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Rheumatology Advance Access originally published online on March 23, 2004
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Rheumatology 2004; 43: 795-799
Rheumatology Vol. 43 No. 6 © British Society for Rheumatology 2004; all rights reserved

Grand Rounds in Rheumatology

Adult onset Still's disease and collapsing glomerulopathy: successful treatment with intravenous immunoglobulins and mycophenolate mofetil

A. N. Bennett, P. Peterson, S. Sangle, R. Hangartner1, I. C. Abbs2, G. R. V. Hughes and D. P. D'Cruz

The Lupus Research Unit, The Rayne Institute, St Thomas’ Hospital, London, 1 The Department of Histopathology, St Thomas’ Hospital, London, 2 The Department of Nephrology, Guy's and St Thomas’ Hospital, London, UK.

Correspondence to: D. D’Cruz, The Lupus Research Unit, The Rayne Institute, St Thomas’ Hospital, London SE1 7EH, UK. E-mail: david.d'cruz{at}kcl.ac.uk

Abstract

In this Grand Round we present a 32-yr-old African man who became severely ill after a 5-month history of weight loss, pyrexia, arthralgia, sweats and rash. He went on to develop pericarditis, pericardial effusion with tamponade, hepatomegaly with abnormal liver function tests, lymphadenopathy, massive proteinuria and required ventilatory, circulatory and renal support. The differential diagnosis was adult onset Still's disease, systemic lupus erythematosus (SLE), infection and lymphoma. Primary infection and lymphoma were excluded and he was treated, with dramatic success, with intravenous immunoglobulins (IVIG). Subsequent renal biopsy excluded SLE but confirmed collapsing glomerulopathy. The proteinuria improved dramatically following treatment with mycophenolate mofetil. We discuss some of the difficult diagnostic and management issues raised by this patient and the different uses and mechanisms of action of IVIG.

KEY WORDS: Adult onset Still's disease, Collapsing glomerulopathy, Intravenous immunoglobulin, Mycophenolate mofetil.


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