Medicine
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We conducted the current study to characterize the clinical presentation of primary Sjögren syndrome (SS) in a large cohort of Spanish patients and to determine whether epidemiologic, clinical, and analytical features modulate disease expression. Patients were from the GEMESS Study group, which was formed in 2005 and included 12 Spanish reference centers. By March 2007, the database included 1010 consecutive patients, recruited since 1994, both incident and prevalent cases. ⋯ Hypocomplementemia was associated with a higher frequency of vasculitis and lymphoma, and cryoglobulins with a higher frequency of parotid enlargement, vasculitis, and leukopenia. Epidemiologic, clinical, and analytical features have a significant impact on the clinical presentation of primary SS, influencing the results of the main diagnostic tests, the prevalence and diversity of extraglandular involvement, and the frequency of the main immunologic markers. Primary SS should be considered as a systemic autoimmune disease that can express in many guises beyond sicca involvement.
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Comparative Study
Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology.
Pulmonary veno-occlusive disease (PVOD) is defined by specific pathologic changes of the pulmonary veins. A definite diagnosis of PVOD thus requires a lung biopsy or pathologic examination of pulmonary explants or postmortem lung samples. However, lung biopsy is hazardous in patients with severe pulmonary hypertension, and there is a need for noninvasive diagnostic tools in this patient population. ⋯ Among the 16 PVOD patients who received PAH-specific therapy, 7 (43.8%) developed pulmonary edema (mostly with continuous intravenous epoprostenol, but also with oral bosentan and oral calcium channel blockers) at a median of 9 days after treatment initiation. Acute vasodilator testing with nitric oxide and clinical, functional, or hemodynamic characteristics were not predictive of the subsequent occurrence of pulmonary edema on treatment. Clinical outcomes of PVOD patients were worse than those of PAH patients.
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Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) is increasingly recognized in older patients. The differences in disease presentation and outcome between older and younger patients remain controversial. We conducted the current study to analyze the characteristics of patients aged over 65 years with AAV and to compare the younger and older cohorts. ⋯ During follow-up, older patients had a higher risk of secondary pulmonary infection (p < 0.001), and those with pulmonary interstitial fibrosis were more likely to develop secondary pulmonary infections (p < 0.05). In conclusion, compared with younger patients, older patients with AAV had more severe and more prevalent pulmonary lesions, which might contribute to subsequent pulmonary infections after the initiation of immunosuppressive therapy. Age and pulmonary infection were independent predictors of death.