Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases
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We describe 2 patients with systemic juvenile idiopathic arthritis and macrophage activation syndrome. Treatment with recombinant interleukin 1 receptor antagonist (anakinra) and a corticosteroid rapidly induced remission, which could be maintained with anakinra monotherapy at a stable dose of 2 mg/kg per day. ⋯ Untoward effects of corticosteroid treatment were mild because prolonged therapy with high-dose corticosteroids could be avoided. These results suggest that early institution of interleukin 1 blockade merits further investigation for the treatment of macrophage activation syndrome and, perhaps, related conditions such as hemophagocytic lymphohistiocytosis.
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Three types of group A streptococcal infections are particularly feared: necrotizing fasciitis, myositis, and streptococcal toxic shock syndrome (TSS). We present 3 cases of necrotizing fasciitis due to Streptococcus pyogenes, one in an immunocompromised patient who had received kidney transplant and 2 healthy patients. Mean age of patients was 52 years (range, 42-67 years), and all 3 were male. ⋯ Aggressive surgical debridement in the first 24 to 48 hours and antibiotic treatment, including penicillin and clindamycin, are the cornerstones in the management of these infections. Adjuvant intravenous immunoglobulin therapy might be useful in case of TSS. Diagnostic and treatment delays are the main causes of mortality in these infections.
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Case Reports
Desquamative interstitial pneumonia as the initial manifestation of systemic sclerosis.
Interstitial lung disease (ILD) is a frequent pulmonary complication of systemic sclerosis (SSc), and nonspecific interstitial pneumonia is the most commonly recognized pattern of lung injury in these patients. In this report, we describe a never-smoker female presenting with Raynaud phenomenon and ILD that demonstrated desquamative interstitial pneumonia (DIP) on surgical lung biopsy. After 8 months, she was diagnosed with pulmonary hypertension at which time clinical examinations and serologic findings established the diagnosis of SSc. This case report expands the spectrum of patterns of ILD seen in association with SSc to include DIP.
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Review Case Reports
Avascular necrosis of the metacarpals in juvenile dermatomyositis.
Avascular necrosis (AVN) of the metacarpal heads is uncommon and has been associated with trauma, systemic lupus erythematosus, and corticosteroid usage. There have been no previous reports of metacarpal AVN described in patients with juvenile dermatomyositis. Descriptions of AVN in juvenile dermatomyositis are rare. We present 2 cases of patients with juvenile dermatomyositis who developed multifocal avascular necrosis after corticosteroid therapy with unusual distribution and review the literature on metacarpal AVN.