American College of Rheumatology classification criteria for Sjögren's syndrome: a data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance cohort.

OBJECTIVE: We propose new classification criteria for Sjögren's syndrome (SS), which are needed considering the emergence of biologic agents as potential treatments and their associated comorbidity. These criteria target individuals with signs/symptoms suggestive of SS. METHODS: Criteria are based on expert opinion elicited using the nominal group technique and analyses of data from the Sjögren's International Collaborative Clinical Alliance. Preliminary criteria validation included comparisons with classifications based on the American­European Consensus Group (AECG) criteria, a model-based "gold standard"obtained from latent class analysis (LCA) of data from a range of diagnostic tests, and a comparison with cases and controls collected from sources external to the population used for criteria development. RESULTS: Validation results indicate high levels of sensitivity and specificity for the criteria. Case definition requires at least 2 of the following 3: 1) positive serum anti-SSA and/or anti-SSB or (positive rheumatoid factor and antinuclear antibody titer >1:320), 2) ocular staining score >3, or 3) presence of focal lymphocytic sialadenitis with a focus score >1 focus/4 mm2 in labial salivary gland biopsy samples. Observed agreement with the AECG criteria is high when these are applied using all objective tests. However, AECG classification based on allowable substitutions of symptoms for objective tests results in poor agreement with the proposed and LCA-derived classifications. CONCLUSION: These classification criteria developed from registry data collected using standardized measures are based on objective tests. Validation indicates improved classification performance relative to existing alternatives, making them more suitable for application in situations where misclassification may present health risks.

Inclusion body myositis in connective tissue disorders: case report and review of the literature.

We report a patient with systemic lupus erythematosus (SLE) and secondary Sjögren's syndrome (SS) who developed inclusion body myositis (IBM) which, contrary to the typical presentation of this disorder, was symmetrical in nature although the diagnosis was only made after electron microscopy was performed. Therapy with increased doses of methotrexate proved to be beneficial, with the patient having full recovery after 8 months of therapy. It appears that a subset of IBM may be related to autoimmune disorders, an issue that was disputed in the past, and these patients may have a better prognosis than typical IBM patients. This is the first case report of IBM in a patient who had the dual diagnosis of SLE and SS.

Corneal melt as the initial presentation of primary Sjögren's syndrome.

Corneal melting is a rare complication of S ogren's syndrome (SS). Previously reported cases of corneal ulceration occurred in patients with established SS, usually secondary to RA. We describe the first case of corneal ulceration with stromal melting as the initial presentation of primary SS. A 79-year-old man without prior sicca symptoms developed a large sterile corneal ulcer that required extensive treatment over several months with ocular lubricants, systemic immunosuppressives, and surgical repair. Evaluation for an underlying connective tissue disease revealed positive antinuclear antibodies (1:640 speckled) and anti-SSA antibody. A lip biopsy established the diagnosis of SS. Ulceration later occurred in the contralateral eye. Two years after the last corneal ulcer and no longer taking prednisone, the patient's ocular disease remained quiescent taking azathioprine 175 mg and hydroxychloroquine 400 mg daily. This case highlights the potential for primary SS to present with serious ocular complications despite lack of a priori sicca symptoms, as well as the importance of immunosuppressive therapy in the treatment of this complication.

Scintigraphic features of chronic sialadenitis and Sjögren's syndrome: a comparison.

An abnormal salivary scintigram is an accepted objective criterion in the diagnosis of primary and secondary Sjögren's syndrome, an immune-mediated disorder characterized by xerostomia and kerato-conjunctivitis sicca. However, chronic sialadenitis constitutes a major differential diagnostic consideration in the xerostomic population. We investigated 39 cases of biopsy-confirmed chronic sialadenitis and 152 individuals with first- or second-degree Sjögren's syndrome, according to international classification criteria. We analysed scintigraphic defects in terms of glands per patient, distribution patterns, kinetics and severity. Relative to Sjögren's syndrome, chronic sialadenitis showed significantly fewer defective glands per patient, less frequent dual parotid-submandibular defects, fewer combined deficits of uptake and discharge, and milder uptake failure. No statistically significant differences were found in the frequency of single gland abnormality, predilection for submandibular involvement, and respective proportions of uptake-only and discharge-only defects. Unevaluable discharge due to low uptake, although comprising only 34% of test-positive cases, appeared to be a highly specific but insensitive scintigraphic marker for Sjögren's syndrome. In non-irradiated xerostomic populations, scintigraphy provides specific, albeit limited, diagnostic information. The procedure's ability to distinguish uptake failure from secretory failure may be a useful asset in guiding clinical management strategies and estimating outcomes.

Synovial fluid characteristics and the lupus erythematosus cell phenomenon in drug-induced lupus. Findings in three patients and review of pertinent literature.

The characteristics of synovial fluid obtained from 3 patients with drug-induced lupus erythematosus are described. Two patients had "inflammatory" counts of synovial leukocytes, in the range of 2,500-39,000/mm3, with mononuclear predominance in 1 patient and neutrophil predominance in the other. The third patient had "noninflammatory" fluid, with mononuclear predominance. Lupus erythematosus cells formed in vivo were observed in the synovial fluid of 2 of the patients. Biopsy of the synovium of 1 patient showed nonspecific chronic inflammatory changes. Our findings in these patients with drug-induced lupus are indistinguishable from those previously described in patients with idiopathic lupus erythematosus.