ILD Roundup 10/11/22

This week, we discussed the case of a 48 yo with chronic cough who presented to care for evaluation of abnormal CT chest, which showed multifocal peripheral and peribronchovascular pulmonary nodules.

I. How can we use distribution of nodules to narrow differential diagnosis? 

Back to our patient! Using our knowledge and the peribronchovascular/subpleural distribution of nodules, a focused differential diagnosis was discussed (sarcoidosis, silicosis/coal-workers pneumoconiosis, lymphangitic carcinomatosis). Absent occupational exposures were noted, and lack of associated adenopathy/effusions/extrathoracic disease was discussed. Bronchoscopy with EBUS/TBNA and TBBx was performed. A representative sample of the transbronchial biopsy is shown below:

Transbronchial biopsy showing tightly packed non-caseating granulomas with partial hyalinization in a peribronchial distribution compatible with nummular sarcoidosis

 

Based on the CT and biopsy findings, the group arrived upon a diagnosis of nummular sarcoidosis. Given relatively mild symptoms and normal pulmonary function tests, Dr. Russell suggested the use of high dose inhaled steroids.  

II. What is the role of inhaled corticosteroids (ICS) in the management of sarcoidosis?

The rationale for use? Sarcoidosis is a disease process that follows a lymphatic distribution in the lungs – ICS targets the endobronchial lymphatics. In short, the data is limited! A Cochrane review of corticosteroid use in sarcoidosis included 7 studies that assessed ICS (budesonide 800-1600 mcg/d or fluticasone 880-2000 mcg/d) use, specifically in patients with stage 1 & 2 disease. No improvements in CXR or PFTs were observed, although one study showed a modest improvement in DLCO/VA and others reported improvement in symptom scores.  

III. What are the extra-pulmonary screening recommendations in sarcoidosis? 

Sources:

  • Chest 2006; 129(3):805-15. (link) 
  • Cochrane Database Syst Rev. 2005; 2005(2): CD001114. (link) 
  • AJRCCM 2020; 201(8):e26-e51. (link) 

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