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ResumoO lúpus eritematoso sistêmico (LES) é uma doença autoimune multissistêmica que tem como uma das manifestações mais marcantes a nefrite. Apesar de poder coexistir com outras doenças autoimunes e determinar a predisposição a diversas complicações infecciosas, o LES raramente é descrito em associação a nefropatias de etiologia não lúpica. Relatamos o caso da rara associação entre LES e nefropatia por IgA (NIgA) primária, a glomerulopatia primária mais frequente na população mundial. A paciente foi diagnosticada com LES pela ocorrência de eritema malar, alopecia, derrame pleural, proteinúria, pancitopenia, FAN 1:1.280 padrão nuclear pontilhado fino e anticardiolipina IgM 280 U/mL. A biópsia renal revelou hipercelularidade mesangial com depósitos isolados de IgA, compatível com NIgA primária. Foi tratada com antimalárico, prednisona e inibidor da enzima conversora de angiotensina e apresentou boa evolução. Por consistirem em doenças relativamente frequentes, a coexistência de LES e NIgA pode ser de fato um achado incomum por motivos desconhecidos ou uma condição subdiagnosticada. Este relato atenta para a importância da distinção entre a atividade de doença renal do LES e nefropatias não lúpicas, em especial a NIgA, definição que tem implicações importantes sobre o prognóstico renal e regimes terapêuticos a serem adotados em curto e longo prazo.AbstractSystemic erythematosus lupus (SLE) is a multisystemic autoimmune disease which has nephritis as one of the most striking manifestations. Although it can coexist with other autoimmune diseases, and determine the predisposition to various infectious complications, SLE is rarely described in association with non‐lupus nephropathies etiologies. We report the rare association of SLE and primary IgA nephropathy (IgAN), the most frequent primary glomerulopathy in the world population. The patient was diagnosed with SLE due to the occurrence of malar rash, alopecia, pleural effusion, proteinuria, ANA 1: 1,280, nuclear fine speckled pattern, and anticardiolipin IgM and 280 U/mL. Renal biopsy revealed mesangial hypercellularity with isolated IgA deposits, consistent with primary IgAN. It was treated with antimalarial drug, prednisone and inhibitor of angiotensin converting enzyme, showing good progress. Since they are relatively common diseases, the coexistence of SLE and IgAN may in fact be an uncommon finding for unknown reasons or an underdiagnosed condition. This report focus on the importance of the distinction between the activity of renal disease in SLE and non‐SLE nephropathy, especially IgAN, a definition that has important implications on renal prognosis and therapeutic regimens to be adopted in the short and long term.

Systemic erythematosus lupus (SLE) is a multisystemic autoimmune disease which has nephritis as one of the most striking manifestations. Although it can coexist with other autoimmune diseases, and determine the predisposition to various infectious complications, SLE is rarely described in association with non-lupus nephropathies etiologies. We report the rare association of SLE and primary IgA nephropathy (IgAN), the most frequent primary glomerulopathy in the world population. The patient was diagnosed with SLE due to the occurrence of malar rash, alopecia, pleural effusion, proteinuria, ANA 1: 1280, nuclear fine speckled pattern, and anticardiolipin IgM and 280U/mL. Renal biopsy revealed mesangial hypercellularity with isolated IgA deposits, consistent with primary IgAN. It was treated with antimalarial drug, prednisone and inhibitor of angiotensin converting enzyme, showing good progress. Since they are relatively common diseases, the coexistence of SLE and IgAN may in fact be an uncommon finding for unknown reasons or an underdiagnosed condition. This report focus on the importance of the distinction between the activity of renal disease in SLE and non-SLE nephropathy, especially IgAN, a definition that has important implications on renal prognosis and therapeutic regimens to be adopted in both the short and long terms.