Sandbox: sadaf

Pulmonary lymphangioleiomyomatosis

Lymphangiomyocytosis is defined as a multifocal neoplasm with differentiation of the perivascular epithelioid cell that involves multiple organs.

• Lymphangiomyomatosis is the result of disorderly smooth muscle proliferation throughout the bronchioles, alveolar septa, perivascular spaces, and lymphatics, resulting in the obstruction of small airways (leading to pulmonary cyst formation and pneumothorax) and lymphatics (leading to chylous pleural effusion).
• LAM occurs in a sporadic form, which only affects females, who are usually of childbearing age.

For more information about pulmonary lymphangioleiomyomatosis, click here.

Pulmonary hemorrhage syndromes

Pulmonary hemorrhage syndromes might cause interstitial lung disease.

• There are several pulmonary hemorrhage syndromes that affect the lung parenchyma and eventually lead to pulmonary fibrosis. Some of them are as follow:
  • Goodpasture syndrome
  • Idiopathic pulmonary hemosiderosis
  • Isolated pulmonary capillaritis

Granulomatous lung response

• Hypersensitivity pneumonitis
• Sarcoidosis
• Granulomatous vasculitides
  • Granulomatosis with polyangiitis (Wegener)
  • Eosinophilic granulomatosis with polyangiitis (Churg Strauss)
• Bronchocentric granulomatosis

For more information about hypersensitivity pneumonitis, click here.

Interstitial lung disease associated with systemic diseases

Interstitial lung disease associated with connective tissue diseases

• Systemic lupus erythematosus
• Rheumatoid arthritis
• Ankylosing spondylitis
• Systemic sclerosis
• Sjögren syndrome
• Polymyositis/dermatomyositis
• Granulomatosis with polyangiitis (Wegener)
• Eosinophilic granulomatosis with polyangiitis (Churg Strauss)

Interstitial lung disease associated with inherited diseases

• Tuberous sclerosis
• Neurofibromatosis
• Niemann−Pick disease
• Gaucher disease
• Hermansky−Pudlak syndrome

Interstitial lung disease associated with gastrointestinal or liver diseases

• Crohn disease
• Primary biliary cirrhosis
• Chronic active hepatitis
• Ulcerative colitis

Interstitial lung disease associated with graft−versus−host disease

• Bone marrow transplantation
• Solid organ transplantation