Progressive systemic sclerosis sine scleroderma presenting as pulmonary interstitial fibrosis

Article Abstract:

Systemic sclerosis is an autoimmune disease affecting blood vessels and connective tissue. (The body produces a disordered immunological response against itself.) Systemic sclerosis is characterized by fibrous degeneration of connective tissue and most frequently affects the skin, but may affect other organs such as the lungs, esophagus and kidneys. To isolate the factors that might assist in the diagnosis of progressive systemic sclerosis sine scleroderma (without skin changes), the authors retrospectively studied the medical records of 10 patients in whom systemic sclerosis first appeared as interstitial lung disease (chronic inflammation of air sacs, or alveoli which are unable to function normally in gas exchange). This review included six patients from the authors' clinic and four case studies obtained by computer search. In five of the six clinic patients, dyspnea (shortness of breath) was the first symptom that occurred. Five of the six patients exhibited Raynaud's phenomena, a disorder characterized by abnormal constriction blood vessels in the extremities upon exposure to cold or stress. The antinuclear antibody (ANA) test was positive in 87 percent of the subjects and esophageal dysfunction was present in 89 percent. Nailbed capillary examinations revealed scleroderma-type capillaries in all patients. Other symptoms included restrictive pulmonary function tests (75 percent) and a decreased capacity to diffuse carbon monoxide (86 percent). Six of the 10 patients later (up to seven years) developed typical scleroderma. Three patients had occupations that could have caused lung disease; two were miners and one a shipyard worker. All of the patients were men, whereas typical scleroderma most frequently affects women. It was concluded that when interstitial lung disease is present without scleroderma, systemic sclerosis is a possible diagnosis. Some of the diagnostic procedures that can detect this condition are esophagram, ANA analysis and widefield nailfold capillary examination performed under a microscope. The physician should also look into the possibility of occupational exposure as a contributing factor.

Case studies, Diagnosis, Systemic scleroderma, Pulmonary fibrosis, Diagnosis, Differential, Differential diagnosis, Collagen diseases, Sclerosis

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Progressive systemic sclerosis complicated by severe hypertension: reversal after silicone implant removal

Article Abstract:

Patients who receive silicone implants, particularly breast implants, may develop immune disorders such as systemic lupus erythematosus, rheumatoid arthritis, or progressive systemic sclerosis (PSS). PSS, also known as scleroderma, is the best recognized of these adjuvant diseases, but the severity, disease course, and reversibility following implant removal are unpredictable or unclear. A case is reported of a 43-year-old woman who developed PSS following silicone breast implantation. The woman had been in good health until diagnosis of breast cancer in June 1986. The woman received a silicone implant four months after surgery. The patient began to develop symptoms of PSS in August 1987, including painful swelling over face and extremities, poor grip of objects, pigmentation on hands, sensory disorders, and Raynaud's phenomenon (discoloration and numbness of fingers and toes commonly induced by exposure to cold). Skin tightness progressed, and high blood pressure (hypertension) developed in spite of medication. Following implant removal in March 1989, Raynaud's phenomenon and hypertension resolved, and the skin began to return to normal. Hypertension had not previously been observed as a complication of this type of PSS. This case is also unique because of the close relationship between implant removal and remission of disease. The relationship between silicone implants and PSS is supported and provides evidence that implant removal is beneficial in at least some cases. (Consumer Summary produced by Reliance Medical Information, Inc.)

Mammaplasty, Breast reconstruction, Silicones in medicine, Medical silicones

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Evaluation and management of scleroderma lung disease using bronchoalveolar lavage

Article Abstract:

Scleroderma is a long-term disease characterized by sclerosis or hardening of the skin and other organs due to excessive growth of fiber-like tissue. The incidence of alveolitis (inflammation of the alveoli, the gas-exchanging units of the lung) was assessed using bronchoalveolar lavage (BAL), the washing out of the airways. The changes in BAL findings over time and the relation of such changes to the function of the lung were also evaluated in 43 nonsmoking patients with scleroderma. The BAL fluid was analyzed for the presence of inflammatory cells, such as macrophages and granulocytes; immunoglobulin G (IgG), a type of antibody; immune complexes, consisting of antibody attached to antigens; and fibronectin, a protein involved in protection against foreign particles in the blood. Patients with scleroderma had higher levels of IgG and immune complexes in BAL fluid and greater release of fibronectin from macrophages than normal subjects. Alveolitis was detected using BAL in 21 patients and was associated with increased numbers of macrophages and granulocytes in the BAL fluid; more difficulty breathing; and increased deterioration of lung function over time. Treatment with the drugs cyclophosphamide and prednisone improved breathing and lung function in patients with alveolitis. The results show that alveolitis is prevalent among patients with scleroderma and can be detected by BAL, and that treatment of alveolitis can lead to improved lung function. (Consumer Summary produced by Reliance Medical Information, Inc.)

Pulmonary alveoli, Bronchoalveolar lavage

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