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Ultrasonographic Evaluation of Salivary Glands for Sjogren's Syndrome: Diagnostic and Monitoring Insights
Published on: October 13, 2023
[Membranous glomerulonephritis during primary Gougerot-Sjögren syndrome]
R Laraki1, D Chauveau, L H Noel
1Cabinet de médecine interne, Casablanca, Maroc.
Introduction:
Glomerulonephritis, mainly membranoproliferative or membranous (MG), is observed much less often than interstitial involvement in Sjögren's syndrome (SS).
Case:
We report a case of MG revealed by thrombosis of the inferior vena cava and of a renal vein in a 40-year-old woman with primary SS, which began with polyarthritis, immune-type lymphadenopathy, and Hashimoto thyroiditis and did not include obvious sicca syndrome. After failure of moderate-dose steroids and then azathioprine, each over separate 9-month periods, the MG responded well within a few weeks to monthly alternation of methylprednisolone and oral cyclophosphamide for 6 months.
Discussion:
SS may be an underestimated cause of glomerulonephritis, especially membranoproliferative and membranous glomerulonephritis. They should be considered even in the absence of obvious sicca syndrome. Although the prognosis is usually good, renal insufficiency can occur. In cases of MG, if moderate-dose steroids fail, monthly alternation of methylprednisolone and cyclophosphamide for 6 months appears effective and well tolerated, with a low risk of carcinogenicity.
Insights
Sjögren's syndrome (SS) can cause kidney disease like membranous glomerulonephritis (MG), even without dry eyes or mouth. A combination therapy of methylprednisolone and cyclophosphamide effectively treated MG in an SS patient when steroids failed.
Area of Science:
- Nephrology
- Rheumatology
- Immunology
Background:
- Sjögren's syndrome (SS) is primarily associated with interstitial kidney disease.
- Glomerulonephritis, specifically membranous glomerulonephritis (MG), is a less common renal manifestation of SS.
Observation:
- A 40-year-old woman with primary SS presented with inferior vena cava and renal vein thrombosis, indicative of underlying MG.
- Her SS symptoms included polyarthritis, lymphadenopathy, and Hashimoto thyroiditis, but notably lacked overt sicca syndrome.
Findings:
- Standard treatments with moderate-dose steroids and azathioprine were ineffective for her MG.
- A treatment regimen involving monthly alternating methylprednisolone and oral cyclophosphamide led to rapid and positive response within weeks.
Implications:
- SS should be considered in the differential diagnosis of glomerulonephritis, particularly MG, even in the absence of classic sicca symptoms.
- The combination therapy of alternating methylprednisolone and cyclophosphamide offers an effective and well-tolerated treatment option for refractory MG in SS patients.
- Early consideration and appropriate management of SS-associated glomerulonephritis are crucial to prevent renal insufficiency.
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