Childhood sarcoidosis manifesting as juvenile rheumatoid arthritis.

Sarcoidosis in childhood may manifest primarily as arthropathy and uveitis, mimicking juvenile rheumatoid arthritis. The characteristic appearance and course of the inflammation at these sites, particularly the uveitis, is often a major clue to correct diagnosis. These children generally do not have the fulminant systemic manifestations such as pulmonary disease usually ascribed to sarcoidosis.

Eosinophilic fasciitis presenting as inflammatory polyarthritis.

Eosinophilic fasciitis is an uncommon disorder characterized by peripheral blood eosinophilia, hypergammaglobulinemia, and scleroderma-like inflammation of skin and fascia. Joint contractures occur but typically without synovitis and effusion. Our patient presented with symmetric polyarthritis and subsequently characteristic features of eosinophilic fasciitis developed. It is important to consider this disorder in patients presenting with arthritis as well as those presenting with skin changes because prior treatment with corticosteroids can alter biopsy findings, leading to diagnostic confusion, and possible adverse effects on the long-term treatment regimen.

The validity and reliability of parental ratings of disease activity in juvenile rheumatoid arthritis.

This study assessed the validity and reliability of parental ratings of morning stiffness, activity limitations, and pain complaints on a sample of 31 patients with juvenile rheumatoid arthritis (JRA). Parental ratings were found to be significant predictors of active joint counts, accounting for 40% of the variance in joint counts. Internal consistency reliability was moderately high (0.819), but test-retest reliability was moderate to low; this is not unexpected given the fluctuations in symptoms of JRA over time. The results suggest that parental ratings are valid and moderately reliable measures of disease activity in JRA. Regular monitoring of symptoms by parents could be a useful source of information for health care providers in making treatment decisions and can increase parental and patient involvement in the treatment process.

Improving compliance with prednisone therapy in pediatric patients with rheumatic disease.

Compliance with regimens for pediatric rheumatic diseases is often poor, and few studies have evaluated strategies for improving compliance. This study utilized relatively simple behavioral and educational strategies to improve compliance with prednisone for three patients with pediatric rheumatic diseases (systemic lupus erthematosus and dermatomyositis). These strategies were implemented in a pediatric rheumatology setting and resulted in improved compliance that was maintained at 6- and 12-month follow-up. During baseline, patients were found to be overmedicating as well as undermedicating. This study is a systematic replication of an earlier study that demonstrated that behavioral and educational strategies can improve compliance with medications for juvenile rheumatoid arthritis. It also raises the possibility of overmedicating as a compliance problem to be managed.

Pharmacotherapy of juvenile rheumatoid arthritis.

Salicylate is the drug of first choice in the initial treatment of juvenile rheumatoid arthritis. In therapeutic dosage it will adequately control joint symptoms in the majority of patients. For children who do not respond to or are intolerant of salicylate, a change to one of the other nonsteroidal anti-inflammatory agents is appropriate. In progressive polyarthritis unresponsive to the above agents, the addition of gold, antimalarials, or penicillamine is indicated, preferably in that order. Corticosteroid therapy should be reserved for selected patients meeting specific criteria. Pharmacotherapy of juvenile rheumatoid arthritis should always be individualized. For optimal treatment of the whole child it must be combined with both physical and educational measures.

Chronic pain and emotional distress in children and adolescents.

Pediatric chronic pain continues to be relatively underinvestigated and undertreated. The objective of the present cross-sectional study was to investigate the emotional distress hypothesized to be concurrently associated with the chronic pain experience in children and adolescents. One hundred and sixty children and adolescents with chronic pain and their parents completed standardized assessment instruments measuring pain intensity, depressive symptoms, state anxiety, trait anxiety, general self-esteem, and internalizing and externalizing behavior problems. Consistent with the a priori Biobehavioral Model of Pediatric Pain, higher patient-perceived pain intensity was associated with higher depressive and anxious symptoms, lower general self-esteem, and higher behavior problems. The results are discussed in regard to preventing and treating pain and suffering in children and adolescents with chronic pain.

Nonsteroidal antiinflammatory drugs. Renal toxicity. Review of pediatric issues.

Nonsteroidal antiinflammatory drugs (NSAIDs) are an important part of therapy for childhood rheumatic disease and for the symptomatic management of clinical problems such as fever, musculoskeletal pain, and dysmenorrhea. Overall, there is a low incidence of significant complications resulting from NSAID therapy in children, but serious adverse effects on renal function have occurred. The physician should be aware of the potential renal toxicity of these drugs and monitor patients accordingly.

The pain puzzle: a visual and conceptual metaphor for understanding and treating pain in pediatric rheumatic disease.

