I 17 ;facial erythema,scaling and heliotrope rash in a child with JDM I 18 ; patchy, erythematous macular rash of the cheeks and upper eyelid inJDM.
The erythematous rash spares the perioral region I 19 ;patchy,erythematous macular rash of the cheeks and upper eyelid in JDM. The erythemasus rash spares the perioral region ; patchy, erythematous macular rash of the cheeks and upper eyelid and a heliotrope rash in JDM. The erythematous rash spares the perioral region ; patchy, erythematous macular rash with atrophic changes of the cheeks and perioral area inJDM.
The erythematous rash spares the perioral region Juvenile dermatomyositis 91 Patchy, erythematous macular rash of the cheeks and mouth inJDM. The erythematous rash spares the perioral region ; erythematous rash on cheek in JDM ; erythematous rash and atrophic changes on the face in JDM ; erythematous rash with atrophic changes on the neck in JDM ; erythematous rash and calcinosis on posterior neck in JDM ; erythematous rash and calcinosis on neck in JDM ; erythematous rash in a shawl pattern and aV-shaped distribution on the anterior chest, upper arms, and anterior neck in JDM Nailfold telangiectasias, periungal erythema, and hypertrophic ragged cuticles may be found with end-row loop capillary loss and bushy loops of capillary dilation and branching , , a.
Additional skin findings include panniculitis and gum erythema with bleeding Juvenile dermatomyositis 93 Gottron papules, erythematous, violaceous papules overlying the elbow joints in a child with JDM. Calcinosis has been well described in JDM. The deposits are firm, flesh-colored nodules present over bony prominences, long strips of calcium in the muscle bundles or in the subcutaneous tissue , , a, b.
The most commonly affected areas are the elbows, knees, and extremities , Calcinosis onset may occur within 3 years of diagnosis, and predictors for devel- I 38, Vasculitic skin lesion with severe Raynaud phenomenon in a child with JDM-scleroderma overlap. Juvenile dermatomyositis 95 a, b A girl with erythema and calcinosis on the anterior chest a ; cutaneous calcinosis b ; radiograph of liquified and crystallized calcinosis in the foot of a patient with J DM ; radiograph of the arm of a patient with JDM showing sheet-like calcinosis of the fascia Lipodystrophy is seen in a subset of JDM, usually those with long-standing and diffi- cult-to-treat disease.
Loss of extremity fat and an increase in truncal fat, with elevated tryg- lycerides, alterations of glucose, and insulin metabolism. Poikiloderma is commonly seen associated with lipodystrophy Symmetric, proximal muscle weakness is seen in most children, although it may present insidiously. Muscle involvement usually occurs in the deltoids and the quadriceps muscles, or both muscle groups. Other organ systems may be commonly involved in JDM - including interstitial lung disease , GI vasculitis, dysphonia, and dysphagia - and less commonly cardiac, ophthalmologic, and CNS disease.
Muscle enzymes may return to normal within a few months of disease onset if the child is treated adequately at times when disease remains active. There may be cutaneous, subcutaneous, and fascial inflammation. MRI may help to localize an area for muscle biopsy. Muscle ultrasound is generally not used in the diagnostic workup, but may reveal increased muscle echogenicity and attenuation.
However, muscle ultrasound may be used to localize and monitor calcinosis Other connective tissue disease mimickers include juvenile idiopathic arthritis and juvenile polyarteritis nodosa. Juvenile dermatomyositis 99 Other childhood muscle diseases that resemble myositis include the muscular dystrophies, Duchenne and Becker, and the metabolic myopathies. Other disease entities to consider are sarcoid, viral myositis, celiac disease, and IBDs Table Approximately one- third of children died from the disease and another third suffered from severe functional disability.
With adequate and early treatment, however, children are now able to live normal lives. Calcinosis cutis has been a major cause of morbidity due to cosmetic disfigurement, contractures, and ulcerations resulting in secondary skin infections. Assessment scales of strength and physical endurance such as the Childhood Cross-sectional CT scan of bilateral thighs demonstrating diffuse sheets of calcification along the myofascial planes of both thighs in JDM. Weaning of corticosteroids may take 2 years and when additional immunosuppressive agents are used weaning may at times occur over 1 year.
