An Unusual Cause of Recurrent Haemoptysis in a Young Indian Girl

Abstract

SESSION TITLE: Miscellaneous SESSION TYPE: Global Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Vasculitis features as a rare entity on the list of ‘causes of haemoptysis’. We report a young Indian girl with a decade long history of recurrent haemoptysis due to Takayasu Arteritis (TA). CASE PRESENTATION: A 24-year-old non-pregnant female presented to us with chief complaint of recurrent streaky haemoptysis over last ten years. On further questioning, she narrated a positive history of retrosternal chest discomfort, exertional breathlessness, headache, visual disturbances, palpitations, syncopal episodes, malaise and weight loss. She also described a feeling of pulselessness in her left upper limb along with weakness, paresthesias, Raynaud’s phenomenon and claudication. Physical examination revealed absent left carotid, brachial and radial pulses and feeble left femoral, popliteal and dorsalis pedis pulsations. The blood pressure in right and left upper limbs was 206/100 and 104/78 mmHg, and in corresponding lower limbs was 244/96 and 228/86 mmHg, respectively. A right carotid bruit was appreciated. Routine blood investigations, electrocardiogram and echocardiography were unremarkable except for an ESR of 58mm in first hour. Chest roentgenogram showed prominent vessels at the left hilum. A contrast-enhanced computed tomogram/angiogram of the thorax and abdomen revealed numerous dilated vessels in the left upper lobe; dilated ascending aorta; streak-like left common carotid and left subclavian arteries; circumferentially thickened, irregular walls of the aortic arch, descending thoracic and abdominal aorta with luminal narrowing; and left renal artery stenosis. Aneurysms were noted at the arch and descending thoracic aorta. Pulmonary arteries were largely normal. Considering the systemic nature of involvement, classical clinical features and typical angiographic findings, the patient was diagnosed as TA. The condition was discussed with her and anti-hypertensives, steroids, hypolipidemic drugs and aspirin were prescribed. However, she was lost to follow-up. DISCUSSION: TA is a rare, chronic inflammatory vasculitis most commonly affecting the aorta and large vessels. It is mostly reported among Asian females of child bearing age, usually in the 2nd or 3rd decade, with a global incidence of 2.6/million/year. Aetiology is largely unknown, with roles of infection, autoimmunity and genetic factors implicated in its pathogenesis. Vessel wall inflammation leads to its thickening, fibrosis, stenosis and thrombosis, thus producing ischemic organ damage. Clinical manifestations may range from constitutional symptoms like fever, malaise, arthralgia, weight loss and anaemia, to classical features like pulselessness, claudication, blood pressure (BP) discrepancy between limbs, bruits, heart failure, headache, visual disturbances, stroke, seizures, retinopathy, hypertension, aortic regurgitation and aneurysm formation. The last four are the most dreaded complications. Pulmonary manifestations could be in the form of dyspnoea, chest pain, cough, haemoptysis, alveolar haemorrhage, pulmonary hypertension, pleural effusion, cavitation, pulmonary thromboembolism, pneumonia, pulmonary fibrosis and pulmonary artery aneurysm. Haemoptysis may arise from rupture of collaterals/microaneurysms, pulmonary venous congestion, systemic hypervascularization or diffuse alveolar haemorrhage. The gold standard for diagnosis is CT angiography which identifies the affected vessels. Our patient belonged to Type III category according to the angiographic classification of TA and met five of the six diagnostic criteria proposed by the American College of Rheumatology (≥3 required for diagnosis). The current best evidence based treatments include steroids with a 50% response rate, and methotrexate with a further 50%. Other components of medical management include treatment of hypertension, thrombosis, dyslipidemia and atherosclerosis. Surgical options are limited to select patients during quiescence. Overall ten-year survival rate is ≈90%. Cardiac and cerebrovascular complications contribute to mortality in most of the cases. CONCLUSIONS: TA is an uncommon cause of haemoptysis. Careful patient evaluation and awareness about the condition facilitates diagnosis. Reference #1: SL Johnston, et al. Takayasu arteritis: a review. J Clin Pathol 2002;55:481-6. Reference #2: O. Brugiere, et al. Isolated pulmonary arteries involvement in a patient with Takayasu's arteritis. Eur Respir J 1998;11:767-770. Reference #3: EY Chan, et al. Massive haemoptysis in an 11-year-old girl with isolated pulmonary arteritis. Pediatr Pulmonol 2007;42:177-80. DISCLOSURE: The following authors have nothing to disclose: Mayank Mishra, Subodh Kumar No Product/Research Disclosure Information