Development of cryptogenic fibrosing alveolitis during pregnancy.
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Idiopathic pulmonary fibrosis (IPF) is a condition that has a poor prognosis, with a median survival of 4-5 years irrespective of treatment. Ziesche et al (N Engl J Med 1999, 341: 1264-1269) describe an open randomised trial of 18 patients with IPF, unresponsive to corticosteroid treatment at high dose. Nine patients were treated with continued corticosteroid and nine with prednisolone plus interferon-gamma 1b (IFN-gamma). Significant benefits in physiological parameters are reported in the IFN-gamma-treated group. An analysis of lung tissue by reverse-transcriptase-mediated polymerase chain reaction showed corresponding decreases in the transcription of transforming growth factor-beta1 and connective tissue growth factor. This is the first report of treatment showing efficacy in this disease, albeit in a very preliminary study, but the data should be viewed with caution. This study is discussed in the context of other published studies of treatment for IPF and the scientific rationale on which it was based.
The aim of this study was to determine the prevalence of enlarged mediastinal lymph nodes in systemic sclerosis (SSc) and relate this to the extent of pulmonary disease and disease activity as judged by high resolution computed tomography (HRCT). The HRCT scans of 78 patients with SSc were reviewed. The extent of lung disease and HRCT pattern were analysed and CT scans examined on soft tissue window settings for evidence of mediastinal lymph node enlargement. Sixty six (85%) patients had evidence of lung involvement on CT. Enlarged mediastinal lymph nodes were present in 21 (32%) patients with lung involvement but in only 1 (8%) patient without. The prevalence of enlarged mediastinal nodes increased with more extensive lung involvement on CT (p < 0.025), but correlated poorly with the type of CT appearance and concurrent erythrocyte sedimentation rate. Mediastinal lymph node enlargement occurs frequently in patients with SSc, particularly if lung involvement is extensive.
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An 8-month-old, female, mixed-breed dog presented to the Iowa State University Veterinary Teaching Hospital with a 1-month history of vomiting and diarrhea. An exploratory laparotomy was performed revealing markedly distended and fluid-filled small and large intestines that were not obstructed. The clinical condition of the dog did not improve subsequent to exploratory surgery, and it was euthanized. At necropsy, both the small and large intestines were distended (approximately 4 cm in diameter) and fluid-filled, and the wall was thin. The abdominal cavity contained approximately 500 ml of a brownish clear fluid. Microscopic lesions of the intestines were confined to the intestinal tunica muscularis and muscularis mucosae and consisted of locally extensive-to-diffuse replacement of the smooth muscle by fibrous tissue and multifocal infiltration by a moderately dense mononuclear inflammatory infiltrate. A unique finding was the presence of similar microscopic lesions in the tunica muscularis of the urinary bladder and stomach.
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