Chronic pain is a primary clinical manifestation of pediatric rheumatic diseases that for some children persists into adulthood and is associated with increased disability. The "pain puzzle" is presented as a visual and conceptual metaphor for understanding and treating pediatric rheumatic disease related pain. This metaphor is consistent with a biobehavioral model of pain that focuses on the unique and interactive components of nociceptive activity, emotions, cognitions, and behavior in the experience of pain. We describe the parts of the pain puzzle and review the implications for treating pediatric rheumatic disease related pain.

The Cutaneous Assessment Tool: development and reliability in juvenile idiopathic inflammatory myopathy.

OBJECTIVES: Clinical care and therapeutic trials in idiopathic inflammatory myopathies (IIM) require accurate and consistent assessment of cutaneous involvement. The Cutaneous Assessment Tool (CAT) was designed to measure skin activity and damage in IIM. We describe the development and inter-rater reliability of the CAT, and the frequency of lesions endorsed in a large population of juvenile IIM patients. METHODS: The CAT includes 10 activity, 4 damage and 7 combined lesions. Thirty-two photographic slides depicting IIM skin lesions were assessed by 11 raters. One hundred and twenty-three children were assessed by 11 paediatric rheumatologists at 10 centres. Inter-rater reliability was assessed using simple agreements and intra-class correlation coefficients (ICC). RESULTS: Simple agreements in recognizing lesions as present or absent were generally high (0.5-1.0). ICCs for CAT lesions were moderate (0.4-0.75) in both slides and real patients. ICCs for the CAT activity and damage scores were 0.71 and 0.81, respectively. CAT activity scores ranged from 0 to 44 (median 7, potential range 0-96) and CAT damage scores ranged from 0 to 13 (median 1, potential range 0-22). The most common cutaneous lesions endorsed were periungual capillary loop changes (63%), Gottron's papules/sign (53%), heliotrope rash (49%) and malar/facial erythema (49%). CONCLUSIONS: Total CAT activity and damage scores have moderate to good reliability. Assessors generally agree on the presence of a variety of cutaneous lesions. The CAT is a promising, semi-quantitative tool to comprehensively assess skin disease activity and damage in IIM.

Uses of nonsteroidal anti-inflammatory drugs in pediatrics.

Nonsteroidal anti-inflammatory drugs have an expanding role in pediatrics. The indications include (1) treatment of childhood rheumatic disease and other chronic inflammatory conditions; (2) symptomatic treatment of clinical problems such as fever, musculoskeletal pain, or dysmenorrhea; and (3) induction of closure of patent ductus arteriosus. Nonsteroidal anti-inflammatory drugs are generally well tolerated with a low incidence of serious side effects. However, potentially serious gastrointestinal, hepatic, central nervous system, and renal adverse effects have occurred. The pediatrician should be familiar with the pharmacokinetics and dosages of the various nonsteroidal anti-inflammatory drugs as well as laboratory tests necessary to monitor their use. Guidelines are suggested.

Juvenile rheumatoid arthritis and spondyloarthropathies.

Further insight into the etiology and pathogenesis of juvenile rheumatoid arthritis (JRA) is presented in recent immunogenetic studies, particularly the allele associations of the pauciarticular pattern of disease. Evidence suggests that bacterial heat-shock proteins may be significant in the chronic inflammatory response in children with arthritis. Data on the role of complement activation and cytokines and their receptors also are presented. Coagulopathy in JRA may have more than one etiologic factor, including a viral agent, as may the disease itself. In the treatment of growth abnormalities in JRA, the neuroendocrine system, recombinant growth hormone, intravenous iron therapy, and nutritional supplementation are all areas of recent investigation. In outcome studies, ocular involvement and the presence of circulating IgM rheumatoid factor appear to be risk factors for disability. However, disease of less than 2 years' duration and absence of radiographic lesions likely predict good response to methotrexate therapy.

Adjunctive use of hydroxychloroquine in childhood dermatomyositis.

We examined retrospectively the use of hydroxychloroquine in 9 patients with childhood dermatomyositis (DM). These patients had an incomplete response to corticosteroid therapy or had disease exacerbation when attempting to taper corticosteroids. They received hydroxychloroquine in a mean dosage of 3.6 mg/kg (range 2-5 mg/kg) while maintaining corticosteroid therapy. Significant improvement was noted at 3 months of therapy in rash (p less than 0.0001), proximal (p = 0.0004) and abdominal muscle strength (p = 0.0001) and at 6 months of therapy in reduced prednisone dosage (p less than 0.02). These data suggest hydroxychloroquine may be helpful in some patients with childhood DM, particularly those with prominent cutaneous disease or significant corticosteroid toxicity.

Arthritis associated with inflammatory bowel disease in children.

