Sandbox: sadaf
Malabsorption |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2]
Overview
Classification
Pathophysiology
- Interstitial lung disease is a group of disorders that involve pulmonary parenchyma.
- The exact pathogenesis of these disorders are 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 and fibrosis pattern
- Granulomatous pattern
Differentiating Interstitial Lung Disease from other Diseases
To review the complete differential diagnosis of dyspnea, click here.
To review the complete differential diagnosis of hemoptysis, click here.
To review the complete differential diagnosis of restrictive lung disease, click here.
Disease | Clinical manifestation | Investigations | ||||||||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
History | Symptoms | Physical examination | Lab findings | Imaging | Pulmonary function test | Bronchoscopy and BAL | Gold standard | |||||||||||||||
Duration | Age | Gender | Family history | Smoking history | Environmental exposure | HIV | Dyspnea | Cough | Wheezing | Chest pain | Tachypnea | Auscultation | Cyanosis | Clubbing | Spirometry | ABG | ||||||
Idiopathic pulmonary fibrosis[1] | Chronic | 60−70 years old | Men | + | + | ± | − | + | Dry | + | + | + | − | + |
|
|
|
|
|
| ||
Idiopathic nonspecific interstitial pneumonia[2] | Acute/Chronic | 50−60 years old | Female | + | − | − | + | + | + | + | − | + |
|
− | ± |
|
|
|
|
| ||
Cryptogenic organising pneumonia[3] | Acute/subacute | 50−60 years old | Both | − | ± | − | − | + | Dry | − | − | − |
|
− | − |
|
|
|
| |||
Disease | History | Symptoms | Physical examination | Lab findings | Imaging | Pulmonary function test | Bronchoscopy and BAL | Gold standard | ||||||||||||||
Duration | Age | Gender | Family history | Smoking history | Environmental exposure | HIV | Dyspnea | Cough | Wheezing | Chest pain | Tachypnea | Auscultation | Cyanosis | Clubbing | Spirometry | ABG | ||||||
Acute interstitial pneumonia (Hamman−Rich syndrome)[4] | Acute | 50−60 years old | Both | − | − | − | − | + | + | − | − | + |
|
− | − |
|
|
|
| |||
Lymphocytic interstitial pneumonia[5] | Subacute | 30−40 years old | Female | − | − | − | ± | + | + | + | + | − |
|
− | + |
|
|
| ||||
Respiratory bronchiolitis−interstitial lung disease[6] | Subacute | 30−40 years old | Both | − | + | − | − | + | Dry | + | − | − | − | − |
|
|
|
| ||||
Desquamative interstitial pneumonia[7][8] | Chronic | 40−50 years old | Both | − | + | − | − | + | Dry | + | − | − |
|
− | − |
|
|
|
|
| ||
Disease | History | Symptoms | Physical examination | Lab findings | Imaging | Pulmonary function test | Bronchoscopy and BAL | Gold standard | ||||||||||||||
Duration | Age | Gender | Family history | Smoking history | Environmental exposure | HIV | Dyspnea | Cough | Wheezing | Chest pain | Tachypnea | Auscultation | Cyanosis | Clubbing | Spirometry | ABG | ||||||
Pulmonary Langerhans cell granulomatosis[9] | Chronic | 20−40 years old | Both | + | + | − | − | ± | Dry | + | + | − |
|
− | − |
|
|
|
|
|
| |
Pulmonary alveolar proteinosis[10][11] | Acute/chronic | 40−50 years old | Male | + | + | + | − | + | + | + | − | − |
|
+ | + |
|
|
|
|
| ||
Pulmonary lymphangioleiomyomatosis[12] | Acute/chronic | 30−40 years old | Female | + | + | − | − | + | Bloody | + | + | − |
|
− | + |
|
|
|
|
|
| |
Eosinophilic pneumonia[13] | Acute/chronic | 20−40 years old | Male | − | − | − | − | + | Dry | + | + | + |
|
− | − |
|
|
|
|
|
| |
Hypersensitivity pneumonitis[14] | Acute/subacute/chronic | 40−60 years old | Both | − | ± | + | − | + | Dry/productive | + | + | + |
|
− | + |
|
|
|
|
|
| |
Disease | History | Symptoms | Physical examination | Lab findings | Imaging | Pulmonary function test | Bronchoscopy and BAL | Gold standard | ||||||||||||||
Duration | Age | Gender | Family history | Smoking history | Environmental exposure | HIV | Dyspnea | Cough | Wheezing | Chest pain | Tachypnea | Auscultation | Cyanosis | Clubbing | Spirometry | ABG | ||||||
