Pulmonary Manifestations of SLE
Cheema GS, Quismorio FP. Interstitial lung disease in systemic lupus erythematosus.
Curr Op Pulm Med 2000; 6: 424-9.
Murray and Nadel: Textbook on Respiratory Medicine, 3rd ed.
Primer on the Rheumatic Diseases, 12th ed; Chapter 17: SLE.
• Pleurisy is the most common form of pulmonary disease in SLE
• Both acute lupus pneumonitis and diffuse alveolar hemorrhage are fairly uncommon but have a
• Chronic lupus pneumonitis/lupus-associated ILD resembles IPF clinically
• Don’t forget to exclude infection, infection, infection
Pleurisy: May or may not be associated with pleural rub and/or effusion. If effusion is
present, it will usually be an exudate with low complement, mildly low glucose, and high
LDH but low total protein.
• Prevalence in one series: 44% with pleurisy, 20% with effusion.
2. Acute lupus pneumonitis: Fairly uncommon (1-12% of patients).
• Presents with fever, cough +/- hemoptysis, SOB, bibasilar fluffy infiltrates
• ?Association with anti-dsDNA, anti-Ro/SSA antibodies
• Chest CT usually demonstrates ground glass infiltrates
• Pathologic findings are mixed but frequently include diffuse alveolar damage
• Mortality 50%; survivors left with restrictive lung disease
3. Chronic lupus pneumonitis: Sometimes follows an acute episode; prevalence < 5%
• Clinically similar to IPF with bibasilar crackles and infiltrates
• PFTs with restrictive pattern
• Chest CT may demonstrate a ground glass appearance (consistent with biopsy
showing cellular inflammation) or a reticulonodular appearance (consistent with
biopsy showing fibrotic pattern); this differentiation will help determine
4. Pulmonary HTN: 12-28% of patients with SLE
• Clinical presentation as with other pulmonary HTN patients
• Associated with Raynaud’s phenomenon, elevated RF titer, anti-RNP, and
5. “Shrinking lung” synd