Pulmonary Manifestations of Systemic Disease: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease I |

Thromboangitis Obliterans, Dysphagia, and Dyspnea: More Than Meets the Eye FREE TO VIEW

Vishisht Mehta, MBBS; Rouhin Sen, MD; Gaurav Goyal, MBBS; Lakshmi Chintalacheruvu, MBBS; Karishma Bhatia, MBBS; Mohsin Mirza, MD; Tammy Wichman, MD
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Creighton University, Omaha, NE

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1078A. doi:10.1016/j.chest.2016.08.1185
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SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease I

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Systemic sclerosis (SSc) has varied presentations. If only skin manifestations are addressed, associated systemic conditions may be missed. Only esophageal involvement is more common than pulmonary involvement [1]. Multi-organ manifestations of SSc mimic many other conditions. We illustrate the dangers of delayed diagnosis.

CASE PRESENTATION: A 57 year old Caucasian male with a 20 year history of Thromboangitis obliterans (TO) presented primarily with fatigue and melena. He had a history of digit amputations for TO. Examination revealed regular tachycardia, basilar crackles, digit amputations and ulcerations, sclerodactyly and telangiectasias. Pan-endoscopy showed gastric arteriovenous malformations, watermelon stomach and was complicated by paroxysmal atrial flutter. Echocardiogram revealed preserved ejection fraction, reduced right ventricular function with increased size and right ventricle systolic pressure of 46 mm Hg. Further history revealed long standing Raynaud’s phenomenon, exertional dyspnea and progressive facial skin tightening. A chest CT showed sub-pleural interstitial thickening and fibrosis, consistent with usual interstitial pneumonia (UIP) pattern, in addition to bilateral pleural effusions (Fig.1 ). Serology revealed high titers of RNA polymerase III antibodies. Thus we challenged the diagnosis of TO and the patient was diagnosed with limited cutaneous SSc. Further evaluations are now ongoing.

DISCUSSION: Apart from digit ulcerations and amputations, other systemic manifestations were insidious and subtle, leaving his diagnosis of TO unchallenged. ILD and PH are the most common pulmonary manifestations of SSc. ILD is present in diffuse (50%) and limited (25%) SSc. It occurs earlier in diffuse SSc and in both it portends a worse prognosis. Fatigue, exertional dyspnea and dry cough are common presenting symptoms and fine, inspiratory, ‘velcro’ crackles common exam findings. ILD is evaluated with high resolution CT scans. Lung biopsy is rarely indicated if imaging does not suggest ILD. UIP pattern is infrequent and is associated with a shorter survival time. SSc-PAH (group 1) has a higher mortality than PAH alone. SSc may be associated with group 2 and less commonly group 3 PH. Those with long-standing limited SSc are at greatest risk. All patients must have full PFTs and an echocardiogram [2]. A right heart catherterization should be promptly performed in those with echocardiographic evidence of PH or profound symptoms as PH, rather than ILD, might be causing symptoms.

CONCLUSIONS: SSc mimics other conditions. It may be insidious and SSc-ILD and SSc-PH even more so. However, the systemic effects of systemic sclerosis can be missed if the patient is incorrectly diagnosed as thromboangitis obliterans.

Reference #1: Systemic sclerosis: demographic, clinical, and serologic features and survival in 1,012 Italian patients. Ferri C et al. Medicine (Baltimore) 2002

Reference #2: Behr J et al. Pulmonary function tests. Rheumatology (Oxford) 2008

DISCLOSURE: The following authors have nothing to disclose: Vishisht Mehta, Rouhin Sen, Gaurav Goyal, Lakshmi Chintalacheruvu, Karishma Bhatia, Mohsin Mirza, Tammy Wichman

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