Occupational lung disease[15] | Chronic | Elderly | Male | + | + | + | − | ± | + | + | + | + |
|
Peripheral/central | + |
|
|
|
|
|
| |
Radiation−induced lung injury[16] | Subacute/chronic | Any age | Both | − | − | + | − | + | Dry | + | + | + |
|
+ | − |
|
|
|
|
|
| |
Pulmonary hemorrhage syndromes | Goodpasture syndrome[17] | Chronic | All ages | Male | + | ± | − | − | ± | Bloody | ± | − | − |
|
− | − |
|
|
|
|
|
|
Idiopathic pulmonary hemosiderosis[18] | Acute/subacute/chronic | Children − 10 years old | Both | + | ± | − | − | + | Bloody | + | + | − |
|
− | − |
|
|
|
|
|
| |
Isolated pulmonary capillaritis[19] | Chronic | 40−60 years old | Both | + | − | ± | − | + | Bloody | + | + | + |
|
− | − |
|
|
|
|
|
| |
Disease | History | Symptoms | Physical examination | Lab findings | Imaging | Pulmonary function test | Bronchoscopy and BAL | Gold standard | ||||||||||||||
Duration | Age | Gender | Family history | Smoking history | Environmental exposure | HIV | Dyspnea | Cough | Wheezing | Chest pain | Tachypnea | Auscultation | Cyanosis | Clubbing | Spirometry | ABG | ||||||
Sarcoidosis[20] | Acute/subacute/chronic | 20−40 years old | Female | + | ± | − | − | ± | + | + | ± | − |
|
+ | − |
|
|
|
|
|
| |
Granulomatous vasculitides | Granulomatosis with polyangiitis (Wegener)[21] | Chronic | Elderly | Both | + | − | − | − | + | + | + | ± | − |
|
− | − |
|
|
|
|
|
|
Eosinophilic granulomatosis with polyangiitis (Churg Strauss)[22] | Chronic | 40−50 years old | Both | + | − | − | − | − | + | + | − | − |
|
− | − |
|
|
|
|
|
| |
Bronchocentric granulomatosis[23] | Chronic | 30−70 years old | Both | − | − | − | − | ± | ± | + | ± | − |
|
− | − |
|
|
|
|
|
| |
Pulmonary lymphomatoid granulomatosis[24] | Chronic | 30−50 years old | Male | − | − | − | − | + | + | + | + | − |
|
− | − |
|
|
|
|
|
| |
Amyloidosis[25][26] | Subacute/chronic | 50−70 years old | Male | + | − | − | − | − | Bloody | + | − | − |
|
− | − |
|
|
|
|
|
| |
Disease | History | Symptoms | Physical examination | Lab findings | Imaging | Pulmonary function test | Bronchoscopy and BAL | Gold standard | ||||||||||||||
Duration | Age | Gender | Family history | Smoking history | Environmental exposure | HIV | Dyspnea | Cough | Wheezing | Chest pain | Tachypnea | Auscultation | Cyanosis | Clubbing | Spirometry | ABG |
Diagnosis
- There are multiple laboratory tests that might be helpful to ascertain or rule out the diagnosis of interstitial lung disease.
Condition | Disease | Test | |
---|---|---|---|
All patients with suspicious interstitial lung disease | Complete blood count and differential | ||
Liver function tests | Alanine aminotransferase (ALT, SGPT) | ||
Aspartate aminotransferase (AST, SGOT) | |||
Alkaline phosphatase | |||
Renal function tests | Urinalysis | ||
Blood urea nitrogen (BUN) | |||
Creatinine (Cr) | |||
Suspicious of systemic rheumatic disease | RA | Serology | Anti−cyclic citrullinated peptide (Anti−CCP) |
SLE | Serology | Anti−double stranded (ds) DNA antibodies | |
Amyopathic dermatomyositis | Serology | Anti−melanoma differentiation−associated gene 5 (MDA−5) | |
Nonspecific | Serology | Antinuclear antibody (ANA) | |
Serology | Rheumatoid factor (RF) | ||
Serology | Anti−neutrophil cytoplasmic antibody (ANCA) | ||
Enzyme | Creatine kinase (CK), aldolase | ||
Mechanic hands | Myositis | Myositis−associated antibodies | Anti−tRNA synthetases Jo−1 |
Anti−tRNA synthetases PL−7 | |||
Anti−tRNA synthetases PL−12 | |||
Sicca features or positive anti−extractable nuclear antigen (ENA) | Sjögren’s syndrome | Serology | Anti−RO (SS−A) |
Serology | Anti−La (SS−B) | ||
Mixed connective tissue disease | Serology | Anti−ribonucleoprotein (RNP) | |
IgG4−related disease | Serology | Serum IgG4 | |
