Intravenous pulse cyclophosphamide (IPC) was used to treat lupus interstitial lung disease in 2 patients. Vital capacity increased by 67–110% of baseline values with IPC treatment. Total lung capacity and diffusing capacity also improved. Dyspnea and other manifestations of lupus improved dramatically. This is the first report documenting the efficacy of the early use of IPC for acute and chronic interstitial lung disease associated with lupus.

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Hypocomplementemic urticarial vasculitis (HUV) is often misdiagnosed. The response to drug therapy may be unsatisfactory, and immunosuppressive therapy may be associated with significant side effects. A 35-year-old patient whose condition was resistant to cyclophosphamide, corticosteroids, pentoxyphylline, cyproheptadine, sulindac, and colchicine was maintained in remission with dapsone, which may be the drug of choice for HUV. Emphysema has been reported to complicate HUV, but this is the first account of vasculitis-related emphysema with no confounding history of tobacco smoke exposure. The relationship of proteolytic injury to the pulmonary and other manifestations is considered, as is the possible role for dapsone in reducing it.

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