Objective: Wegener’s granulomatosis (WG) is a necrotizing vasculitis that mainly affects the respiratory tract and kidneys, but can also affect other systems such as the eye, joints, skin, muscles, nerves, and gastrointestinal tract. Cardiac involvement is traditionally believed to be rare. We report a patient with silent myocardial infarction (MI) and review previously reported cases showing this association. Methods: A Medline database search of cases published between January 1978 and July 2008 both in English and Spanish, reporting silent MI complicating WG, was conducted. Results: We describe a typical patient with WG who had both respiratory and renal involvement and died unexpectedly following a silent MI after a period of clinical improvement induced by treatment with prednisone and cyclophosphamide. We report necropsy findings and the association with 5 additional cases of WG with silent MI reported in the literature. Conclusions: Clinicians should be aware of potential cardiac involvement due to WG. Careful evaluation of each patient, with or without cardiac symptoms, using ECG, echocardiogram, and myocardial enzymes is prudent.
Vasculitis in systemic lupus erythematosus (SLE) has a broad spectrum of clinical manifestations from cutaneous to visceral involvement and its prognosis ranges from mild to life-threatening. We report the case of a previously healthy 17-year-old woman with eight months' history of arthralgias and myalgias. Subsequently, she developed facial and lower limbs edema, and hair loss. Two weeks before admission to a secondary level hospital, she developed fever up to 40°C followed by abdominal pain, rectal bleeding, hematemesis and blisters on both legs, reason for which she was hospitalized. With active bullous SLE with rapidly progressive glomerulonephritis suspected, she was treated with methylprednisolone pulses without response. After one week of treatment, she was transferred to a tertiary level hospital. On admission she presented acute arterial insufficiency of the lower extremities, respiratory failure with apnea, metabolic acidosis and shock; six hours later she died. Autopsy findings showed active diffuse lupus nephritis and diffuse systemic vasculitis that involved vessels from the skin, brain, myocardium, spleen, iliac and renal arteries. In addition, serositis of the small intestine and colon, acute and chronic pericarditis, pericardial effusion and myocarditis were found. Immunologic tests confirmed SLE diagnosis. In this case the fulminant course was the result of SLE high disease activity, visceral vasculitis of several organs and late diagnosis, referral and treatment. Early diagnosis, and opportune referral to the rheumatologist for intensive treatment can improve the outlook in these patients.
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