An IgA1-lambda-type monoclonal immunoglobulin deposition disease associated with membranous features in a patient with IgG4-related kidney disease: a case report

Abstract

BackgroundIgG4-related disease (IgG4-RD) is a newly recognized fibroinflammatory condition. The kidney is one of the organs commonly affected by IgG4-RD. Tubulointerstitial nephritis (TIN) is the main feature, and membranous nephropathy (MN) has also been described frequently. In MN, polyclonal immunoglobulins and complements are deposited in granular form along the glomerular basement membranes (GBMs). Unusual cases of monoclonal immunoglobulin deposition disease (MIDD) associated with membranous features have been reported. MIDD is morphologically similar to MN but contains immunoglobulins considered to be derived from single B-cell clone.Case presentationWe describe a 65-year-old man who was referred to our hospital because of hyperproteinaemia, eosinophilia, anaemia, and proteinuria. A renal biopsy demonstrated infiltration of plasma cells and eosinophils in the interstitium, and the ratio of IgG4-positive plasma cells to IgG-positive plasma cells was 55%. The patient was diagnosed as having IgG4-related TIN. Periodic acid methenamine silver staining under light microscopy revealed a bubbling appearance and spike formation in the GBM. On immunofluorescence, the expression of IgG and complements was negative; however, IgA was positively expressed in a granular pattern along the GBM. An IgA subclass analysis revealed a significant deposition of IgA1-lambda (IgA1-λ). Electron microscopy revealed irregular and small non-organized and non-Randall-type granular electron-dense deposits in the GBM that were shaped like snow leopard spots.ConclusionsAfter corticosteroid therapy was initiated, the patient’s eosinophilia remarkably improved and his serum creatinine, IgG, and IgG4 levels decreased to within the normal ranges. However, massive proteinuria persisted. To our knowledge, this is the first reported case of IgG4-related TIN associated with IgA1-λ-type MIDD with membranous features.

Membranous nephropathy with solitary polyclonal IgA deposition: A case report and literature review

“…There are several case reports of monoclonal IgA-type MN or IgA-type MIDD with membranous features [3, 4, 5]. Sethi [3] reported an MN patient with IgA-κ deposits and crescents.…”

“…In this case, the relationship between chronic hepatitis C viral infection and monoclonal gammopathy was considered. Kitazawa et al [5] reported a case of IgA1-λ-type MIDD associated with membranous features. This case also had IgG4-related tubulointerstitial nephritis.…”

“…Biopsy findings that should prompt careful search for SMN include dominant depositions of IgG1/IgG3, IgA, IgM, or C1q, and electron-dense deposits in subendothelial or mesangial locations [1]. To our knowledge, there are several case reports of monoclonal IgA-type MN or IgA-type monoclonal immunoglobulin deposition disease (MIDD) with membranous features [3, 4, 5] and only one case report of MN with solitary polyclonal IgA deposition [6]. Here, we report an additional case of MN with solitary polyclonal IgA deposition.…”

Eosinophilia and Kidney Disease: More than Just an Incidental Finding?

“…For primary glomerulopathies such as MN [131,189,190], IgA nephropathy [191,192,193,194], or FSGS [195,196,197], an association with PBE was mainly found when described in the context of primary eosinophilia-associated diseases like Kimura’s disease, EGPA, IgG4-RKD, or HES.…”

Influence of Hyperproteinemia on Insect Innate Immune Function of the Circulatory System in Bombyx mori

“…Hyperproteinemia is a metabolic disease, which has recently been reported to be associated with an abnormally elevated plasma protein concentration (PPC) as a clinical symptom [1][2][3][4][5]. This hyperproteinemia is commonly exhibited in many important clinical diseases in humans, as well as other mammals (e.g., multiple myeloma [4,6], nephropathy [7][8][9][10], hepatopathy [11,12], and pathogen infections [2,13]). Hyperproteinemia also appears in metabolic acidosis and even clinical plasma therapy or intravenous immunoglobulin therapy [9,[14][15][16].…”