Main differential diagnosis of cystic lung diseases
| Frequency# | [Ref.] | |
| Multiple cysts as main feature | ||
| Lymphangioleiomyomatosis (sporadic or associated with tuberous sclerosis) | ∼100% | [1] |
| Pulmonary Langerhans cell histiocytosis¶ | >90% | [2] |
| Birt–Hogg–Dubé syndrome; familial pneumothorax | >80% | [3] |
| Nonamyloid Ig deposition disease | [4] | |
| Pulmonary amyloidosis | [5] | |
| Primary and metastatic neoplasms | Rare | |
| Infections, e.g. Pneumocystis jirovecii | Rare | |
| Other, e.g. Niemann–Pick disease, neurofibromatosis, Ehlers–Danlos syndrome, Proteus syndrome, MALT lymphoma, Castleman disease, congenital pulmonary airway malformation, BPD, fire-eater's lung, hyper-IgE syndrome, Erdheim–Chester disease | ||
| Multiple cysts in interstitial lung disease | ||
| Lymphocytic interstitial pneumonia | >60% | [6, 7] |
| Hypersensitivity pneumonitis (subacute/chronic) | 13–39% | [8, 9] |
| Sjögren syndrome | 14–23% | [10, 11] |
| Desquamative interstitial pneumonia | 32% | [12] |
| Follicular bronchiolitis | [13] | |
| Other, e.g. ankylosing spondylitis, lymphomatoid granulomatosis |
Ig: immunoglobin; MALT: mucosa-associated lymphoid tissue; BPD: bronchopulmonary dysplasia. #: frequency of pulmonary cysts, when available; ¶: in the early stage of pulmonary Langerhans cell histiocytosis, a purely nodular pattern can be seen.