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Review : Grand Rounds from International lupus centres Severe reversible cardiomyopathy associated with systemic vasculitis in primary Sjögren's syndrome

Department of Clinical Immunology, Bnai Zion Medical Center and the Bruce Rappaport Faculty of Medicine

D. Keren

Department of Medicine A, Bnai Zion Medical Center and the Bruce Rappaport Faculty of Medicine

N. Elias

Department of Medicine A, Bnai Zion Medical Center and the Bruce Rappaport Faculty of Medicine

JE Naschitz

Department of Medicine A, Bnai Zion Medical Center and the Bruce Rappaport Faculty of Medicine

E. Toubi

Department of Clinical Immunology, Bnai Zion Medical Center and the Bruce Rappaport Faculty of Medicine

I. Misselevich

Department of Pathology, Bnai Zion Medical Center and the Bruce Rappaport Faculty of Medicine, Technion-Israel, Institute of Technology, Haifa, Israel

D. Yeshurun

Department of Medicine A, Bnai Zion Medical Center and the Bruce Rappaport Faculty of Medicine

A 40y old woman with primary Sjögren's syndrome developed elevated purpura, peripheral neuropathy, muscular tenderness, abdominal pain, heart failure, and convulsive spells. The hallmarks of this disease were high titers of anti-Ro antibodies and low complement levels in the serum, leukocytoclastic small vessel vasculitis in the cutaneous biopsy specimen, and a life threatening clinical course. Echocardiography revealed left ventricular hypokinesis with low ejection fraction, which is unlike the more common features of cardiomyopathy complicating Sjögren's syndrome. The rapidly deteriorating heart failure and other systemic complications remitted on pulse corticosteroid and cyclophosphamide therapy. The pathogeneis of heart failure, which appeared concurrently with vasculitis and was reversed on immunosuppressive therapy, is explained in the context of the systemic disease. Leukocytoclastic vasculitis might be at the origin of this rare variant of acute, severe but reversible cardiomyopathy in pSS.

Key Words: primary Sjögren's syndrome • systemic vasculitis • left heart failure

Lupus, Vol. 6, No. 6, 505-508 (1997)
DOI: 10.1177/096120339700600605


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