ACR2012 Highlights: Sjögren's Syndrome


Among major reports about Sjögren’s syndrome at the American College of Rheumatology meeting: An encouraging phase 2 trial for belimumab, two studies on severe manifestations, and a new screening test.

Among major reports about Sjgren’s syndrome at the American College of Rheumatology meeting: An encouraging phase 2 trial for belimumab, two studies on severe manifestations, and a new screening test that uses tears. (To read the complete abstract online, click on the abstract number below.)

  The BELISS phase 2 trial of belimumab for primary Sjgren’s syndrome (pSS) had “encouraging” results. Of 30 patients, 19 (63%) reached the primary endpoint at 28 weeks. All patients had anti-SSA/SSB antibodies; 11 had early disease and 15 had systemic complications. The primary endpoint was ≥2 of the following: ≥30% reduction in dryness, fatigue, musculoskeletal pain, or systemic activity; or ≥25% reduction of B cell activation biomarkers; or ≥25% increase in C4. The French and Italian researchers observed “significant” changes in serum IgG, IgA, kappa and gamma free light chain, and rheumatoid factor. There was no significant change in salivary flow or Schirmer test. One patient had pneumococcus meningitis, and recovered completely. In a secondary endpoint at 52 weeks, non-malignant parotid swelling improved, which was reflected in lowering the ESSDAI score from 7 to 4. (Abstract #2555 and 2189)

•   Interstitial nephritis has a favorable prognosis, but glomerulonephritis has high risk for lymphoma and poor survival. Of 715 patients with pSS, 35 (5%) had renal involvement on biopsy, 12 (34%) had interstitial nephritis, 18 (51 %) had glomerulonephritis, and 5 (14%) had both. The primary outcomes included death, hemodialysis, lymphoma and chronic renal failure. There were 9 deaths and 9 cases of lymphoma. The glomerulonephritis group had 8/9 (89%) of the deaths and 8/9 (89%) of the lymphomas (Abstract #2552)

•   20% of pSS patients have severe systemic manifestations, at least among those treated at 4 Italian reference centers. The case records of 1115 patients (1067 female) with a diagnosis of pSS  showed that the most common clinical manifestations were xerostomia (93%), xerophthalmia (95%), articular involvement (62%), hematological involvement (32%) and salivary gland enlargement (31%). Severe extra-glandular manifestations included active synovitis (11%), axonal sensory-motor neuropathy (2%), diffuse purpura or ulcers (6%), renal involvement (0.7%), myositis (0.5%), cerebral vasculitis (0.5%), and transverse myelitis (0.2%). Finally, 50 (4.5%) had non-Hodgkins lymphoma. Patients with more severe disease were younger and had more serologic markets. Patients with elevated cryoglobulins had an odds ratio of 7.6 for severe extraglandular involvement. (Abstract #2185)

•   Cathepsin S collected in Shirmer’s tests is a simple marker for Sjgren’s syndrome. Shirmer’s tests were given to 47 patients with Sjgren’s syndrome, as well as patients with only rheumatoid arthritis, systemic lupus erythematosus, other autoimmune diseases, dry eyes, and blepharitis. The test strips were placed on ice and analyzed for cathepsin S using commercial assay kits. Cathepsin S activity was 2 to 3 times as high in Sjgren’s syndrome as the other groups. Cathepsin S is a protease in the lysosome that helps MHC class II molecules present antigens. (Abstract #2194)

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