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Membranous nephropathy in a patient with Sjögren's disease
Kubra Kaynar1, Şeymanur Serdar Kılıçaslan2, Sevdegül Mungan3
1Department of Nephrology, School of Medicine, Karadeniz Technical University, Trabzon.
Abstract:
Sjögren's disease (SjD) was first described in a middle-aged female patient with chronic rheumatism in 1930. Membranous nephropathy (MN) is the most commonly identified type of glomerulonephritis in older adults with nephrotic syndrome. One of the autoimmune diseases that causes secondary MN is SjD. A 68-year-old female patient with a medical history of 25 years of hypertension, 9 years of SjD, depressive mood disorder, and intracoronary stent placement applied with peripheral edema. Hypoalbuminemia, hypothyroidism, hematuria, proteinuria, and albuminuria were also detected. In the autoantibody panel, antinuclear antibodies, anti-Ro-52 antibody, anti-Ro/SS-related antigen A antibody, and anticentromere antibody were positive. Kidney biopsy revealed MN. Anti-phospholipase A2 receptor antibody was negative. Methylprednisolone, cyclosporine, hydroxychloroquine, nifedipine, metoprolol, valsartan, L-thyroxine, acetylsalicylic acid, artificial tear drops, and fluoxetine were administered. Partial remission was detected in the first month of treatment. However, the patient, who had all vaccinations, developed swine flu infection and subsequently widespread candidiasis, and despite amphotericin B treatment and discontinuation of immunosuppressives, died in the fifth month due to septic shock. Anti-PLA2R antibody negative MN is one of the kidney manifestations of SjD. The poor prognosis of our patient was due to high SjD disease activity and severe infectious complications, which are independent risk factors for overall mortality.
Insights
Sjögren's disease can cause secondary membranous nephropathy, even when anti-PLA2R antibodies are negative. This case highlights how active Sjögren's disease and infections can lead to poor outcomes in kidney patients.
Area of Science:
- Nephrology
- Rheumatology
- Immunology
Background:
- Sjögren's disease (SjD) is an autoimmune disorder.
- Membranous nephropathy (MN) is a common cause of nephrotic syndrome in adults.
- SjD can lead to secondary MN, particularly in anti-phospholipase A2 receptor (anti-PLA2R) antibody-negative cases.
Purpose of the Study:
- To report a case of anti-PLA2R antibody-negative MN secondary to Sjögren's disease.
- To discuss the clinical presentation, management, and outcome of this rare complication.
- To emphasize the role of disease activity and infections in prognosis.
Main Methods:
- Case report of a 68-year-old female patient with SjD and peripheral edema.
- Diagnostic workup included autoantibody testing and kidney biopsy.
- Treatment involved immunosuppressants, supportive care, and management of infections.
Main Results:
- Kidney biopsy confirmed MN, with negative anti-PLA2R antibodies.
- The patient experienced partial remission initially but later developed severe infections (swine flu, candidiasis).
- Despite treatment, the patient succumbed to septic shock, attributed to high SjD activity and infectious complications.
Conclusions:
- Anti-PLA2R antibody-negative MN is a recognized kidney manifestation of Sjögren's disease.
- High disease activity in SjD and severe infections are significant risk factors for mortality.
- Prompt diagnosis and management of both kidney disease and infections are crucial for improving outcomes.
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