Sjögren’s syndrome complicated with membranous nephropathy, a cause or coincidence?

Abstract

Background Sjögren's syndrome (SS) has been a well‐documented cause of secondary membranous nephropathy (MN); however, the prevalence is quite low. Since primary MN is also a common disease in middle age, whether MN is secondary to SS or just coincidence remains uncertain. The detection of phospholipase A2 receptor (PLA2R), which is most often positive in idiopathic MN, has been rarely reported in such cases. Methods We retrospectively studied 13 cases diagnosed with MN and SS in Huashan Hospital between 2009 and 2020, and performed PLA2R detection. We also review the literature by searching in the PubMed database. Results Among the 13 patients, 8 were found to be PLA2R‐positive and 5 were negative. Nine patients were female. All but 1 patients had normal renal function at the time of biopsy. All patients showed positive anti‐nuclear antibody and anti‐SSA. Two of the 8 PLA2R‐positive patients showed positive anti‐SSB and 1/8 had mild hypocomplementemia, while all 5 PLA2R‐negative patients had positive anti‐SSB and 3 showed hypocomplementemia. Renal biopsy revealed focal MN and markedly mesangial hyperplasia in PLA2R‐negative patients. Mesangial electron deposits were observed in 1 PLA2R‐positive patient in small amounts, and in 3 PLA2R‐negative patients with 2 in large amounts. During follow‐up, 2 patients in the PLA2R‐negative group presented with progressively decreasing serum complement levels, and another one was diagnosed with systemic lupus erythematosus (SLE). Conclusions In patients with both MN and SS, PLA2R‐negative MN should be considered as a secondary form. Careful screen for SLE is necessary in these patients during follow‐up.

Incidence and Clinical Pattern of Mixed Connective Tissue Disease in Sudanese Patients at Omdurman Military Hospital: Hospital-Based Study

“…A high titer speckled ANA whose fine specificity is anti-U1 RNP is the diagnostic serological finding in MCTD. 10 The management of MCTD generally rests upon the known effectiveness of specific therapies for similar problems seen in SLE, Scleroderma, or Polymyositis. 10 , 11…”

“… 10 The management of MCTD generally rests upon the known effectiveness of specific therapies for similar problems seen in SLE, Scleroderma, or Polymyositis. 10 , 11…”

A Diagnostic and Therapeutic Dilemma Concerning Exostosin 1/Exostosin 2-associated Lupus-like Membranous Nephropathy with Positive Antinuclear Antibody in an Elderly Man with Various Immune Abnormalities

“…Wang et al reported that membranous LNs accounted for 96% of the total EXT1/EXT2-positive patients with secondary MN, and few EXT1/EXT2-associated MN cases were related to Hashimoto thyroiditis or pSS; furthermore, they may progress to typical SLE over the course of the disease ( 23 ). Chen et al reported that, in patients with both MN and pSS, PLA2R-positive MN is more likely to be a coincident primary MN than PLA2R-negative MN, and PLA2R-negative MN should be considered as secondary MN, with careful screening for SLE necessary during follow-up ( 27 ). The same might be true in the present patient with both EXT1/EXT2-associated MN and SS.…”

The Spectrum of Extraglandular Manifestations in Primary Sjögren’s Syndrome

“…In type I distal RTA, alkaline products are prescribed for acidemia, while in persistent hypokalemia, supplemental potassium is required [ 171 ]. In membranoproliferative glomerulonephritis, and cryoglobulinemic vasculitis, specific treatment with immunosuppressants is recommended [ 174 ]. In severe cases, plasma exchange may be an option [ 171 ].…”