Abstract: FR-PO667
Remission of Secondary Membranous Nephropathy From Sjogren Syndrome After Treatment With Hydroxychloroquine
Session Information
- Glomerular Diseases: Clinical, Outcomes, Trials - II
November 04, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1303 Glomerular Diseases: Clinical‚ Outcomes‚ and Trials
Authors
- Al-Tuhafy, Dina Ra, NYU Langone Hospital - Long Island, Mineola, New York, United States
- Ancion, Jean Herold, NYU Langone Hospital - Long Island, Mineola, New York, United States
- Sajid, Saira, NYU Langone Hospital - Long Island, Mineola, New York, United States
- Hysi, Katerina, NYU Langone Hospital - Long Island, Mineola, New York, United States
- Drakakis, James, NYU Langone Hospital - Long Island, Mineola, New York, United States
Introduction
Membranous nephropathy (MN) is a common cause of proteinuria. It can be divided into primary and secondary forms. The primary form is characterized by nephrotic syndrome and accounts for 70% of cases of MN. The other 30% may be secondary, attributed to underlying causes, such as infections, drugs, malignancies, or autoimmune diseases. The treatment of secondary MN is targeted to the etiologic cause and when effectively administered, can lead to a remission or cure of MN. While treatment options are well defined for underlying SLE or RA in the setting of MN, those for Sjogren's are much less so.
Case Description
69 year old female with PMHx of Sjogren's syndrome (sicca symptoms, +ANA/SS-B), initially presented with anasarca and proteinuria quantified at 7 g/day. Kidney biopsy was performed showing membranous nephropathy, with negative staining for PLA2R. In addition, serum PLA2R Ab neg, favoring secondary form (likely Sjogren's related). At first immunosuppression was held. Renal function remained stable (serum creatinine 1.3 mg/dL). After several months, due to lack of improvement, she received two doses of 1000 mg of Rituximab. Proteinuria reached nadir of 3.5 g/g, but 6 months after the infusion, rose back up to 8 g/g. At this point, she started Hydroxycholoroquine 400 mg per day. Improvement in proteinuria followed after 6 months of this therapy (down to 3.7 g/g). After an additional 8 months, the urine protein quantification was 561 mg/g and by 1 year later down to <200 mg/g.
Discussion
Renal involvement in Sjogren's syndrome is typically rare, only affecting <10% of patients. When present, this is usually in the form of tubulointerstitial nephritis and renal tubular acidosis. However, MN (secondary) has also been reported as a cause of renal disease in patients with Sjogren's, as high as in 36% in one cohort. Those patients with glomerular involvement are said to carry a worse prognosis. In fact, little data is available about the effectivness of steroids or other immunosuppression to slow progression of renal disease and improve proteinuria. Our case is illustrative in that it showed a complete remission of secondary MN from Sjogren's syndrome only after treatment with Hydroxychloriquine. This strategy was enacted only after Rituximab proved ineffective.