ILD Roundup 7/15/22

1. Necrobiotic rheumatoid nodules were discovered on biopsy in a patient with RA-ILD

What are the observed pulmonary manifestations of Rheumatoid arthritis?

Graingers and Allison’s Diagnostic Radiology, 9, 206-230.

What is epidemiology, clinical, radiologic, and pathologic criteria associated with necrobiotic rheumatoid nodules?

• Rare complication (1% with RA)
• More common in:
  • Men > Women
  • Long-standing disease
  • RF positivity
  • Active disease (esp cutaneous rheumatoid nodules’ also with joint disease, and elevated RA serologies)
• Morphology:
  • Single or multiple
  • mm to several cm in diameter
  • Well-circumscribed
  • May cavitate à leading to pneumothorax or bronchopleural fistula
  • May wax/wane
  • May be PET avid (although those >8 mm are not typically)
• Pulmonary nodules can paradoxically worsen with the start of RA therapy (most well observed with methotrexate)
• Nodules with upper/midzone predilection, located along interlobular septa or subpleural regions
• May cavitate (due to proteolytic enzymes) or rupture (leading to PTX or bronchopleural fistula)

European Respiratory Review 2015 24:1-16 (link)

Fishman’s Pulmonary Disease and Disorders, Chp 60, 5e.

In our case, a VATS biopsy was performed to rule out malignancy and infection as other causes of cavitating lung lesions.

2. Our second case featured a patient with history of Sjogren Syndrome (SS) with cystic lung disease. In this case, the mural thickening and nodularity of the cysts and presence of lymphadenopathy raised our concern for potential malignancy. Gabby Liu raised MALT lymphoma as a potential diagnosis.

What is the association between SS and MALT lymphoma?

• Among autoimmune disorders, SS is most strongly associated with the development of lymphoma
• In a small retrospective study describing biopsy-proven MALT lymphoma with lung involvement, 54% met criteria for SS

What are the commonly associated patterns of ILD in SS, and how are cystic lung diseases characterized and distinguished?

• Follicular bronchiolitis
• Lymphocytic interstitial pneumonia (LIP)
• Non-specific interstitial pneumonia (NSIP)
• Amyloidosis
• Lymphoproliferative disorders, including lymphoma

Clin Med Res. 2017; 15(1-2):6-12 (link)

AJRCCM 2015; 191(12) 1354-1666 (links 1 and 2 included below)

3. The third case involved a former smoker with CT findings of a probable UIP pattern with air trapping. The differential diagnosis included fibrotic hypersensitivity pneumonitis (fHP) vs IPF (i.e., CPFE). A bronchoscopy with BAL and lung biopsy were discussed as potential next steps for establishing diagnosis.

How is the diagnosis fHP arrived upon? How is fHP distinguished from other fibrotic ILDs?

AJRCCM 2020; 202(3):e36-e69. (link)