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Diffuse interstitial pulmonary fibrosis

MedGen UID:
39340
•Concept ID:
C0085786
•
Disease or Syndrome
Synonyms: Acute Interstitial Pneumonia; Acute Interstitial Pneumonias; Acute Interstitial Pneumonitides; Acute Interstitial Pneumonitis; Hamman Rich Disease; Hamman Rich Syndrome; Hamman-Rich Disease; Hamman-Rich Syndrome; Interstitial Pneumonia, Acute; Interstitial Pneumonitis, Acute; Pneumonia, Acute Interstitial; Pneumonitis, Acute Interstitial
SNOMED CT: Usual interstitial pneumonitis (196125002); UIP - Usual interstitial pneumonitis (196125002); Hamman Rich syndrome (236302005); Diffuse interstitial pulmonary fibrosis (196125002); DIPF - Diffuse interstitial pulmonary fibrosis (196125002); Fibrosing alveolitis (196125002)
 
Related genes: SFTPA2, MUC5B, RTEL1, TERT, SFTPC, PARN

Definition

Acute idiopathic interstitial pneumonitis characterized by diffuse PULMONARY ALVEOLI damage with uniform edematous connective tissue proliferation. It is often associated with extensive fibroblastic distortion of the lung parenchyma and leads to ADULT RESPIRATORY DISTRESS SYNDROME in later stages. [from MeSH]

Recent clinical studies

Etiology

HLA association with sarcoidosis and diffuse interstitial pulmonary fibrosis.
Mahbubani V, Trikannad VS, Sainani GS
J Assoc Physicians India 1995 Jan;43(1):28-9. PMID: 9282635
Lung volume, diffusing capacity, chest roentgenogram and dyspnea index in interstitial lung disease.
Yang SC, Wu HD, Yang SP
J Formos Med Assoc 1991 Mar;90(3):244-9. PMID: 1677399
Barotrauma and air embolism in hyperbaric oxygen therapy.
Wolf HK, Moon RE, Mitchell PR, Burger PC
Am J Forensic Med Pathol 1990 Jun;11(2):149-53. doi: 10.1097/00000433-199006000-00009. PMID: 2343842
Asbestos-related benign disease and cancer: symptoms and treatment.
Gruber UF
Anticancer Drugs 1990 Dec;1(2):187-97. doi: 10.1097/00001813-199012000-00012. PMID: 2151850
Diffuse interstitial pulmonary fibrosis and lung cancer.
Kawai T, Yakumaru K, Suzuki M, Kageyama K
Acta Pathol Jpn 1987 Jan;37(1):11-9. doi: 10.1111/j.1440-1827.1987.tb03130.x. PMID: 3033984

Diagnosis

Imaging diagnosis of pneumoconiosis with predominant nodular pattern: HRCT and pathologic findings.
Akira M, Suganuma N
Clin Imaging 2023 May;97:28-33. Epub 2023 Feb 18 doi: 10.1016/j.clinimag.2023.02.010. PMID: 36878176
Radiologic Diagnosis of Asbestosis in Korea.
Cha YK, Kim JS, Kim Y, Kim YK
Korean J Radiol 2016 Sep-Oct;17(5):674-83. Epub 2016 Aug 23 doi: 10.3348/kjr.2016.17.5.674. PMID: 27587956Free PMC Article
Methotrexate-induced diffuse interstitial pulmonary fibrosis.
Bedrossian CW, Miller WC, Luna MA
South Med J 1979 Mar;72(3):313-8. doi: 10.1097/00007611-197903000-00023. PMID: 424824
Open lung biopsy.
Krákora P, Rehák F
Bronchopneumologie 1976 Sep-Oct;26(5):438-41. PMID: 1009485
Diffuse interstitial pulmonary fibrosis (cryptogenic fibrosing alveolitis).
Kunstling TR, Goodwin RA Jr, Des Prez RM
South Med J 1976 Apr;69(4):479-84, 487. doi: 10.1097/00007611-197604000-00029. PMID: 772819

