• Jungyoon Choi, Tae Won Lee, Eunjin Bae, Ha Nee Jang, Hyo Jung An, Se-Ho Chang, and Dong Jun Park.
• Department of Internal Medicine, Gyeongsang National University Changwon Hospital, Changwon, South Korea.
• Medicine (Baltimore). 2023 Jun 2; 102 (22): e33892e33892.

RationaleOnly 1 case of IgA nephropathy (IgAN) with minimal change disease (MCD) associated with primary Sjögren's syndrome (SS) has been reported. We additionally describe IgAN with MCD associated with primary SS.Patient ConcernsA 80-year-old woman visited our hospital complaining of generalized edema that had started 4 weeks prior. She reported a sense of thirst and dry eye for the last 5 years.DiagnosesHer initial laboratory findings were compatible with nephrotic syndrome; both the antinuclear antibody (1:80) and anti-SS-A (Ro) antibody (200 U/mL) tests were positive. A salivary gland scan revealed markedly decreased uptake for both the parotid and submandibular glands. The Schirmer test was positive. The random urine protein/creatinine ratio was 10 mg/mg. Renal biopsy was compatible with IgAN with superimposed MCD.InterventionsFurosemide was intravenously administered with intermittent albumin infusion for her edema control. She was started on prednisone 40mg daily for 6 weeks, which was tapered to 5 mg for another 6 months after starting prednisolone.OutcomesOver the next 6 months, her edema improved and the proteinuria decreased significantly.LessonsPhysician should suspect IgA with MCD when patient with SS clinically showed nephrotic syndrome, and perform renal biopsy for pathologically diagnosis and appropriate treatment.Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.

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