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Fatal hemoptysis in Ehlers-Danlos syndrome. Old malady with a new curse.

We describe the case of a 27-year-old man with Ehlers-Danlos syndrome, type IV. The patient had recurrent and eventually fatal pulmonary hemorrhage. Type IV Ehlers-Danlos syndrome is a rare disorder of type III collagen synthesis. It is characterized by an unusual facies, thin, translucent skin with venous vascular pattern, and hypermobility of the small joints. The cause of death is usually due to rupture of a viscus or a major arterial hemorrhage and, in women, rupture of the gravid uterus. Fatal lung hemorrhage in Ehlers-Danlos syndrome, to our knowledge, has not been previously described.

Adult↗

Positive antineutrophil cytoplasmic antibodies-associated vasculitis presenting with hemoptysis and a mediastinal mass.

A patient with end-stage renal failure, due to IgA nephropathy, was found to have a mediastinal mass. Biopsy specimen of the mass showed a necrotizing vasculitis. Antineutrophil antibodies to myeloperoxidase were strongly positive. To our knowledge, no case of a mediastinal mass due vasculitis has been reported in the literature, and our observation should lead to broadening of the spectrum of clinical manifestations of vasculitis.

Antibodies, Antineutrophil Cytoplasmic↗

Hemoptysis season.

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Bronchial Diseases↗

Catamenial hemoptysis from tracheobronchial endometriosis: reappraisal of diagnostic value of bronchoscopy and bronchial brush cytology.

OBJECTIVES: To analyze the clinical data of four patients with a diagnosis of tracheobronchial endometriosis, and to reappraise the diagnostic value of bronchoscopy and bronchial brush cytology in these patients. METHODS: We conducted a retrospective study of four patients with documented tracheobronchial endometriosis treated at National Taiwan University Hospital from 1994 to 1998. The complete histories, diagnostic time interval, results of physical examinations, laboratory data, bronchoscopic findings, cytologic results, chest radiographs, and chest CT of these patients were analyzed. RESULTS: These patients tend to be younger and nonmultiparous as compared to other patients with thoracic endometriosis. Bronchoscopic examination performed within 1 day or 2 days of menses disclosed multiple purplish-red submucosal patches bilaterally that bled easily when touched. Cytologic evaluation of the brushing specimens demonstrated clusters of small cuboid cells consistent with an endometrial origin. Follow-up bronchoscopic examination in the middle of the menstrual cycle showed disappearance of the previous tracheobronchial lesions. The mean diagnostic interval was 3.25 months. All four patients were successfully treated with danazol therapy. CONCLUSIONS: Tracheobronchial endometriosis consists of a special subgroup of patients with thoracic endometriosis. Proper timing of bronchoscopic examination plays an important diagnostic role in these patients. Cytologic features as well as cyclic changes in bronchoscopic findings are sufficient to warrant the diagnosis. The results of treatment with danazol in these patients seemed favorable.

Adult↗