Arthritis occurred in 23 of 136 (17 per cent) children and teenagers with inflammatory bowel disease, in 18 of 86 (21 per cent) patients with ulcerative colitis, and 5 of 50 (10 per cent) with granulomatous bowel disease. Eighteen children had peripheral arthritis which characteristically affected only a few large joints and was of brief duration and benign outcome. Five boys had spondylitis which was progressive and inseparable clinically from ankylosing spondylitis. Occurrence of joint manifestations was not associated with severity of bowel disease. Anemia and growth retardation occurred frequently. Mucocutaneous lesions were associated with peripheral arthritis but not with spondylitis. No patient had iridocyclitis. The possibility of bowel disease should be considered in children presenting with arthritis, particularly if gastrointestinal complaints, mucocutaneous lesions, anemia, or growth retardation are associated with pauciarticular arthritis. Peripheral arthritis is benign and regresses with improvement of underlying bowel disease but spondylitis is progressive and requires recognition and management for prevention of deformity.

Overview and report on international registry of sarcoid arthritis in childhood.

Interest in childhood sarcoidosis prompted the formation of an international registry in 1991. Over the next 5 years, 53 patients were registered by 23 physicians from 14 countries. All the patients had definite histologic evidence of sarcoidosis: noncaseating granulomas of the skin (31), synovium (15), liver (10), lymph node (eight), lung (five), muscle (four), conjunctiva (three), or kidney (one). All but nine patients developed polyarthritis; 38 of 44 had persistent arthritis. Of those with persistent polyarthritis, arthritis occurred at presentation in 16 of 38 patients and inflammation of the uveal tract of the eye occurred in 44 with involvement of both anterior and posterior segments in 21. One patient was blind at the time of the report. Other ocular complications included chorioretinitis, glaucoma, and phthisis bulbi. Laboratory abnormalities included mild anemia and elevated erythrocyte sedimentation rate (39 out of 45). Angiotensin converting enzyme levels were elevated in 14 out of 37 patients. Information on these patients with sarcoidosis helps develop a better understanding of this rare childhood disease. These patients are discussed in conjunction with an overview of sarcoid arthropathy.

Neonatal lupus syndrome.

Neonatal lupus erythematosus is a rare syndrome occurring in the first six months of life. It is characterized by the presence of Ro antibodies in the mother and affected infant, and at least one clinical manifestation--cardiac, hematologic, or dermatologic. If present, congenital heart block is irreversible and may be life-threatening in the neonatal period. Mothers of infants with neonatal lupus erythematosus may be asymptomatic when the affected infant is born but may later develop connective-tissue disease. The affected infants are at risk to develop systemic lupus erythematosus during adolescence or adulthood.

Legal rights of children with musculoskeletal disabilities.

As market forces increasingly control medicine, we must not forget the disabled, especially children. Although the costs of appropriate comprehensive services are substantial, it is well worth the cost to facilitate an adulthood in which these patients are on their own, possibly earning their livelihood, and experiencing the best quality of life our society can provide.

Seronegative juvenile rheumatoid arthritis and mast cell-associated gastritis.

We describe 4 children with seronegative inflammatory arthritis who had persistent, severe nausea and abdominal pain over several months, in spite of vigorous medical therapy, including antacids and histamine H2 receptor antagonists. Endoscopy and biopsy of gastric and duodenal mucosa showed antral gastritis and an increased number of mast cells in 3 of the 4 patients. In the fourth patient, urinary histamine levels were elevated. These findings suggest an association between inflammatory arthritis and localized mast cell disease in some individuals. Further studies are needed to determine whether this association represents an independent syndrome or whether mast cell-related disease is secondary to long-term treatment with nonsteroidal antiinflammatory drugs in children with mild arthritis.

Late recurrence of childhood dermatomyositis.

Patients with childhood dermatomyositis are generally categorized into one of 3 groups: limited or monocyclic, polycyclic, or continuous. Disease resolution in those with monocyclic disease after a period of 1 to 2 years is expected and those patients are not considered at life-long risk for connective tissue disease. We describe a patient, with an uncomplicated initial course, who had disease recurrence after 8 years of disease inactivity and almost 4 years after discontinuation of all medication.

Nonsteroidal anti-inflammatory drug therapy in chronic childhood iridocyclitis.

The adjunctive use of nonsteroidal anti-inflammatory drugs (NSAIDs) for the treatment of chronic iridocyclitis was evaluated in 14 patients, eight with juvenile rheumatoid arthritis and six with idiopathic iridocyclitis. In all patients, the activity of the iridocyclitis improved with the addition of NSAIDs to their treatment regimens, permitting reduction in the dose of corticosteroid drugs. These data suggest that NSAID therapy may have an adjunctive role in the treatment of chronic iridocyclitis in childhood.