Other immunosuppressive agents are added, at the onset of disease, to the corticosteroid regimen to reduce steroid side effects, disease relapse, and progressive disease. A commonly used agent is methotrexate with folic acid supplementation. Other immunosuppressive agents used include IVIG, cyclosporine, and rituximab.
Combination therapy is often used as two agents generally work better to induce a rapid response and this is associated with less steroid toxicity. Opportunistic infections can occur from corticosteroids and other immunuosuppressive therapies used to treat JDM.
- Also Available In:.
- The unknown Mongol!
- Also Available As:.
- Paediatric Rheumatology - Helen E. Foster; Paul A. Brogan - Oxford University Press.
- Account Options.
It is a systemic autoimmune disease of the muscles without skin involvement. Because of its rarity, the incidence of JPM is unknown. As in JDM, girls are affected more than boys with a sex ratio of The etiology of JPM is largely unknown. Pathogenesis of JPM likely involves the innate and adaptive immune system including the cell- mediated pathways causing muscle damage. Muscle weakness is the hallmark feature, usually occurring in the deltoids and the quadriceps muscles; sometimes it is severe resulting in hypotonia.
Similar to JDM, constitutional symptoms may occur prior to obvious muscle weakness. A non-erosive arthritis, dysphonia, hoarseness, esophageal dysmotility, and muscle pain are other associated features. There are no cutaneous or nailfold capillary abnormalities. As with JDM, the child may demonstrate an abnormal Gower maneuver with an inability to rise to a standing position without the use of the arms. Muscle enzymes may return to normal within a few months of disease onset if the child is treated adequately.
Autoantibodies to the signal recognition particle have been observed in African-American girls with more aggressive and refractory disease, or cardiac manifestations. As in JDM, muscle ultrasound generally is not used in the diagnostic workup, but may reveal increased muscle echogenicity and attenuation. Evaluation with muscle biopsy is key to differentiate JPM from other causes of myopathy. However, JPM is often chronic and may be refractory to steroids. Weaning of corticosteroids may take 2 years or longer.
Other immunosuppressive agents are added to reduce steroid side effects, disease relapse, and progressive disease. As in JDM, a commonly used immunosuppressant is methotrexate with folic acid supplementation. Viruses associated with myositis include influenza A and B and Coxsackie B viruses. Other infectious causes include toxoplasmosis, Trichinclla spimlis , and staphylococcal and streptococcal bacteremia. Also, clostridia, Salmonella spp. Pyomyositis, as from Staphylococcus spp.
Illness lasts days. Trichinosis may cause fever, diarrhea, and abdominal pain. Trichinosis may cause periorbital edema and facial swelling. If pyomyositis is present, the affected muscle may be warm and tender. Myositis associated with schistosomiasis or trichinosis may be associated with marked peripheral blood eosinophilia. Mebendazole or thiabendazole is used to treat trichinosis. As a result of the ubiquity of blood vessels, the variability of their structure and function, and the multitude of ways in which they may be affected, vasculitis can lead to anything from numbness to pain, thrombosis to bleeding, aneurysms to obstruction.
Large gaps in the understanding of nosology, etiology, and pathogenesis confound the study of vasculitis. This lack of information, in turn, complicates attempts at classification. Primary vasculitides may be classified according to their clinical manifestations, the size of blood vessels involved, the histology of vascular damage, or the presumed disease pathogenesis. It is diagnosed by clinical criteria, not histology or angiography.
Asians are affected times as frequently as whites; blacks and Hispanics have an intermediate risk. The etiology of KD is unclear, but vascular damage likely plays a role. Blood vessel damage in KD results from an aberrant immune response leading to endothelial cell injury and vessel wall damage. The fever and systemic inflammation of KD reflect elevated levels of proinflammatory cytokines such as TNF, IL-1, and IL-6 that are also thought to mediate the underlying vascular inflammation. Pathologically, the finding of macrophages and IgA-producing plasma cells in vessel walls of children with KD is unique.
