Bronchiolitis obliterans with organizing pneumonia (BOOP) heralding anti-Jo-1-positive polymyositis.

We report a 51-year-old man who presented with 3 weeks of polyarthritis with fever, nonproductive cough, bibasilar crackles, tachypnea, and hypoxia. Initial laboratory data showed an increased erythrocyte sedimentation rate, rheumatoid factor, and anti-Jo-1 antibody. Imaging studies showed bilateral lower lobe infiltrates of the lung. A transbronchial biopsy specimen revealed characteristic findings for bronchiolitis obliterans organizing pneumonia (BOOP). About 6 months later, he developed profound proximal muscle weakness with a dramatic increase in creatine phosphokinase and aldolase and a further elevation of anti-Jo-1. Muscle biopsy specimen findings were consistent with polymyositis.This represents an unusual case in which BOOP occurred at the onset of an illness initially suggestive of rheumatoid arthritis (RA). The anti-Jo-1-positivity led to close follow up and later discovery of evolution into polymyositis. BOOP can be an early feature of polymyositis as well as RA.

Reversal of the vasospastic component of lupus vasculopathy by infusion of prostaglandin E1.

The responses of 3 patients with systemic lupus erythematosus (SLE) and progressive digital ischemia to intravenous prostaglandin E1 (PGE1) were studied prospectively in an open 3-day trial. All patients were unresponsive to corticosteroids, one had vasculitis proven by biopsy. Digital ischemia diminished in all 3 patients. In one patient, angiograms documented reappearance of a previously obstructed deep palmar arch. Vasospasm plays a role in the outcome of SLE vasculitis even in the absence of Raynaud's phenomenon. As suggested by animal models of necrotizing and leukocytoclastic vasculitis, and by case reports, intravenous PGE1 may be a relatively nontoxic, adjunctive treatment for vasculitis.

Variants/subsets of SLE.

Although systemic lupus erythematosus can be considered a single diagnostic entity, a number of clinical subsets have been described including late-onset lupus, drug-induced lupus, neonatal lupus, discoid lupus, and subacute cutaneous lupus.