Severe GERD or sclerodactyly | Limited systemic scleroderma | Serology | Anti−centromere |
Systemic scleroderma | Serology | Anti−topoisomerase I (anti−Scl−70) | |
Dyspnea | Heart failure | Enzyme | Brain natriuretic peptide (BNP) |
Pulmonary hypertension | N−terminal proBNP (NT−proBNP) | ||
Anemia and/or hemoptysis | Coagulopathies | Coagulation studies | |
Goodpasture syndrome | Serology | Anti−glomerular basement membrane (GBM) antibodies | |
Antiphospholipid syndrome | Serology | Antiphospholipid antibodies | |
Idiopathic pulmonary hemosiderosis | Serology | Serum IgA endomysial or tissue transglutaminase antibodies | |
Mediastinal lymphadenopathy | Multiple myeloma | Serum protein electrophoresis | |
Beryllium exposure | Berylliosis | Peripheral blood beryllium lymphocyte proliferation test | |
Risk factors for HIV | HIV | ELISA | |
Western blot test |
Occupational lung disease
- Occupational lung diseases are caused by the accumulation of different dust particles in the alveolar space.[27]
- As the particles accumulate, the body's elimination mechanisms begin to fail, resulting in activation of chemotactic factors that exacerbate the inflammatory response, and subsequently lead to fibrosis.
- The most common particles that cause pneumoconiosis are asbestos, silica, coal, magnesium silicate, aluminum silicate, bauxite, cobalt, beryllium and iron.
For more information about occupational lung disease, click here.
Drug−induced lung injury
- Medications that might cause interstitial lung disease include:[28]
Antimicrobial Agents | Anti−Inflammatory Agents | Biological Agents | Cardiovascular Agents | Immunomodulator agents | Antineoplastic agents | Miscellaneous |
---|---|---|---|---|---|---|
|
|
|
|
|
|
Radiation−induced lung injury
- Pulmonary injury following radiation is directly related to duration and dose of radiation.
- There are early and late reaction in pulmonary tissues to radiation.
- Early pulmonary reaction to radiation usually occurs in 4 to 12 weeks following irradiation.
- Late fibrotic response usually occurs 6 to 12 months following irradiation.
- Desquamative interstitial pneumonia
- Respiratory bronchiolitis–associated interstitial lung disease
- Pulmonary Langerhans cell granulomatosis
Idiopathic interstitial pneumonias
- Major idiopathic interstitial pneumonias
- Idiopathic pulmonary fibrosis
- Idiopathic nonspecific interstitial pneumonia
- Respiratory bronchiolitis−interstitial lung disease
- Desquamative interstitial pneumonia
- Cryptogenic organising pneumonia
- Acute interstitial pneumonia
- Rare idiopathic interstitial pneumonias
- Idiopathic lymphoid interstitial pneumonia
- Idiopathic pleuroparenchymal fibroelastosis
- Unclassifiable idiopathic interstitial pneumonias
- For more information about Idiopathic interstitial pneumonia, click here.
- For more information about Cryptogenic organizing pneumonia, click here.
- For more information about idiopathic pulmonary fibrosis, click here.
Pulmonary alveolar proteinosis
For more information about pulmonary alveolar proteinosis, click here.
Lymphocytic infiltrative disorders
- Lymphocytic interstitial pneumonitis
- Pulmonary lymphomatoid granulomatosis
For more information about lymphocytic interstitial pneumonitis, click here.
Pulmonary lymphangioleiomyomatosis
- 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 connective tissue diseases
- Systemic lupus erythematosus
- Rheumatoid arthritis
- Ankylosing spondylitis
- Systemic sclerosis
- Sjögren syndrome
- Polymyositis/dermatomyositis
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
- ↑ Poletti, Venerino; Ravaglia, Claudia; Buccioli, Matteo; Tantalocco, Paola; Piciucchi, Sara; Dubini, Alessandra; Carloni, Angelo; Chilosi, Marco; Tomassetti, Sara (2013). "Idiopathic Pulmonary Fibrosis: Diagnosis and Prognostic Evaluation". Respiration. 86 (1): 5–12. doi:10.1159/000353580. ISSN 1423-0356.
