Medicine
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Pulmonary renal syndrome (PRS) is a term most commonly used to describe a combination of glomerulonephritis and pulmonary hemorrhage as a manifestation of a multisystem autoimmune disease. It is usually associated with ANCA vasculitis and anti-GBM disease. Diffuse alveolar hemorrhage in a patient with ANCA and anti-GBM negative pauci-immune glomerulonephritis is rare and optimal management is unknown. ⋯ Pauci-immune crescentic necrotizing glomerulonephritis is usually associated with the presence of ANCA, however, ANCA may be absent in 10% of these cases. Immunosuppression is the mainstay of treatment for ANCA and anti-GBM associated PRS. This case highlights the importance of immunosuppression and plasmapheresis in patients with ANCA negative vasculitis due to presence of unidentified serum antibodies. If left untreated, these patients can have a fulminant course with high mortality ranging from 25 to 50%.
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IgG4-related disease (IgG4-RD) is a chronic fibro-inflammatory disorder which is characterized by elevated levels of serum IgG4 and infiltration of IgG4-bearing plasma cells in the involved organs. Primary biliary cirrhosis (PBC) and Primary Sjögren's syndrome (pSS) are both distinct from IgG4-related disease. We herein describe a Chinese patient with IgG4-related RPF overlapping with PBC and pSS. ⋯ This rare clinical condition has seldom been reported in the literature, which suggests that common immunogenetic factors may be involved in the development of IgG-related RPF, PBC and pSS.
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Observational Study
Prevalence, risk factors, and prognosis of interstitial lung disease in a large cohort of Chinese primary Sjögren syndrome patients: A case-control study.
To determine the prevalence of pulmonary complications in primary Sjögren syndrome (pSS), and to identify the risk factors and the prognosis associated with pulmonary involvement in pSS patients. A total of 1341 hospitalized patients (853 with pSS and 488 with secondary Sjögren syndrome [sSS]) were retrospectively reviewed. Of these, 165 hospitalized patients with pSS-associated interstitial lung disease (ILD) were analyzed and recruited as a study group. ⋯ Age, RF, and CRP were independent predictors of ILD after adjustment for confounders. Lung involvement is a common and severe complication of Sjögren syndrome. Age and disease activity are correlated with pulmonary involvement in pSS patients.
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Case Reports
Continuous radiofrequency thermocoagulation under CT-guidance for glossopharyngeal neuralgia: Two case reports.
Glosssopharyngeal neuralgia (GPN) is a painful syndrome characterized by paroxysmal neuropathic pain in the pharynx, tonsil, posterior base of tongue, inner ear, and mandibular angle. The traditional therapies of GPN, including medication and surgical operation, are not always effective in pain controlling. Radiofrequency therapy is a minimally interventional technique to provide analgesia in chronic pain. ⋯ The CRF thermocoagulation produced no complications or side effects in the two cases, and was proved effective in the treatment of GPN.
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Case Reports
Obstructive bronchitis and recurrent pneumonia in esophageal achalasia in a child: A CARE compliant case report.
Esophageal achalasia is characterized by impaired relaxation of the cardia and dilation of the intrathoracic part of the esophagus. We describe the late presentation of achalasia in an 11-year old girl. ⋯ Late presentation of achalasia in children can mimic respiratory and gastrointestinal diseases. Laparoscopic Heller myotomy combined with Dor fundoplication is feasible and advisable in children suffering from achalasia of the cardia.