The diagnosis requires the presence of fever lasting 5 days or more without any other explanation, combined with at least four of five manifestations of mucocutaneous inflammation. Children who do not meet the criteria may none-the-less develop coronary artery changes. Vesicular or bullous lesions are rare. Late in the course of the disease, children develop extremity changes, including indurated edema of the dorsum of the hands and feet and diffuse erythema of the palms and soles.
In addition, one-third of children have a self-limited small-joint polyarthritis during the first week of illn ess. When present, lymphadenopathy tends to involve primarily the anterior cervical nodes overlying the sternocleidomastoid muscle, while diffuse lymphadenopathy or splenomegaly should prompt a search for alternative diagnoses. Intra- hepatic congestion often leads to elevated transaminase levels or mild hyperbilirubinemia.
Coronary angiography is rarely necessary as echocardiograms adequately visualize involved vessels in most children. Measles, echovirus, and adenovirus may share many of the signs of mucocutaneous inflammation, but they typically cause less inflammation and lack the extremity changes seen in KD. Toxin-mediated illnesses, especially 3-hemolytic streptococcal infection and toxic shock syndrome, lack the ocular and articular involvement typical of KD.
PROGNOSIS Although most small aneurysms fully resolve by echocardiogram, there is a concern that vascular reactivity may not return to normal despite grossly normal appearance. Children should thus be followed indefinitely after KD, and additional cardiac risk factors should be monitored closely. When symptoms are prolonged beyond weeks, consideration should be given to an alternative diagnosis, including chronic vasculitides such as polyarteritis nodosa PAN.
At times, children are treated for suspected KD when the diagnosis is uncertain but no clear alternative explanation for the clinical findings can be identified.
Rarely other cardiac involvement occurs, such as myocarditis. Similar to Kawasaki disease, most cases occur in children under 7 years of age, almost twice as many boys as girls contract the condition, and peak incidence tends to be during the colder winter months. HSP appears to represent an aberrant immune response to an infectious or antigenic stimulus. In HSP, the alternate pathway of complement activated by large IgA-containing immune complexes may be important, though precisely how this contributes to the HSP phenotype is poorly understood.
Renal biopsies typically show an endocapillary proliferative glomerulonephritis involving endothelial and mesangial cells. Proliferation of extracapillary cells also may occur, resulting in variable degrees of crescent formation. Arthritis may affect any joint in an asymmetric and migratory pattern, though lower extremities are more commonly involved. GI involvement ranges from colicky abdominal pain to profuse bleeding, intussusception, and perforation.
Renal disease usually is observed more than 10 days after the onset of illness. Edema may involve the scrotum , dorsa of the hands or feet, and even the scalp. The arthritis of HSP is transient, causing neither chronic joint changes nor permanent sequelae. BUN and creatinine levels and urinalysis are monitored to evaluate renal involvement. In severe cases, renal biopsy is helpful for excluding other causes of acute nephritis, nephrotic syndrome, or renal failure, and for estimating prognosis.
Since primarily small vessels are involved, skin or renal biopsies are generally more helpful than vascular imaging if definitive evidence of vasculitis is needed. Exclusion of other vasculitides causing palpable purpura, especially hypersensitivity vasculitis, Wegener granulomatosis, and Churg-Strauss syndrome, might require testing for ANCA, serum complement levels, or even a skin biopsy. Acute hemorrhagic edema of infancy , a benign febrile condition of infants, presents with ecchymotic-like macular purpura distinct from that of HSP.
Younger patients tend to have milder disease of shorter duration, while adults have a higher incidence of severe renal involvement. Generally there is a correlation between the severity of urinary abnormalities and the chances of developing chronic renal disease, with those demonstrating both nephritic and nephrotic changes at greatest risk. Use of steroids, whether in children who fail to respond to NSAIDs or in those thought to be at highest risk of developing renal compromise, is controversial.
