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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

Mahbubani V, Trikannad VS, Sainani GS
J Assoc Physicians India 1995 Jan;43(1):28-9. PMID: 9282635
Yang SC, Wu HD, Yang SP
J Formos Med Assoc 1991 Mar;90(3):244-9. PMID: 1677399
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
Gruber UF
Anticancer Drugs 1990 Dec;1(2):187-97. doi: 10.1097/00001813-199012000-00012. PMID: 2151850
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

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
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
Bedrossian CW, Miller WC, Luna MA
South Med J 1979 Mar;72(3):313-8. doi: 10.1097/00007611-197903000-00023. PMID: 424824
Krákora P, Rehák F
Bronchopneumologie 1976 Sep-Oct;26(5):438-41. PMID: 1009485
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

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
Weiss W
J Occup Med 1988 Jan;30(1):33-9. PMID: 3280757
Bedrossian CW, Miller WC, Luna MA
South Med J 1979 Mar;72(3):313-8. doi: 10.1097/00007611-197903000-00023. PMID: 424824
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
Walker WC, Wright V
Ann Rheum Dis 1969 May;28(3):252-9. doi: 10.1136/ard.28.3.252. PMID: 5772520Free PMC Article

Prognosis

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
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
Shah JR
Indian J Chest Dis 1972 Jul;14(3):142-50. PMID: 4639410
Steel SJ, Winstanley DP
Thorax 1969 Sep;24(5):576-84. doi: 10.1136/thx.24.5.576. PMID: 5348325Free PMC Article
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

Schneider F, Sporn TA, Roggli VL
Arch Pathol Lab Med 2010 Mar;134(3):457-61. doi: 10.5858/134.3.457. PMID: 20196673
Kamp DW, Dunne M, Weitzman SA, Dunn MM
J Lab Clin Med 1989 Nov;114(5):604-12. PMID: 2809401
Weiss W
J Occup Med 1988 Jan;30(1):33-9. PMID: 3280757
Fan K, D'Orsogna DE
Chest 1984 Feb;85(2):150-5. doi: 10.1378/chest.85.2.150. PMID: 6362997
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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