ATYPICAL ANTI-GBM DISEASE IN ASSOCIATION WITH SYSTEMIC MELIOIDOSIS.
JANELLE PRUNSTER1, MAEVE O’CONNOR1, ROBERT HAND1, ANOUSHKA KRISHNAN1, HEMANT KULKARNI1, RAJALINGAM SINNIAH2, SZE ANN WOON1
1Royal Perth Hospital, Perth, Western Australia
2Fiona Stanley Hospital, Perth, Western Australia
Abstract
Background:
Anti-glomerular basement membrane (anti-GBM) disease is an autoimmune vasculitic disease affecting glomerular and pulmonary capillaries and is due to antibodies directed against basement membrane antigens. Atypical, or seronegative anti-GBM disease accounts for approximately 10% of cases. Association between anti-GBM disease and infections have been reported. Here we report the first case of atypical anti-GBM disease associated with systemic melioidosis.
Case Report:
A 38 year-old male from the Kimberley region was transferred to a Perth tertiary hospital after presenting locally with intermittent macroscopic haematuria and haemoptysis on a background of six months of fatigue, fevers and weight loss. He was a smoker and had exposure to soil and brackish water. He had an acute kidney injury with a peak creatinine of 414 umol/L and haemoproteinuria, suggestive of a rapidly progressive glomerulonephritis (RPGN). GN screen was negative for anti-GBM and Anti-Neutrophil Cytoplasmic Antibody (ANCA). Renal biopsy demonstrated a crescentic GN (fibrocellular/cellular crescents) with linear GBM deposits of IgG and C3. Septic screen isolated Burkholderia pseudomallei in blood and urine and a prostatic abscess was demonstrated on imaging. He was treated with intravenous then oral corticosteroids for the anti-GBM disease and meropenem and oral tetracycline for melioidosis. Plasma exchange was not done because he was seronegative for anti-GBM. He did not require haemodialysis. The prostatic abscess was drained and after two weeks of antimicrobial treatment, oral cyclophosphamide was added. At three months follow-up his creatinine is 253 umol/L and repeat serological testing has remained negative for anti-GBM antibody.
Conclusions:
There is association between anti-GBM disease and infection. Infection causing exposure of usually sequestered GBM antigens or molecular mimicry are biologically plausible causes of this association.
Biography
Janelle Prunster is a third year Advanced Trainee based in Perth.