- ↑ Travis, William D.; Hunninghake, Gary; King, Talmadge E.; Lynch, David A.; Colby, Thomas V.; Galvin, Jeffrey R.; Brown, Kevin K.; Chung, Man Pyo; Cordier, Jean-François; du Bois, Roland M.; Flaherty, Kevin R.; Franks, Teri J.; Hansell, David M.; Hartman, Thomas E.; Kazerooni, Ella A.; Kim, Dong Soon; Kitaichi, Masanori; Koyama, Takashi; Martinez, Fernando J.; Nagai, Sonoko; Midthun, David E.; Müller, Nestor L.; Nicholson, Andrew G.; Raghu, Ganesh; Selman, Moisés; Wells, Athol (2008). "Idiopathic Nonspecific Interstitial Pneumonia". American Journal of Respiratory and Critical Care Medicine. 177 (12): 1338–1347. doi:10.1164/rccm.200611-1685OC. ISSN 1073-449X.
- ↑ Mehrian, P.; Doroudinia, A.; Rashti, A.; Aloosh, O.; Dorudinia, A. (2017). "High-resolution computed tomography findings in chronic eosinophilic vs. cryptogenic organising pneumonia". The International Journal of Tuberculosis and Lung Disease. 21 (11): 1181–1186. doi:10.5588/ijtld.16.0723. ISSN 1027-3719.
- ↑ Parambil, Joseph; Mukhopadhyay, Sanjay (2012). "Acute Interstitial Pneumonia (AIP): Relationship to Hamman-Rich Syndrome, Diffuse Alveolar Damage (DAD), and Acute Respiratory Distress Syndrome (ARDS)". Seminars in Respiratory and Critical Care Medicine. 33 (05): 476–485. doi:10.1055/s-0032-1325158. ISSN 1069-3424.
- ↑ Panchabhai, Tanmay S.; Farver, Carol; Highland, Kristin B. (2016). "Lymphocytic Interstitial Pneumonia". Clinics in Chest Medicine. 37 (3): 463–474. doi:10.1016/j.ccm.2016.04.009. ISSN 0272-5231.
- ↑ Sieminska, Alicja; Kuziemski, Krzysztof (2014). "Respiratory bronchiolitis-interstitial lung disease". Orphanet Journal of Rare Diseases. 9 (1). doi:10.1186/s13023-014-0106-8. ISSN 1750-1172.
- ↑ Ryu, Jay H.; Myers, Jeffrey L.; Capizzi, Stephen A.; Douglas, William W.; Vassallo, Robert; Decker, Paul A. (2005). "Desquamative Interstitial Pneumonia and Respiratory Bronchiolitis-Associated Interstitial Lung Disease". Chest. 127 (1): 178–184. doi:10.1378/chest.127.1.178. ISSN 0012-3692.
- ↑ Craig, P J; Wells, A U; Doffman, S; Rassl, D; Colby, T V; Hansell, D M; du Bois, R M; Nicholson, A G (2004). "Desquamative interstitial pneumonia, respiratory bronchiolitis and their relationship to smoking". Histopathology. 45 (3): 275–282. doi:10.1111/j.1365-2559.2004.01921.x. ISSN 0309-0167.
- ↑ Blakley, Matthew P.; Dutcher, Janice P.; Wiernik, Peter H. (2018). "Pulmonary Langerhans cell histiocytosis, acute myeloid leukemia, and myelofibrosis in a large family and review of the literature". Leukemia Research. 67: 39–44. doi:10.1016/j.leukres.2018.01.011. ISSN 0145-2126.
- ↑ Carrington JM, Hershberger DM. PMID 29493933. Missing or empty
|title=
(help) - ↑ Kiani, Arda; Parsa, Tahereh; Adimi Naghan, Parisa; Dutau, Hervé; Razavi, Fatemeh; Farzanegan, Behrooz; Pourabdollah Tootkaboni, Mahsa; Abedini, Atefeh (2018). "An eleven-year retrospective cross-sectional study on pulmonary alveolar proteinosis". Advances in Respiratory Medicine. 86 (1): 7–12. doi:10.5603/ARM.2018.0003. ISSN 2543-6031.
- ↑ Xu, Kai-Feng; Lo, Bee Hong (2014). "Lymphangioleiomyomatosis: differential diagnosis and optimal management". Therapeutics and Clinical Risk Management: 691. doi:10.2147/TCRM.S50784. ISSN 1178-203X.
- ↑ Bernheim, Adam; McLoud, Theresa (2017). "A Review of Clinical and Imaging Findings in Eosinophilic Lung Diseases". American Journal of Roentgenology. 208 (5): 1002–1010. doi:10.2214/AJR.16.17315. ISSN 0361-803X.
