Bronchiolitis obliterans in systemic lupus erythematosus: beneficial effect of intravenous cyclophosphamide

Article Abstract:

Complications involving the lungs are common in systemic lupus erythematosus (SLE), occurring in up to 70 percent of patients. Among the pulmonary problems are pleural effusion (seepage of fluid into the membranous area surrounding the lungs), pneumonitis (inflammation of the lung), atelectasis (collapse of part or all of a lung), and hemorrhage. Bronchiolitis obliterans, constriction of smaller airways caused by proliferation of granulation tissue (blood vessel-rich tissue formed on surfaces of wounds) following damage, is rarely associated with SLE but has been linked to infection or exposure to toxic fumes. A case is reported of a 49-year-old nonsmoking woman who had SLE for 30 years without pulmonary complications. Her symptoms included severe dyspnea (shortness of breath) with an unproductive cough. She had used low-dose prednisone (a steroid) for 20 years. X-rays showed shadows on the lungs, and tests indicated mildly restricted flow with decreased expiration. Protein was present in urine, indicating kidney dysfunction, and kidney biopsy showed established glomerulonephritis (disease involving the filtration structures of the kidney). No tests were positive for infections, so bronchiolitis obliterans was suspected, with an onset associated with the lupus kidney dysfunction. The steroid dose was increased, and lung function improved initially. However, the condition recurred within five months, without changes in tests of immune or kidney function. Cyclophosphamide was added to the treatment, and the patients improved dramatically within two days. This was stopped after five months due to low white blood cell levels. The patient remained well three years after the onset of lung problems. (Consumer Summary produced by Reliance Medical Information, Inc.)

Bronchiolitis

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Blood rheology in lupus erythematosus

Article Abstract:

Blood rheology (flow and deformation) is abnormal in lupus erythematosus and other autoimmune diseases, which are characterized by abnormal immune responses against cells in the body that are identified as foreign. Abnormal blood rheology is significant in lupus because one of the more severe complications of the disease involves thromboembolization (blood clot formation and travel), and hindrance of blood flow through small blood vessels would potentiate these difficulties. (Thromboembolization refers to the intravascular formation of blood clots that may break off into the circulation and then obstruct smaller vessels.) Details of blood rheology were evaluated in 51 patients with various types of lupus. Except for packed cell volume and red blood cell deformability, rheological values including blood viscosity and red blood cell aggregation were elevated. Red blood cell deformability was reduced in female SLE patients compared with healthy female control subjects. No significant differences were found among patients with chronic discoid or subacute cutaneous lupus erythematosus. However, patients with systemic lupus had significantly higher blood viscosity, lower packed cell volume, and higher red blood cell aggregation as compared with all lupus patients. The report suggests that these marked and significant blood rheological abnormalities likely impede microcirculatory blood flow and deserve further study. (Consumer Summary produced by Reliance Medical Information, Inc.)

Blood, Hemorheology

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Chronic lupus peritonitis with ascites

Article Abstract:

Systemic lupus erythematosus (SLE) is a rheumatic disease with an autoimmune basis, in which the body inappropriately makes antibodies that recognize and attack its own tissues. SLE can involve a variety of organ systems, any of which may be exclusively involved in an affected individual. Chronic ascites, accumulation of fluid in the peritoneum (abdominal cavity), is a rare manifestation of SLE. This report describes the case of a 25-year-old woman with inactive SLE who developed ascites of unknown cause, which was followed by disease flaring with kidney disease and probable vasculitis (inflammation of blood vessels) with peritonitis (inflammation of the membrane lining the abdominal cavity). The patient was initially treated with steroids and hydroxychloroquine with resolution of her symptoms of fever, pericarditis (inflammation of heart sac), anemia, and low levels of white blood cells. Two years later, heartburn and abdominal discomfort associated with gastritis developed, followed by ascites, headache, rapid heartbeat, vertigo, decreased visual acuity, and kidney disease, which were not responsive to increased doses of prednisone. Diuretics were also ineffective, but symptoms began to resolve following treatment with intravenous steroids and azathioprine, which suppresses immune system function. Within two months of treatment, no symptoms were present and the patient remained well eight months later. (Consumer Summary produced by Reliance Medical Information, Inc.)

Ascites

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