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Objectives: Classification criteria for systemic sclerosis (SSc) are being developed. The objectives were to develop an instrument for collating case data and evaluate its sensibility; use forced-choice methods to reduce and weight criteria; and explore agreement among experts on the probability that cases were classified as SSc., Study Design and Setting: A standardized instrument was tested for sensibility. The instrument was applied to 20 cases covering a range of probabilities that each had SSc. Experts rank ordered cases from highest to lowest probability; reduced and weighted the criteria using forced-choice methods; and reranked the cases. Consistency in rankings was evaluated using intraclass correlation coefficients (ICCs)., Results: Experts endorsed clarity (83%), comprehensibility (100%), face and content validity (100%). Criteria were weighted (points): finger skin thickening (14-22), fingertip lesions (9-21), friction rubs (21), finger flexion contractures (16), pulmonary fibrosis (14), SSc-related antibodies (15), Raynaud phenomenon (13), calcinosis (12), pulmonary hypertension (11), renal crisis (11), telangiectasia (10), abnormal nailfold capillaries (10), esophageal dilation (7), and puffy fingers (5). The ICC across experts was 0.73 [95% confidence interval (CI): 0.58, 0.86] and improved to 0.80 (95% CI: 0.68, 0.90)., Conclusions: Using a sensible instrument and forced-choice methods, the number of criteria were reduced by 39% (range, 23-14) and weighted. Our methods reflect the rigors of measurement science and serve as a template for developing classification criteria., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Infections are one of the leading causes of morbidity and mortality in patients with systemic lupus erythematosus (SLE). Therapeutic, disease-related and genetic factors all contribute to a lupus patient's increased susceptibility to infections. Although bacterial pathogens are the most common cause of infections, a wide variety of pathogens have been reported. In high-risk populations, identification and treatment of chronic infections such as tuberculosis, hepatitis B or human immunodeficiency virus, are important prior to the institution of immunosuppression to prevent reactivation or exacerbation of the infection. Judicious use of corticosteroids and cytotoxic drugs is critical in limiting infectious complications. Vaccines against influenza and pneumococcus appear to be safe and immunogenic in SLE patients and their routine administration should be encouraged., (Copyright 2002 Elsevier Science Ltd.)

Aim: To describe our experience with alveolar hemorrhage (AH) in systemic lupus erythematosus (SLE)., Methods: Review of medical records and pertinent medical literature using MEDLINE and reference lists from retrieved publications., Patients: Seven patients with SLE admitted with episodes of AH (n = 11)., Results: Six patients were female, and one was male. Mean age at the time of AH was 31.1 years. Mean duration of SLE was 4.5 years. AH occurred within 3 weeks of SLE onset in two patients. Recurrent AH was observed in four patients. Six patients were already receiving treatment for SLE at the time of AH. All patients presented with dyspnea and new pulmonary infiltrates. Hemoptysis occurred in only 54%. All patients had BAL within 48 h of presentation. Temperature > or =39 degrees C (102.2 degrees F) accompanied 82% of episodes. Glomerulonephritis was the most common nonpulmonary SLE manifestation (74%). Treatment with empiric IV antibiotics was initiated in 10 episodes. Initial treatment included high-dose corticosteroids (prednisone, 1 to 3 mg/kg/d [n = 2]; or IV methylprednisolone, 1 g/d [n = 9], with or without oral cyclophosphamide, 2 to 3 mg/kg/d [n = 7]). Plasmapheresis (three to four sessions) was added in five episodes for persistent AH. All patients survived., Conclusions: AH may mimic pneumonia. Hemoptysis may not be evident. Infection must be aggressively excluded, especially since many patients with AH are already receiving immunosuppressive therapy. AH frequently recurs despite ongoing immunosuppression. Although high mortality rates have been reported with AH in SLE, we observed 100% survival.

Maintenance of blood flow involves the dynamic interactions between the endothelium, circulating cellular components, coagulation factors, and factors involved in fibrinolysis. Autoantibodies, inflammatory cytokines and other undefined triggers in a genetically predisposed person may lead to an imbalance in the equilibrium between the various hemostatic pathways resulting in potentially catastrophic thrombotic events. This article reviews the clinical manifestations and treatment of selected, potentially life-threatening thrombotic syndromes that may occur in association with autoimmune diseases. Special mention is made of thrombotic events associated with Behçet's syndrome.

Deterioration in the function of major organs in patients with systemic lupus erythematosus may be due to causes directly related to the disease itself or to non-lupus related causes. In patients with rapidly progressive immune-mediated disease, therapy may be initiated with pulse glucocorticoids alone or in combination with pulse cyclophosphamide. For selected patients with aggressive life-threatening disease or patients refractory to treatment, experimental therapies such as plasmapheresis or intravenous immunoglobulin may be used as adjuncts in their management.