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

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, mass...

Full description

Saved in:
Bibliographic Details
Published in:Rheumatology (Oxford, England) England), 2004-06, Vol.43 (6), p.795-799
Main Authors: Bennett, A. N., Peterson, P., Sangle, S., Hangartner, R., Abbs, I. C., Hughes, G. R. V., D'Cruz, D. P.
Format: Article
Language:English
Subjects:
Acute Kidney Injury - etiology
Adult
Adult onset Still's disease
Biological and medical sciences
Collapsing glomerulopathy
Combined Modality Therapy
Diagnosis, Differential
Diseases of the osteoarticular system
Follow-Up Studies
Humans
Immunoglobulins, Intravenous - therapeutic use
Immunosuppressive Agents - therapeutic use
Inflammatory joint diseases
Intravenous immunoglobulin
Lupus Erythematosus, Systemic - diagnosis
Male
Medical sciences
Mycophenolate mofetil
Mycophenolic Acid - analogs & derivatives
Mycophenolic Acid - therapeutic use
Proteinuria - etiology
Still's Disease, Adult-Onset - complications
Still's Disease, Adult-Onset - diagnosis
Still's Disease, Adult-Onset - therapy
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:1462-0324
1462-0332
DOI:10.1093/rheumatology/keh172