Hughes-stovin syndrome and massive hemoptysis: a management challenge

Hughes-Stovin syndrome is a very rare clinical entity characterized by pulmonary artery aneurysms and deep vein thrombosis [DVT]. Here we report the case of a 53-year-old man, admitted to Sultan Qaboos University Hospital, Muscat, Oman, with bilateral pulmonary artery aneurysms and lower-limb DVT who developed massive hemoptysis. He was managed successfully with high-dose steroids in combination with cyclophosphamide

Hemoptise / Hemoptysis

A hemoptise é frequentemente encontrada na prática clínica, sendo uma manifestação presente em numerosas doenças. Pode ser uma emergência respiratória com potencial risco de morte, podendo indicar grave doença intratorácica. Este artigo visa realizar revisão bibliográfica sobre hemoptise, enfatizando os principais diagnósticos diferenciais, avaliação laboratorial, imaginológica e manejo.

Hemoptysis is frequently encountered in clinical practice, and may be the presenting symptom of a number of diseases. It can be a respiratory emergency with the potential risk of death and indicate severe underlying disease. This article aims to review literature on hemoptysis, emphasizing the differential diagnosis, laboratory evaluation, imaging and management.

Multifocal epithelioid hemangioendothelioma presenting with hemoptysis.

We present a case of multifocal epithelioid hemangioendothelioma occurring in an adolescent boy who presented with massive hemoptysis and showed a pulmonary perihilar mass with multiple lesions in the liver and two lytic lesions in the ribs on imaging. The diagnosis was confirmed by lung and liver biopsy. He was treated with oral steroids.

Tranexamic acid in patients with hemoptysis.

Hemoptysis is a common respiratory symptom leading to admission to hospital. The main management of hemoptysis depends on treating the underlying cause. The use of tranexamic acid is recommended by many doctors without much information available. MATERIAL AND METHOD: This study was a randomized double blinded placebo controlled trial in using tranexamic acid (Transamine) in hemoptysis patients. The study period was one week. Patients with hemoptysis were separated into 3 groups depending on the amount of blood. Group 1 consisted of patients with blood streak sputum. Group 2 coughed up less than 20 ml of frank blood. Patients in Group 3 were those who coughed up 20-500 ml of blood per day. A record of the amount of bleeding and drug side effects was done. RESULTS: From June 1994 to May 1997, 46 patients with hemoptysis completed the study. There were 21 in the tranexamic acid group and 25 in the placebo group. The placebo group had a tendency not to have underlying lung disease and more patients who had a normal chest X-ray. The benefit of tranexamic acid in shortening the days of hemoptysis is not shown in this study. There was a low incidence of side effects of tranexamic acid in this study. CONCLUSION: This randomized double blinded placebo controlled trial could not demonstrate the benefit of tranexamic acid in shortening the days of hemoptysis and confirm the low incidence of side effects of this drug.

Hughes-Stovin syndrome: a case report and review of the literature.

A young man presented with prolonged pyrexia, recurrent optic neuritis, thrombophlebitis and bilateral pulmonary artery aneurysms with thrombus formation. The life-threatening hemoptysis necessitated mechanical ventilatory support and emergency left lower lobectomy. Systemic corticosteroids conferred clinical improvement and reduction of the remaining right pulmonary artery aneurysm. The patient eventually succumbed to sudden massive hemoptysis. This report underscores the unpredictable nature of this syndrome and emphasises the need for aggressive surgical intervention of pulmonary artery aneurysms in Hughes-Stovin syndrome.

Hemoptisis; analisis de 22 casos / Hemoptysis; analysis of 22 cases

El presente estudio se ha realizado en una serie de 22 casos de hemoptisis leve a masiva (50 a 500 ml en 24 horas) internados en el servicio de neumologia del Hospital Clinico Francisco Viedma de Cochabamba Bolivia en el periodo de mayo de 1990 a mayo de 1992. En todos los casos se realizaron Rx de Torax, microbacteriologico de espectoracion y fibrobroncoscopia. Se realizo tratamiento a base de oxitocicos (Syntocinon), y antibioticos o tratamiento antituberculoso, dependiente del caso. La edad vario de 20 a 90 anos fueron 5 casos de sexo femenino y 17 de sexo masculino. Las alteraciones radiologicas mas frecuentes fueron retraccion cicatricial en V3 superior siendo el hemitorax derecho el mas afectado. Endoscopicamente se observo congestion y aumento de vascularizacion en mucosa bronquial en un 27// de los casos, lesion vegetante 23//, lesion granulomatosa 18//, disminucion de calibre bronquial 18//, ensanchamiento de carina bronquial 14//. El arbol bronquial superior mas afectado fue el derecho , 73// siendo afectado el lobulo superior derecho en un 32// el resultado histopatologico fue: carcinoma bronquial 10 casos (46//), bronquitis cronica 4 casos (18//), tuberculosis pulmonar 3 casos (13//), micosis pulmonar (aspergilosis) 3 casos (13//), fibrosis pulmonar y leptospirosis pulmonar con un caso cada uno (5//). Se concluye que toda hemoptisis deberia ser bien estudiada y siempre pensar en carcinoma broquial en pacientes mayores a 40 anos.