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Pulmonary lymphangioleiomyomatosis

Lymphangiomyocytosis is defined as a multifocal neoplasm with differentiation of the perivascular epithelioid cell that involves multiple organs.^[1]

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

Goodpasture syndrome
Idiopathic pulmonary hemosiderosis
Isolated pulmonary capillaritis

Granulomatous lung response

Hypersensitivity pneumonitis
Sarcoidosis
Granulomatous vasculitides
- Granulomatosis with polyangiitis (Wegener)
- Eosinophilic granulomatosis with polyangiitis (ChurgStrauss)
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

References

↑ Verma AK, Joshi A, Mishra AR, Kant S, Singh A (2018). "Pulmonary lymphangioleiomyomatosis presenting as spontaneous pneumothorax treated with sirolimus - A case report". Lung India. 35 (2): 154–156. doi:10.4103/lungindia.lungindia_60_17. PMID 29487252.

[pmid29487252-1] Verma AK, Joshi A, Mishra AR, Kant S, Singh A (2018). "Pulmonary lymphangioleiomyomatosis presenting as spontaneous pneumothorax treated with sirolimus - A case report". Lung India. 35 (2): 154–156. doi:10.4103/lungindia.lungindia_60_17. PMID 29487252.

[1]

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-Interstitial lung disease is a group of disorders that involve pulmonary [[parenchyma]]. The exact pathogenesis of these disorders is not fully understood. There are multiple initiating factors that cause [[pulmonary]] injury. However, immunopathogenic responses of [[lung]] tissue are quite similar. There are two major histopathologic patterns in response to [[lung]] injury which include [[inflammation]], [[fibrosis]] and [[Granuloma|granulomatous]] response. Interstitial lung disease may be classified into several subtypes based on the lung response to [[tissue]] injury and their causes. The exact underlying cause of interstitial lung disease might be well known such as toxic [[Environmental Health Perspectives|environmental]] or [[Occupational Health|occupational]] exposure, [[Smoking|cigarette smoking]], and [[Radiation therapy|radiation]]. The other causes of interstitial lung disease are idiopathic.
-__NOTOC__
-== Pulmonary alveolar proteinosis ==
-Pulmonary alveolar proteinosis (PAP) is a rare lung disease that is characterized by the intra-alveolar accumulation of [[Pulmonary surfactant|surfactant]] phospholipid and [[apoproteins]].
-* The main pathogenesis of pulmonary alveolar proteinosis is a reduction in [[granulocyte-macrophage colony-stimulating factor]] ([[Granulocyte macrophage colony stimulating factor|GM-CSF]]) levels or function and/or impaired alveolar [[macrophage]] function.
-*  Terminal [[Bronchiole|bronchioles]] and [[Pulmonary alveolus|alveoli]] are filled with a lipoproteinaceous material that will be [[Periodic acid-Schiff stain|periodic acid-Schiff]] ([[Periodic acid-Schiff stain|PAS]]) stain positive.
-* The exact etiology of pulmonary alveolar proteinosis is unknown.
-* If left untreated, patients with pulmonary alveolar proteinosis may progress to develop pulmonary [[fibrosis]] or [[Right heart failure|cor pulmonale]].
-'''For more information about pulmonary alveolar proteinosis, [[Pulmonary alveolar proteinosis|click here]].'''
-== Lymphocytic infiltrative disorders ==
-Lymphocytic infiltrative disorders might cause interstitial lung disease in mostly [[Human Immunodeficiency Virus (HIV)|HIV]] positive children.
-* There are two main manifestation of lymphocytic infiltrative disorders that include:
-** [[Lymphocytic interstitial pneumonia|Lymphocytic interstitial pneumonitis]]
-** Pulmonary lymphomatoid granulomatosis
-* The etiology of lymphocytic infiltrative disorders is unknown. However, there is an evidence of infectious cause such as [[Epstein Barr virus|EBV]] in [[Human Immunodeficiency Virus (HIV)|HIV]] positive patients.
-'''For more information about lymphocytic interstitial pneumonitis, [[Lymphocytic interstitial pneumonitis|click here]].'''
 == Pulmonary lymphangioleiomyomatosis ==
+Lymphangiomyocytosis is defined as a multifocal neoplasm with differentiation of the perivascular epithelioid cell that involves multiple organs.<ref name="pmid29487252">{{cite journal |vauthors=Verma AK, Joshi A, Mishra AR, Kant S, Singh A |title=Pulmonary lymphangioleiomyomatosis presenting as spontaneous pneumothorax treated with sirolimus - A case report |journal=Lung India |volume=35 |issue=2 |pages=154–156 |date=2018 |pmid=29487252 |doi=10.4103/lungindia.lungindia_60_17 |url=}}</ref>
+* Lymphangiomyomatosis is the result of disorderly [[smooth muscle]] proliferation throughout the [[Bronchiole|bronchioles]], [[Alveolus|alveolar]] septa, perivascular spaces, and [[Lymphatic system|lymphatics]], resulting in the obstruction of small airways (leading to pulmonary [[cyst]] formation and [[pneumothorax]]) and [[Lymphatic system|lymphatics]] (leading to [[Chyle|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, [[Lymphangiomyomatosis|click here]].'''