|Year : 2019 | Volume
| Issue : 4 | Page : 365-366
An unusual cause of massive hemoptysis: The demise of clinical history and a teachable moment from a fatal case
Bharath Chhabria1, Aman Sharma2, Ritesh Agarwal1, Valliappan Muthu1
1 Department of Pulmonary Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India
|Date of Web Publication||28-Jun-2019|
Dr. Valliappan Muthu
Department of Pulmonary Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Chhabria B, Sharma A, Agarwal R, Muthu V. An unusual cause of massive hemoptysis: The demise of clinical history and a teachable moment from a fatal case. Lung India 2019;36:365-6
|How to cite this URL:|
Chhabria B, Sharma A, Agarwal R, Muthu V. An unusual cause of massive hemoptysis: The demise of clinical history and a teachable moment from a fatal case. Lung India [serial online] 2019 [cited 2019 Sep 21];36:365-6. Available from: http://www.lungindia.com/text.asp?2019/36/4/365/261717
Massive hemoptysis is a common cause of emergency admission. With the widespread availability of sophisticated imaging and advanced therapeutic options, most cases of hemoptysis are managed successfully. In a patient with ongoing hemoptysis, stabilizing the patient by preventing asphyxia due to aspiration of blood is the priority. Nevertheless, a thorough medical history is necessary, even in the current era. Herein, we present the case of a young male who presented with massive hemoptysis due to an unusual cause, which was identified late in the course of the disease. We emphasize the importance of a detailed history and discuss the various missed opportunities in the index case.
A 30-year-old gentleman presented with fever, productive cough, weight loss, and hemoptysis of 3 months' duration. On the basis of the history and limited clinical examination findings (erythema nodosum), he was started on antitubercular therapy (ATT). Chest radiograph was unremarkable. His fever resolved, but he had an episode of massive hemoptysis 1 month later. In the emergency room, a computed tomography (CT) of the thorax showed dissection of the thoracic aorta. Urgent endovascular repair was undertaken. Postoperatively, he developed a pseudoaneurysm of the right femoral artery (site of vascular access) which required surgical repair. He remained asymptomatic for the next 2 months when hemoptysis recurred and he presented to our hospital. A detailed medical history revealed the presence of multiple oral ulcers for the past 3 years and a single scrotal ulcer a year back that resolved spontaneously. He also reported the development of reddish lesions at the sites of recent intravenous cannulation. Macular hyperpigmented skin lesions over the forearms (at the site of the cannula) and erythema nodosum over both the legs were noted [Figure 1]. C-reactive protein and erythrocyte sedimentation rate were elevated. Contrast-enhanced CT of the thorax revealed bilateral pulmonary aneurysms, and the endovascular aortic graft was seen in situ [Figure 2]. Skin biopsy suggested septal panniculitis with lymphocytic vasculitis; human leukocyte antigen B51 was positive. He was diagnosed as a case of vasculo-Behçet's disease (BD) and managed with intravenous methylprednisolone pulse followed by monthly intravenous cyclophosphamide. He remained asymptomatic for the next 3 months. Four months after the therapy, he had an episode of massive hemoptysis at home and succumbed to the illness.
|Figure 2: Contrast-enhanced computed tomography showing pulmonary artery aneurysm (arrow) and endovascular graft seen in the descending thoracic aorta|
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BD is a multisystemic disorder characterized by recurrent, painful orogenital ulcers, skin lesions, pathergy, ocular inflammation, and occasionally life-threatening vasculitis. Large-vessel involvement is relatively rare, seen in 14.3% in a cohort of 2319 patients of BD from Turkey. Deep venous thrombosis was the most common form of large-vessel involvement, whereas arterial involvement was seen in 3.6% (most frequently involving the pulmonary artery). Vascular complications develop late in the course of the disease, and only 2.1% of BD patients present with vascular symptoms as the first manifestation, indicating that the diagnosis can often be established prior to the development of large-vessel vasculitis. As there is no single diagnostic test for BD, only a comprehensive history and thorough physical examination can lead to early recognition and initiation of appropriate therapy.
High-dose steroids and monthly pulses of cyclophosphamide are the currently recommended treatment for patients with major arterial aneurysms. Tumor necrosis factor-α (TNF-α)-blocking agents have been used in treating patients (as the inflammation is Th-1 mediated and involves TNF-α) who are refractory to cyclophosphamide. Furthermore, surgical or endovascular interventions should not be undertaken until the disease is in remission as any foreign material such as stents or synthetic grafts may lead to disease reactivation. The latter is analogous to the Koebner effect and is known as pathergy in BD.
India bears the highest burden of tuberculosis in the world, having an incidence of 211/100,000 and mortality (in HIV-negative population) of 32/100,000 individuals. With such large numbers being affected every year, empirical therapy with ATT is often justified, especially in individuals with long-standing fever, productive cough, and weight loss. However, performing a sputum microscopy and Xpert MTB/RIF is necessary before a trial of empiric therapy, especially when these investigations are now widely available in India. An attempt to establish a microbiological diagnosis is essential, which was missed in the index case.
Aneurysm of the thoracic aorta in a young nonsmoker male should have raised the suspicion of vasculitis, and few relevant questions were required to ascertain its etiology. A mere inquiry into the past history (of orogenital ulcers) and the origin of the skin lesions on the forearms at the second visit might have channelized the investigations toward BD. Early diagnosis may also have enabled forestalling the endovascular repair until the disease was in complete remission and timely initiation of immunosuppressive therapy.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]