Severe Bilateral Pulmonary Fibrosis in a Young Syrian Refugee

By Humberto Choi, MD, and Susan Vehar, MD

A 35-year-old male Syrian refugee presents to an Ohio emergency department with shortness of breath after arrival in the United States two days prior. He is cachectic with decreased bilateral breath sounds and reports a history of chronic dyspnea on exertion since his late 20s with notable progression in the last 60 days.

Chest radiograph shows bilateral apical pneumothoraces, and bilateral chest tubes are placed. Dense consolidative opacities are most prominent along the mid and lower lung zones. Innumerable tiny and confluent nodules are noted bilaterally.

Chest computed tomography (CT) reveals bilateral coarse reticular opacities, innumerable perilymphatic and centrilobular nodules, confluent fibrotic-type consolidative opacities in all lung lobes and bilateral pneumothoraces.

With a wide variety of options on the differential diagnosis — from war-related exposures to infection to lymphoma — we need more history to help us narrow the differential. His refugee status introduces a number of barriers to obtaining further history: the inability to obtain medical records, lack of access to prior healthcare, lack of financial and social support, post-traumatic stress, a general distrust of healthcare providers, the need for an Arabic translator and the potentially critical nature of his illness.

Talking with the patient reveals prior exposure to industrial and war-related chemicals. He is unable to specifically identify these exposures. He seems hesitant to disclose some historical information due to uncertainty surrounding its potential implications.

Evaluation, board review

His evaluation included testing for multiple infectious etiologies including bronchioalveolar lavage and sputum cultures which were unrevealing. Evaluation for autoimmune and rheumatologic etiologies was unrevealing. In addition, evaluation for pulmonary embolism and heart disease was negative. His TB testing was negative according to his minimal refugee health screening records and on multiple acid-fast bacilli smears during his evaluation.

He had a prolonged hospitalization and did not show improvement with multiple courses of antibiotics and supportive treatment. He underwent multiple attempts with chemical pleurodesis and had persistent pneumothoraces requiring ongoing management with pigtail catheters. Unfortunately, during the repositioning of the chest tube, he experienced cardiac arrest and was intubated and transferred to intensive care. He also experienced C. difficile with megacolon and needed parenteral nutrition. He remained critically ill in the medical intensive care unit, and ultimately bronchoscopy with transbronchial biopsy was performed since he was too ill for an open lung biopsy.

The patient’s case was presented at the interstitial lung disease board review. As pathology showed non-specific focal fibrosis, foreign-body-type giant cell reaction and focal airspace hemorrhage, and as there were no diagnostic features of silicosis or evidence of infectious of malignant etiology, the patient was diagnosed with suspected occupational/environmental exposure causing inflammatory and fibrotic lung disease.

The patient required ongoing respiratory support. A tracheostomy was placed, and he was stabilized and discharged to long-term acute care for rehabilitation. Upon further rehabilitation, he would have been considered for advanced treatment including lung transplantation, but he has not been seen in follow up since discharge.

An unsatisfactory diagnosis

Many factors related to this patient’s refugee status prevented the kind of definitive diagnosis that allows us to provide precise, aggressive care in a critically ill, young male. According to Atul Mehta, MD, Chief Medical Officer at Sheikh Khalifa Medical City and Abu Dhabi and staff in Cleveland Clinic’s Respiratory Institute, “The wealth of pulmonary pathologies encountered in the Middle East probably surpasses all other regions of the world.” In his review in Respirology, he examines the wide spectrum of possibilities one encounters in patients from these areas, including dust/sand exposure precipitating proteinosis and silicosis, byssinosis from textile production, psittacosis from parrot, allergic bronchopulmonary aspergillosis, asbestosis, beryllium disease, radon gas exposure, echinococcosis, leishmaniasis, schistosomiasis, COPD/asthma from water pipe use, brucellosis, chemical and dust attacks in war and more.

This patient’s case highlights the unique challenges refugees can face in maintaining pulmonary health and receiving quality care for pulmonary issues caused by sociopolitical crises. As Dr. Mehta notes, pulmonary care for patients from the Middle Eastern region entails consideration of a wide range of etiologies not commonly seen in other regions. With millions of Syrian refugees being resettled around the world, the global medical community must be aware of and prepared to treat pulmonary pathologies unique to regions in war and crises.

Dr. Choi is staff in the Respiratory Institute. Dr. Vehar is a rising chief resident in the Department of Medicine.