More potent immunosuppressive agents are reserved for children with biopsy- proven crescentic glomerulonephritis or other life-threatening complications such as cerebral or pulmonary hemorrhage. Rarely, intestinal perforation has been reported. The segmental panmural fibrinoid necrosis with nodule and aneurysm formation give this condition its name. PAN is characterized by transmural necrosis of the walls of medium-sized arteries.
The resulting recruitment of polymorphonuclear neutrophils and monocytes, leading to focal areas of necrosis, is typical of an Arthus reaction. It commonly occurs after a sore throat or streptococcal pharyngitis. Livedo reticularis, maculopapular rash, painful skin nodules, edema, and arthritis mostly affect the knees and ankles.
Systemic PAN may involve virtually any muscular artery. Consequently, in addition to constitutional symptoms, it may cause a vast array of organ dysfunction. Arthritis and fever frequently accompany these findings, but other organ systems are rarely involved. Systemic PAN, on the other hand, may involve any organ system, so evidence of this protean and widespread condition may lead to anything from cranial nerve palsies and myocardial infarction to testicular pain and an acute abdomen. Complement levels are normal. CT or MR angiography is generally preferred to conventional angiography because they are less invasive and have less potential for complications a-c.
Echocardiography may be used to visualize coronary arteries when cardiac involvement is suspected. None-the-less, in some patients, conventional angiography may be necessary to determine the extent and severity of vascular involvement As a result of its pleomorphism, PAN may be confused with other rheumatologic conditions including systemic-onset JRA, Kawasaki disease, and dermatomyositis. Small vessels are spared in classic PAN, so glomerulonephritis typically is not a feature of this condition. Such an outcome is predicated, however, on close follow-up and aggressive management of active disease or its complications.
In systemic disease, on the other hand, vascular inflammation must be controlled in order to prevent compromise of vital vascular beds. In both cases the mainstay of therapy is high-dose steroids, but, since the disease tends to persist or relapse, many patients require steroid-sparing agents for long-term management.
Penicillin prophylaxis may prevent disease flares due to recurrent streptococcal infections. Organ ischemia may occur secondary to vascular damage. Potential complications related to therapy include gastritis with NSAIDs, avascular necrosis, and opportunistic infection with corticosteroids. Polyarteritis nodosa a-c CT angiography showing hepatic artery narrowing courtesy of Ann M. Reed, MD, Mayo Clinic c. They primarily target the respiratory, renal, and cutaneous systems, and they often develop in the wake of a viral infection.
The genetic and environmental factors responsible for their development, however, are not known. These antibodies most likely stabilize adherence of rolling neutrophils to endothelium, and activate neutrophils and monocytes to undergo an oxidative burst. Activation of phagocytic cells causes upregula- tion of proinflammatory cytokines such as TNF-oc and IL-8 , with resultant localized endothelial cell cytotoxicity. Arthralgias, ocular findings, and skin or renal involvement occur in more than half, while GI disease and CNS involvement are less typical.
CSS presents initially as allergic rhinitis and asthma. After months or years, peripheral eosinophilia and pulmonary infiltrates develop Only later do manifestations of systemic vasculitis become evident, with weight loss, fever, arthralgia, myalgia, nodular rash, and neuropathy.
Reed, MD, Mayo Clinic ; crescent formation and glomerular collapse due to Wegener granulomatosus courtesy of Michael Somers, MD ; glottic and subglottic stenosis in Wegener granulomatosus Similarly, children with CSS are recognized to have asthma, but then their symptoms worsen and become increasingly refractory to routine management. Unusually persistent or severe manifestations, or development of hemoptysis or hypertension, suggest evolution beyond routine childhood illnesses. Accordingly, a positive ANCA should not replace a tissue biopsy in confirming the diagnosis of vasculitis, nor should ANCA screening substitute for a careful history and physical examination.
Similarly, while peripheral eosinophilia is characteristic of active CSS, it is more frequently encountered in common allergic conditions, and therefore should be only one of many factors weighed during the diagnostic process. Even in asymptomatic children with WG, up to one-third have radiographic abnormalities , , Necrotizing granulomatous vascular inflammation in a child with suggestive clinical features is strongly suggestive of WG.
