Characterization of a subset of patients with primary Sjögren's syndrome initially presenting with C3 or C4 hypocomplementemia.

OBJECTIVE: This study aimed to determine the association of C3 and C4 hypocomplementemia at the diagnosis of primary Sjögren's syndrome (pSS) with clinical manifestations, disease activity, and disease damage. METHODS: A cross-sectional study was conducted in 94 Puerto Ricans with pSS. Patients were aged ≥21 years and met the 2012 American College of Rheumatology Classification Criteria for pSS. Demographic characteristics, health-related features, cumulative extraglandular manifestations, serologic tests at pSS diagnosis, comorbidities, disease activity (per European League Against Rheumatism Sjögren's Syndrome Disease Activity Index [ESSDAI]), disease damage (per Sjögren's Syndrome Disease Damage Index [SSDDI]), and pharmacologic therapy were determined. Serum C3 and C4 levels were measured at pSS diagnosis by immunoturbidimetry. Patients with and without hypocomplementemia were analyzed using bivariate and multivariate logistic regression analyses adjusted for age, sex, and disease duration. RESULTS: The mean age and disease duration of the study population were 52.4±12.4 years and 5.9±4.8 years, respectively; of the total study population, 94% were female. C3 and C4 hypocomplementemia were observed in 9.6% and 13.8% of the patients, respectively. In the multivariate analysis, C3 hypocomplementemia was associated with leukocytoclastic vasculitis, interstitial lung disease, higher SSDDI score, and exposure to rituximab. C4 hypocomplementemia was associated with leukocytoclastic vasculitis, interstitial lung disease, and higher ESSDAI and SSDDI scores. CONCLUSION: In this population of patients with pSS, low C3 and C4 levels at diagnosis were associated with extraglandular manifestations such as vasculitis and interstitial lung disease, as well as disease activity and damage accrual. These results suggest that complements C3 and C4 have clinical and prognostic value in patients with pSS.

Long-Term Outcomes in Puerto Ricans with Rheumatoid Arthritis (RA) Receiving Early Treatment with Disease-Modifying Anti-Rheumatic Drugs using the American College of Rheumatology Definition of Early RA.

BACKGROUND: Early treatment of rheumatoid arthritis (RA) results in better long-term outcomes. However, the optimal therapeutic window has not been clearly established. OBJECTIVE: To determine the clinical outcome of Puerto Ricans with RA receiving early treatment with conventional and/or biologic disease-modifying anti-rheumatic drugs (DMARDs) based on the American College of Rheumatology (ACR) definition of early RA. METHODS: A cross-sectional study was performed in a cohort of Puerto Ricans with RA. Demographic features, clinical manifestations, disease activity, functional status, and pharmacotherapy were determined. Early treatment was defined as the initiation of DMARDs (conventional and/or biologic) in less than 6 months from the onset of symptoms attributable to RA. Patients who received early (< 6months) and late (≥6 months) treatments were compared using bivariate and multivariate analyses. RESULTS: The cohort comprised 387 RA patients. The mean age at study visit was 56.0 years. The mean disease duration was 14.9 years and 337 (87.0%) patients were women. One hundred and twenty one (31.3%) patients received early treatment. In the multivariate analysis adjusted for age and sex, early treatment was associated with better functional status, lower probability of joint deformities, intra-articular injections and joint replacement surgeries, and lower scores in the physician's assessments of global health, functional impairment and physical damage of patients. CONCLUSION: Using the ACR definition of early RA, this group of patients treated with DMARDs within 6 months of disease had better long-term outcomes with less physical damage and functional impairment.

Efficacy of immunosuppressive treatment in a systemic lupus erythematosus patient presenting with inclusion body myositis.

Inclusion body myositis (IBM) is an inflammatory myopathy that is generally unresponsive to immunosuppressive drugs. The coexistence of IBM with other autoimmune connective tissue diseases is rare. We present a case of a 76-year-old woman with systemic lupus erythematosus (SLE) who developed proximal muscle weakness of lower extremities and mild elevation of serum creatine kinase (CK) at 495 U/L. Muscle biopsy showed changes of endomysial inflammation and rimmed vacuoles consistent with IBM. She was treated with prednisone 40 mg daily and methotrexate 12.5 mg weekly. One month later, her physical examination showed minimal proximal weakness of lower extremities. CK levels decreased to 44 U/L. Prednisone dose was gradually decreased to 5.0 mg daily. She remained stable with normal CK levels during a follow-up period of 10 months. This case, together with other reports, suggests that IBM in the setting of SLE represents a different subtype that can benefit from immunosuppressive treatment.