Therapy

Radiologic Diagnosis of Asbestosis in Korea.
Cha YK, Kim JS, Kim Y, Kim YK
Korean J Radiol 2016 Sep-Oct;17(5):674-83. Epub 2016 Aug 23 doi: 10.3348/kjr.2016.17.5.674. PMID: 27587956Free PMC Article
Smoking and pulmonary fibrosis.
Weiss W
J Occup Med 1988 Jan;30(1):33-9. PMID: 3280757
Methotrexate-induced diffuse interstitial pulmonary fibrosis.
Bedrossian CW, Miller WC, Luna MA
South Med J 1979 Mar;72(3):313-8. doi: 10.1097/00007611-197903000-00023. PMID: 424824
Diffuse interstitial pulmonary fibrosis (cryptogenic fibrosing alveolitis).
Kunstling TR, Goodwin RA Jr, Des Prez RM
South Med J 1976 Apr;69(4):479-84, 487. doi: 10.1097/00007611-197604000-00029. PMID: 772819
Diffuse interstitial pulmonary fibrosis: course and prognosis.
Shah JR
Indian J Chest Dis 1972 Jul;14(3):142-50. PMID: 4639410

Prognosis

Methotrexate-induced pulmonary toxicity in psoriatic arthritis (PsA): case presentation and literature review.
Rondon F, Mendez O, Spinel N, Ochoa C, Saavedra C, Penaranda E, Garcia-Valladares I, Espinoza LR, Iglesias-Gamarra A
Clin Rheumatol 2011 Oct;30(10):1379-84. Epub 2011 May 26 doi: 10.1007/s10067-011-1765-7. PMID: 21614476
Tracheobronchomegaly associated with interstitial pulmonary fibrosis.
Vidal C, Peña F, Rodríguez Mosquera M, González Quintela A
Respiration 1991;58(3-4):207-10. doi: 10.1159/000195928. PMID: 1745857
Diffuse interstitial pulmonary fibrosis (cryptogenic fibrosing alveolitis).
Kunstling TR, Goodwin RA Jr, Des Prez RM
South Med J 1976 Apr;69(4):479-84, 487. doi: 10.1097/00007611-197604000-00029. PMID: 772819
Diffuse interstitial pulmonary fibrosis: course and prognosis.
Shah JR
Indian J Chest Dis 1972 Jul;14(3):142-50. PMID: 4639410
FAMILIAL INCIDENCE OF DIFFUSE INTERSTITIAL PULMONARY FIBROSIS.
ELLIS RH
Postgrad Med J 1965 Mar;41(473):150-2. doi: 10.1136/pgmj.41.473.150. PMID: 14266689Free PMC Article

Clinical prediction guides

Asbestos fiber content of lungs with diffuse interstitial fibrosis: An analytical scanning electron microscopic analysis of 249 cases.
Schneider F, Sporn TA, Roggli VL
Arch Pathol Lab Med 2010 Mar;134(3):457-61. doi: 10.5858/134.3.457. PMID: 20196673
The interaction of asbestos and neutrophils injures cultured human pulmonary epithelial cells: role of hydrogen peroxide.
Kamp DW, Dunne M, Weitzman SA, Dunn MM
J Lab Clin Med 1989 Nov;114(5):604-12. PMID: 2809401
Smoking and pulmonary fibrosis.
Weiss W
J Occup Med 1988 Jan;30(1):33-9. PMID: 3280757
Diffuse pulmonary interstitial fibrosis. Evidence of humoral antibody mediated pathogenesis.
Fan K, D'Orsogna DE
Chest 1984 Feb;85(2):150-5. doi: 10.1378/chest.85.2.150. PMID: 6362997
Methotrexate-induced diffuse interstitial pulmonary fibrosis.
Bedrossian CW, Miller WC, Luna MA
South Med J 1979 Mar;72(3):313-8. doi: 10.1097/00007611-197903000-00023. PMID: 424824

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