J Nephropathol. 2019;8(1):11-11.
doi: 10.15171/jnp.2019.11
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  PDF Download: 93

Case Report

Microscopic polyangiitis and systemic lupus erythematosus overlap syndrome; an unusual presentation

Fahd Touzani 1,2 * , Agnieszka Pozdzik 1, Concetta Catalano 1,2

1 Nephrology and Dialysis Department, Centre Hospitalier Universitaire, Hospital Brugmann, Brussels, Belgium
2 Faculty of Medicine, Université Libre de Bruxelles (ULB), Brussels, Belgium


Background: Lupus nephritis (LN) is characterized by glomerular immune-complex deposits usually in a full house (FH) pattern. In contrast, ANCA-associated glomerulonephritis (GN) is typically pauci-immune GN. Patients fulfilling both systemic lupus erythematosus (SLE) and ANCAassociated vasculitis (AAV) classification criteria defining "SLE/AAV overlap syndrome" have been rarely reported. However, FH nephropathy (FHN) without overt SLE at presentation is described and considering an overlap syndrome in this situation is challenging.

Case Presentation: A 40-year-old man presented to the emergency because of hemoptysis and macroscopic haematuria. We found acute kidney injury (plasma creatinine 5.16 mg/dL, N: 0.72- 1.17) with an active urinary sediment (proteinuria 3+, haematuria 3+). Chest computerized tomography showed intra-alveolar diffuse haemorrhage confirming pulmonary-renal syndrome. High titers of anti-MPO ANCA in the absence of ANA strongly correlated with microscopic polyangiitis. Kidney biopsy confirmed crescentic GN, however FH immunofluorescence (IF) pattern suggested a LN (class IV). Despite plasma exchanges associated to steroids and rituximab, kidney function declined and haemodialysis was initiated. During 12 months of follow-up on azathioprine-low dose steroids treatment, he remains still asymptomatic. However, considering the appearance of antinuclear antibodies (ANA), presence of antiphospholipid antibodies and low complement level, we considered the diagnosis of SLE/AAV overlap syndrome.

Conclusions: SLE/AAV overlap syndrome is a very rare condition and has a very poor kidney function outcome. We underline that the patients with AAV and FHN require a regular screening for clinical and biological SLE criteria, even in their absence at initial presentation as it could develop later.

Implication for health policy/practice/research/medical education:

Lupus-like nephropathy associated to AAV has a very poor kidney outcome. The delayed appearance of ANA in association to other immunological SLE parameters correlated with SLE/AAV overlap syndrome diagnosis, we propose a regular screening for clinical and biological SLE criteria even in their absence at initial presentation.

Please cite this paper as: Touzani F, Catalano C, Pozdzik A. Microscopic polyangiitis and systemic lupus erythematosus overlap syndrome; an unusual presentation. J Nephropathol. 2019;8(1):e11. DOI: 10.15171/jnp.2019.11.

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Submitted: 07 Apr 2018
Accepted: 09 Jun 2018
First published online: 12 Jul 2018
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