Significance of Crescentic Glomeruli in Acute Kidney Injury with Rheumatoid Arthritis.

Crescentic glomerulonephritis (GN) without immune reactants or deposits (referred to as pauci-immune) is typically characterized by the presence of anti-neutrophilic cytoplasmic antibodies (ANCA). While ANCA-negative patients might be expected to have a more benign course, they often have poor renal outcomes, especially without treatment with steroids and immune-modulating therapy. Pauci-immune crescentic GN can also co-exist with other autoimmune conditions, including rheumatoid arthritis (RA). Here, we describe an ANCA-negative patient with RA who developed dialysis-requiring acute kidney injury (AKI) with findings consistent with focal pauci-immune crescentic GN (i.e., no IgG or immune complex on kidney biopsy). Coexistent conditions included Klebsiella sepsis attributed to pneumonia, rhabdomyolysis, leukocytoclastic immune-mediated skin vasculitis, and positive ANA. He had spontaneous improvement in renal function without immunosuppressive therapy. This crescentic GN was not associated with poor renal outcome as AKI resolved with supportive care and treatment of his infection. The AKI was likely multifactorial with co-existing acute tubular necrosis in the setting of Kebsiella sepsis and rhabdomyolysis, and the crescentic GN was felt more likely to be related to the infection rather than having a primary role. This case highlights the importance of viewing crescentic GN in the context of the clinical picture, as it may not always lead to the need of aggressive immune suppression and is not a universally poor prognostic kidney finding. However, these cases do warrant close follow-up as our patient had recurrent RA disease manifestations over the next 2 years that eventually led to his death from severe pulmonary hypertension.

Successful management of proliferative glomerulonephritis with monoclonal immune deposits with combined immunosuppressive therapy.

Proliferative glomerulonephritis with monoclonal immune deposits (PGNMID) is a newly described entity characterised by monoclonal IgG deposits consisting of single light chain isotype and single heavy chain subtype (IgG1-4) in the kidneys. We are presenting two cases of patients who presented with acute kidney injury and worsening proteinuria. Kidney biopsy showed membranoproliferative pattern. Special staining for subclass of IgG showed monoclonal IgG3-kappa (case 1) and IgG1-kappa deposits (case 2) suggestive of PGNMID. Workup for underlying infection, malignancy, monoclonal gammopathy was negative. Since pathogenesis of PGNMID involves clonal proliferation of B-cells, we treated both patients with rituximab along with steroids that led to improvement of proteinuria and renal function. We also reviewed current literature to assess efficacy of rituximab in treatment of PGNMID. However, a larger pool of patients and a longer follow-up period is required to establish a role of rituximab and steroids in the treatment of this disease entity.