CTICasesPulmonary Alveolar Proteinosis (PAP) with Atypical Consolidative Appearance
Imaging findings
Chest radiograph demonstrates diffuse lung disease with striking peripheral sparing. High-resolution CT shows extensive, dense consolidative opacities, geographic ground-glass attenuation, and focal areas of crazy-paving. Multiple unusual, cystic, and lucent holes are scattered within the dense consolidations in the absence of lymphadenopathy or effusions. Serology was positive for serum anti-GM-CSF autoantibodies.
Key takeaways
Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by the accumulation of surfactant within the alveoli. Although classically presenting as a symmetric, geographic 'crazy-paving' pattern, PAP can present atypically with dense consolidation and intra-lesional cystic or lucent spaces, mimicking infectious or fibrotic processes. The presence of positive anti-GM-CSF autoantibodies is diagnostic of autoimmune PAP.
AI-assisted summary — may contain errors. Verify against the source video. Learn more
More from this webinar
- Explosive Pleuritis (Streptococcus anginosus Empyema)Infection
- Traumatic Right Hemidiaphragm RuptureTrauma
- Antisynthetase Syndrome (Anti-EJ/glycyl-tRNA synthetase)ILD
- Antisynthetase Syndrome (Anti-Jo-1)ILD
- Bilateral Diaphragmatic Paralysis Post-Heart TransplantIatrogenic
- Mycobacterium xenopi Pulmonary Infection with Chest Wall InvasionInfection
- Naganishia (Cryptococcus) Pulmonary InfectionInfection
- Left Lower Lobe Atelectasis secondary to Bronchial CarcinoidNeoplastic
See all cases from September 6, 2021 →
Related Autoimmune cases
- End-Stage Sarcoidosis with Anterior Pneumothorax
- Sarcoidosis Presenting with Uveitis and a Miliary Nodular Pattern
- ANCA-Associated Vasculitis (Microscopic Polyangiitis) Presenting with Diffuse Alveolar Hemorrhage
- Rheumatoid Arthritis-Associated Constrictive Bronchiolitis
- Graves' Disease-Associated True Thymic Hyperplasia
- Giant Left Atrium Secondary to Rheumatic Heart Disease