肾小球肾炎
不确定意义的单克隆抗体病
单克隆
免疫球蛋白A
病理
多发性骨髓瘤
恶性肿瘤
医学
肾病
免疫学
肾
单克隆抗体
肾脏疾病
抗体
免疫球蛋白G
内科学
内分泌学
糖尿病
作者
Marguerite Vignon,C. Cohen,Stanislas Faguer,Laure-Hélène Noël,C. Guilbeau,Marion Rabant,Sarah C. Higgins,Aurélie Hummel,Alexandre Hertig,H. Francois,M. Lequintrec,Eve Vilaine,Bertrand Knebelmann,Jacques Pourrat,Dominique Chauveau,Jean-Michel Goujon,Vincent Javaugue,Guy Touchard,Khalil El Karoui,Frank Bridoux
标识
DOI:10.1016/j.kint.2016.10.026
摘要
Monoclonal gammopathy of renal significance (MGRS) regroups renal disorders caused by a monoclonal immunoglobulin without overt hematological malignancy. MGRS includes tubular disorders, glomerular disorders with organized deposits, and glomerular disorders with non-organized deposits, such as proliferative glomerulonephritis with monoclonal IgG deposits. Since glomerular involvement related to monotypic IgA deposits is poorly described we performed retrospective analysis and defined clinico-biological characteristics, renal pathology, and outcome in 19 referred patients. This analysis allowed distinction between 2 types of glomerulopathies, α-heavy chain deposition disease (5 patients) and glomerulonephritis with monotypic IgA deposits (14 patients) suggestive of IgA-proliferative glomerulonephritis with monoclonal immunoglobulin deposits in 12 cases. Clinicopathologic characteristics of α-heavy chain deposition disease resemble those of the γ-heavy chain disease, except for a higher frequency of extra-capillary proliferation and extra-renal involvement. IgA-proliferative glomerulonephritis with monoclonal immunoglobulin deposits should be differentiated from diseases with polytypic IgA deposits, given distinct clinical, histological, and pathophysiological features. Similarly to IgG-proliferative glomerulonephritis with monoclonal immunoglobulin deposits, overt hematological malignancy was infrequent, but sensitive serum and bone marrow studies revealed a subtle plasma cell proliferation in most patients with IgA-proliferative glomerulonephritis with monoclonal immunoglobulin deposits. Anti-myeloma agents appeared to favorably influence renal prognosis. Thus, potential progression towards symptomatic IgA multiple myeloma suggests that careful hematological follow-up is mandatory. This series expands the spectrum of renal disease in MGRS.
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