Subglottic stenosis does not respond to systemic therapy, but rather requires surgical dilation and local steroid injections. Most recently, rituximab has been proposed as a potential replacement for both cyclophosphamide and long-term steroids in AAVs. Farge airway disease may lead to subglottic stenosis. Nasal septal perforation has been reported. Reed, MD, Mayo Clinic It most commonly develops during the third decade of life, but the disease has been reported in infancy. In a recent review of childhood TA the mean age of onset was The cause of TA remains unknown, though histopathology and immunohistochemistry of biopsy and autopsy samples from adults with TA suggest a primarily T-cell-mediated mechanism.
Certain HLA associations have been found in Japan, but these have not been confirmed in other populations. TA lesions consist of granulomatous changes progressing from the vascular adventitia to the media, indistinguishable from those seen in giant cell and temporal arteritis. This histopathology, and the correlations between levels of IL-6 and RANTES and disease activity, suggest that T cells and macrophages play a central role in the disease.
The distribution of vessel involvement in children parallels that of adults, with diffuse aortic involvement predominating. Signs and symptoms include hypertension, cardiomegaly, elevated ESR, fever, fatigue, palpitations, vomiting, nodules, abdominal pain, arthralgia, claudication, weight loss, and chest pain. The size of the vessels involved and the spotty nature of the vascular inflammation make biopsies impractical.
CT and MR angiograms have supplanted traditional angiograms as the modalities of choice They are less invasive, and MRI offers the opportunity to monitor disease activity by revealing evidence of ongoing vessel wall inflammation. PET scanning may similarly allow simultaneous visualization of disease anatomy and physiology. Loss of peripheral pulses, on the other hand, generates a more restricted list, especially in children. Before starting these, however, it is important to test patients for tuberculosis, since aortitis is associated with mycobacterial infections, especially in less developed countries.
Hypertension may develop for many reasons. Reed MD, Mayo Clinic. It was originally described in adults in , and diagnostic criteria were proposed in In most cases, however, children are diagnosed with PACNS without a convincing environmental, infectious, or genetic explanation. Biopsies of children with PACNS typically demonstrate lymphocytic infiltration of the wall of cerebral vessels Adults typically have necrotizing granulomas that are not commonly seen in children.
These findings suggest an ongoing immunologic attack against an unidentified antigen, though whether this represents true autoimmunity or a response to an exogenous agent is not known. Though small-vessel disease may present with the same findings, such patients are more likely to have systemic complaints such as fever or malaise as well. Global CNS dysfunction is uncommon, though children may have seizures or gradual cognitive decline.
Buy for others
Small- vessel disease is commonly multifocal, so patients often have subtle evidence of isolated abnormalities of strength or sensation of which they may not even be aware. A patient examiner may also detect cranial neuropathies, optic neuritis, or myelitis. Page 1 of 1 Start over Page 1 of 1. Textbook of Pediatric Rheumatology. Pediatric Rheumatology in Clinical Practice. Editorial Reviews From the Back Cover Physicians caring for children with rheumatic diseases need a varied and highly developed set of clinical skills, including general pediatrics, immunology, musculoskeletal medicine and the management of chronic disease.
Authors at a world renowned center of excellence Concise text with emphasis on more common conditions Wealth of top-quality illustrations. Product details File Size: April 30, Sold by: Share your thoughts with other customers. Write a customer review. Showing of 2 reviews. Top Reviews Most recent Top Reviews. There was a problem filtering reviews right now.
Please try again later. I have bought several copies for pediatric residents. The entire book is black and white! Amazon Giveaway allows you to run promotional giveaways in order to create buzz, reward your audience, and attract new followers and customers. Learn more about Amazon Giveaway. Set up a giveaway. There's a problem loading this menu right now. Learn more about Amazon Prime. Get fast, free shipping with Amazon Prime. Get to Know Us. English Choose a language for shopping. Not Enabled Word Wise: Not Enabled Enhanced Typesetting: Amazon Music Stream millions of songs.
Amazon Advertising Find, attract, and engage customers.