- ↑ Miller, Ross; Allen, Timothy Craig; Barrios, Roberto J.; Beasley, Mary Beth; Burke, Louise; Cagle, Philip T.; Capelozzi, Vera Luiza; Ge, Yimin; Hariri, Lida P.; Kerr, Keith M.; Khoor, Andras; Larsen, Brandon T.; Mark, Eugene J.; Matsubara, Osamu; Mehrad, Mitra; Mino-Kenudson, Mari; Raparia, Kirtee; Roden, Anja Christiane; Russell, Prudence; Schneider, Frank; Sholl, Lynette M.; Smith, Maxwell Lawrence (2018). "Hypersensitivity Pneumonitis A Perspective From Members of the Pulmonary Pathology Society". Archives of Pathology & Laboratory Medicine. 142 (1): 120–126. doi:10.5858/arpa.2017-0138-SA. ISSN 0003-9985.
- ↑ Sirajuddin, Arlene; Kanne, Jeffrey P. (2009). "Occupational Lung Disease". Journal of Thoracic Imaging. 24 (4): 310–320. doi:10.1097/RTI.0b013e3181c1a9b3. ISSN 0883-5993.
- ↑ Giridhar P, Mallick S, Rath GK, Julka PK (2015). "Radiation induced lung injury: prediction, assessment and management". Asian Pac. J. Cancer Prev. 16 (7): 2613–7. PMID 25854336.
- ↑ DeVrieze BW, Hurley JA. PMID 29083697. Missing or empty
|title=
(help) - ↑ Khorashadi, L.; Wu, C.C.; Betancourt, S.L.; Carter, B.W. (2015). "Idiopathic pulmonary haemosiderosis: spectrum of thoracic imaging findings in the adult patient". Clinical Radiology. 70 (5): 459–465. doi:10.1016/j.crad.2014.11.007. ISSN 0009-9260.
- ↑ Thompson, Gwen; Klecka, Mary; Roden, Anja C.; Specks, Ulrich; Cartin-Ceba, Rodrigo (2016). "Biopsy-proven pulmonary capillaritis: A retrospective study of aetiologies including an in-depth look at isolated pulmonary capillaritis". Respirology. 21 (4): 734–738. doi:10.1111/resp.12738. ISSN 1323-7799.
- ↑ Li, Cheng-Wei; Tao, Ru-Jia; Zou, Dan-Feng; Li, Man-Hui; Xu, Xin; Cao, Wei-Jun (2018). "Pulmonary sarcoidosis with and without extrapulmonary involvement: a cross-sectional and observational study in China". BMJ Open. 8 (2): e018865. doi:10.1136/bmjopen-2017-018865. ISSN 2044-6055.
- ↑ Greco A, Marinelli C, Fusconi M, Macri GF, Gallo A, De Virgilio A, Zambetti G, de Vincentiis M (June 2016). "Clinic manifestations in granulomatosis with polyangiitis". Int J Immunopathol Pharmacol. 29 (2): 151–9. doi:10.1177/0394632015617063. PMC 5806708. PMID 26684637.
- ↑ Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, Altissimi G, De Vincentiis M (April 2015). "Churg-Strauss syndrome". Autoimmun Rev. 14 (4): 341–8. doi:10.1016/j.autrev.2014.12.004. PMID 25500434.
- ↑ Myers, Jeffrey L. (1989). "Bronchocentric Granulomatosis". Chest. 96 (1): 3–4. doi:10.1378/chest.96.1.3. ISSN 0012-3692.
- ↑ Ankita, Grover; Shashi, Dhawan (2016). "Pulmonary Lymphomatoid Granulomatosis- a Case Report with Review of Literature". Indian Journal of Surgical Oncology. 7 (4): 484–487. doi:10.1007/s13193-016-0525-1. ISSN 0975-7651.
- ↑ Khoor, Andras; Colby, Thomas V. (2017). "Amyloidosis of the Lung". Archives of Pathology & Laboratory Medicine. 141 (2): 247–254. doi:10.5858/arpa.2016-0102-RA. ISSN 0003-9985.
- ↑ Milani, Paolo; Basset, Marco; Russo, Francesca; Foli, Andrea; Palladini, Giovanni; Merlini, Giampaolo (2017). "The lung in amyloidosis". European Respiratory Review. 26 (145): 170046. doi:10.1183/16000617.0046-2017. ISSN 0905-9180.
- ↑ Castranova V, Vallyathan V (2000). "Silicosis and coal workers' pneumoconiosis". Environ Health Perspect. 108 Suppl 4: 675–84. PMC 1637684. PMID 10931786.
- ↑ Schwaiblmair, Martin (2012). "Drug Induced Interstitial Lung Disease". The Open Respiratory Medicine Journal. 6 (1): 63–74. doi:10.2174/1874306401206010063. ISSN 1874-3064.