Fulminant systemic vasculitis in systemic lupus erythematosus. Case report and review of the literature

Abstract

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.

Lupus Aortitis Successfully Treated with Moderate-dose Glucocorticoids: A Case Report and Review of the Literature

“…Highdose glucocorticoids have been recommended as the initial treatment for Takayasu's arteritis or giant cell arteritis, which are major types of aortitis (34,35). However, fatal cases of lupus aortitis without aortic dissection or aortic aneurysm despite high-dose glucocorticoid administration have been reported (25,26). Since heterogeneous outcomes have been reported and there have been no controlled trials, the need for high-dose glucocorticoids as the initial treatment for lupus aortitis is unclear.…”

Higher Acid-Base Imbalance Associated with Respiratory Failure Could Decrease the Survival of Patients with Scrub Typhus during Intensive Care Unit Stay: A Gene Set Enrichment Analysis

“…In other words, the infection-associated injury could induce tissue acidosis, which is reflected in ABI as base excess or base deficit [36]. Other studies have reported scrub typhus-induced vasculitis to be related to ABI [37,38,39,40,41,42].…”

What is lupus vasculitis? A case with diffuse alveolar hemorrhage

“…Cutaneous vasculitis often responds to antimalarials (hydroxychloroquine), dapsone or thalidomide. Short courses of corticosteroids may be used if a rapid response is desired [45]. For visceral involvement, intravenous methylprednisolone pulse therapy or high-dose prednisone have been reported to be useful [46], and has been administered along with other immunosuppressants, mainly cyclophosphamide or rituximab as induction therapy, and varying regimens of azathioprine, mycophenolate mofetil, and hydroxychloroquine used for maintenance [47][48][49].…”