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

Cavitation and Confusion: A Puzzling Presentation of Granulomatous Disease FREE TO VIEW

Meredith Greer, MD; Udayan Shah, MD
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UT Southwestern Medical Center, Dallas, TX

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

Chest. 2016;150(4_S):1096A. doi:10.1016/j.chest.2016.08.1203
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SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease II

SESSION TYPE: Student/Resident Case Report Poster

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

INTRODUCTION: A 32-year-old HIV-negative African American man presented with a 7-month history of altered mental status (AMS) and functional decline for which he underwent multiple rounds of hospitalizations and diagnostic testing before the correct diagnosis was made.

CASE PRESENTATION: He was first seen at an outlying hospital for AMS, which was attributed to drug use. Chest x-ray (CXR) revealed bilateral cavitary lung lesions, and he was treated for pneumonia and discharged. He returned one month later with persistent AMS. Brain MRI showed diffuse leptomeningeal enhancement. Lumbar puncture (LP) revealed lymphocytic pleocytosis, low glucose, and elevated protein. All infectious testing was negative. Transbronchial lung biopsy showed inflammation, but no organisms. He was discharged on itraconazole for presumed disseminated fungal infection. He returned with progressive mental status changes four months later. MRI and LP were unchanged, but CSF angiotensin-converting enzyme (ACE) was four times normal. Chest imaging again showed cavitary lesions, and CT guided lung biopsy revealed necrotizing granulomatous inflammation. Microbiological tests were again negative except one serum complement fixation (CF), which was positive for histoplasmosis. The patient was treated with amphotericin and discharged on itraconazole until he presented to us two months later. By this time, the patient had developed gait abnormalities and urinary incontinence. Imaging demonstrated progression of prior lesions. Both transbronchial lung and brain biopsies revealed necrotizing and non-necrotizing granulomas. All fungal tests including histoplasmosis fungal CF were negative. Serum ACE level was elevated and, considering prior elevated CSF ACE and granulomas in the lung and brain, sarcoidosis was diagnosed. The patient was treated with high dose steroids and his clinical status, along with lung and brain lesions, improved.

DISCUSSION: The elusiveness of this diagnosis in large part stemmed from the common diagnostic pitfalls of anchoring bias and reliance on general disease paradigm rather than specific clinical context. Despite the severity and progression of the patient’s symptoms on treatment, an incorrect diagnosis persisted on the basis of a single positive complement fixation test without microbiological confirmation. Additionally, the elevated CSF ACE was ignored in this unusual presentation of sarcoidosis. Although sarcoid is classically thought of as non-necrotizing, recent literature suggests sarcoidosis can cause a spectrum of granulomatous inflammatory tissue responses.

CONCLUSIONS: This case illustrates the importance of ongoing diagnostic re-evaluation with consideration of atypical presentations of rare diseases when faced with discordant laboratory data or unexpected clinical progression.

Reference #1: Rosen Y. Four decades of necrotizing sarcoid granulomatosis: what do we know now? Arch Pathol Lab Med. 2015;139(2):252-62.

DISCLOSURE: The following authors have nothing to disclose: Meredith Greer, Udayan Shah

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