Authors Benjamin Gerke1, MD; Pulmonary and Critical Care Fellow, University of Arizona. Division of Pulmonary and Critical Care Medicine Mei So 1, MD; Internal Medicine Resident, University of Arizona. Department of Medicine Swathy Puthalapattu 2, MD; Clinical Assistant Professor, University of Arizona. Division of Pulmonary and Critical Care Medicine
Case
51-year -old man with a history of hypertension, prediabetes,
presented to emergency room with symptoms of subjective fevers for
several months, fatigue, dry cough, shortness of breath, and
unintentional weight loss of 12 lbs. in the past 2 months.
Representative images of the CT chest done in emergency department are
shown below.
Figure 1. Axial images of chest CT.
Figure 2. Anterior segment of right upper lobe (RUL) on bronchoscopic
examination.
Question
What is the most likely diagnosis?
A. Mycobacterial infection B. Hypersensitivity pneumonitis C. Sarcoidosis D. Inflammatory bowel disease
Answer
C. Sarcoidosis with endobronchial involvement
Discussion
Sarcoidosis is a diagnosis of exclusion and should be diagnosed based
on clinical, radiographic, and pathologic findings. Axial mediastinal
CT images from Figure 1 show significant mediastinal and hilar
lymphadenopathy. Lung windows from Figure 1 demonstrate classic peri
lymphatic nodules predominantly along broncho-vascular bundles in the
right upper lobe (RUL) as well as sub-pleural and interlobar fissure
nodules. Endobronchial ultrasound (EBUS) guided biopsies of lymph nodes
and RUL “cobble stoning” showed epithelioid granulomas (Figure 3).
Bronchoalveolar lavage (BAL) cultures and biopsies were negative for
acid-fast-bacillus and fungal organisms. BAL cell count from RUL showed
predominance of neutrophils (54%) and lymphocytes (27%). Liver function
tests, serum angiotensin converting enzyme level, and calcium level were
unremarkable. Immunoglobulin levels and autoimmune studies were within
normal limits. Pulmonary function test results and electrocardiogram
results were normal as well. Patient was treated with 20 mg of
prednisone daily which was weaned off over a period of 6 months. Patient
had improvement in lymphadenopathy and lung nodules on repeat imaging.
Patient is maintained on inhaled budesonide therapy with good response.
Figure 3: Diff-Quik stain of lymph node
Differential diagnosis of sarcoidosis includes both infectious and noninfectious causes of granulomatous disorders 1.
Sarcoidosis can affect both upper and lower respiratory tract. Lower
respiratory tract involvement is associated with bronchial or less
commonly tracheal stenosis, bronchiectasis, cobble-stone appearance from
sub-mucosal infiltration of granulomas (Figure 2) as well as
bronchiolitis and air trapping with or without associated airway
hyperreactivity 2. Mycobacterial infection is unlikely in
this case, the patient had negative acid-fast-bacillus and fungal
organisms as well as absence of caseous necrosis on histology and
generally mycobacterial infection presents with centrilobular or random
distribution of nodules (Answer A is incorrect). Patient did not have
significant exposure history and typical CT findings in acute or
sub-acute hypersensitivity pneumonitis include patchy ground-glass
opacities, nodular opacities and ground glass nodules distributed in a
centrilobular fashion (Answer B is incorrect). Pulmonary involvement in
inflammatory bowel disease is rare and presents as pulmonary vasculitis,
interstitial pneumonitis, and airway involvement in the form of
ulcerations, stenosis, air trapping from small airway disease,
bronchitis, and bronchiectasis (Answer D is incorrect) 2.
In the era of EBUS, several studies including an RCT with 117 sarcoid
patients found the yield of EBUS guided lymph node biopsy of 74.5%
which was similar to previously reported sensitivities of pooled
observational data and smaller RCTs 4. Adding endobronchial
biopsy as adjunct to EBUS did not significantly improve the yield in
this study, possibly due to the low number of patients with visible
endobronchial involvement on bronchoscopy. The same group previously
published prospective observational data of 125 biopsy proven
sarcoidosis patients showing higher yield for endobronchial biopsy in
those with targetable mucosal involvement vs those with normal airways
biopsied systematically at secondary carina (75.8% vs. 41.7% positivity,
P<0.05) 5. Endobronchial biopsy, especially in those
with identifiable mucosal abnormalities, remains a valid consideration
due to simplicity of attainment and relatively little risk of harm.
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