Endogenous Lipoid Pneumonia in Adult Autoinflammatory Disease

Area of Science:

• Immunology
• Pulmonology
• Rheumatology

Background:

• Endogenous lipoid pneumonia (ELP) is rarely described in adult autoinflammatory diseases.
• Adult-onset Still disease (AOSD) and tumor necrosis factor receptor-associated periodic syndrome (TRAPS) are autoinflammatory conditions with potential for systemic complications.
• Lung involvement in autoinflammatory diseases involves complex interactions between innate and adaptive immunity.

Purpose of the Study:

• To report a rare case of ELP in an adult with AOSD and a genetic background of TRAPS.
• To elucidate the pathophysiology of lung disease in the context of refractory autoinflammatory conditions.
• To review existing literature on ELP in adult autoinflammatory diseases.

Main Methods:

• Case report of a 53-year-old immunocompromised woman with refractory autoinflammatory disease and macrophage activation syndrome (MAS).
• Diagnostic work-up included chest CT, bronchoscopy with bronchoalveolar lavage, and lung biopsy.
• Literature review of ELP in autoinflammatory diseases (AOSD, TRAPS, soJIA) using PubMed.

Main Results:

• The patient presented with dyspnea and fever, showing diffuse lung disease on CT.
• Lung biopsy confirmed ELP with cholesterol crystals and foamy macrophages.
• The patient had a history of treatment-refractory autoinflammatory disease, including MAS, and responded to corticosteroids, cyclophosphamide, and tacrolimus, but retained interstitial lung disease.

Conclusions:

• This case underscores the complex interplay of immune systems in autoinflammatory lung disease.
• ELP is a potential, albeit rare, manifestation in adults with autoinflammatory conditions like AOSD.
• Aggressive immunosuppressive therapy may be required for severe lung manifestations in these patients.