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Interstitial pneumonitis

MedGen UID:
61507
Concept ID:
C0206061
Disease or Syndrome
Synonyms: Interstitial Pneumonia; Interstitial Pneumonias; Interstitial Pneumonitides; Interstitial Pneumonitis; Pneumonia, Interstitial; Pneumonias, Interstitial; Pneumonitides, Interstitial; Pneumonitis, Interstitial
SNOMED CT: Interstitial pneumonia (64667001); Interstitial pneumonitis (64667001)
 
HPO: HP:0006515

Definition

Inflammation of interstitial lung tissue, usually associated with infection. [from NCI]

Conditions with this feature

Surfactant metabolism dysfunction, pulmonary, 2
MedGen UID:
410078
Concept ID:
C1970470
Disease or Syndrome
Pulmonary surfactant metabolism dysfunction-2 (SMDP2) is a rare autosomal dominant disease associated with progressive respiratory insufficiency and lung disease with a variable clinical course. The pathophysiology of the disorder is postulated to involve intracellular accumulation of a structurally defective SPC protein (Thomas et al., 2002). For a general phenotypic description and a discussion of genetic heterogeneity of pulmonary surfactant metabolism dysfunction, see SMDP1 (265120).
Autoinflammation-PLCG2-associated antibody deficiency-immune dysregulation
MedGen UID:
766875
Concept ID:
C3553961
Disease or Syndrome
Autoinflammation, antibody deficiency, and immune dysregulation (APLAID) is an autosomal dominant systemic disorder characterized by recurrent blistering skin lesions with a dense inflammatory infiltrate and variable involvement of other tissues, including joints, the eye, and the gastrointestinal tract. Affected individuals have a mild humoral immune deficiency associated with recurrent sinopulmonary infections, but no evidence of circulating autoantibodies (summary by Zhou et al., 2012).
STAT3-related early-onset multisystem autoimmune disease
MedGen UID:
863232
Concept ID:
C4014795
Disease or Syndrome
Infantile-onset multisystem autoimmune disease-1 is characterized by early childhood onset of a spectrum of autoimmune disorders affecting multiple organs. Common manifestations include insulin-dependent diabetes mellitus and autoimmune enteropathy, or celiac disease, and autoimmune hematologic disorders. Other features include short stature and nonspecific dermatitis. More variable features include hypothyroidism, autoimmune arthritis, and delayed puberty. Some patients may show recurrent infections. The disorder results from an inborn error of cytokine signaling (summary by Flanagan et al., 2014 and Milner et al., 2015). Genetic Heterogeneity of Infantile-Onset Multisystem Autoimmune Disease See also ADMIO2 (617006), caused by mutation in the ZAP70 gene (176947) on chromosome 2q12, and ADMIO3 (620430), caused by mutation in the CBLB gene (604491) on chromosome 3q13.
DOCK2 deficiency
MedGen UID:
901370
Concept ID:
C4225328
Disease or Syndrome
Immunodeficiency-40 is an autosomal recessive primary form of combined immunodeficiency mainly affecting T-cell number and function, with other more variable defects in B-cell and NK-cell function. Patients have onset of severe invasive bacterial and viral infections in early childhood and may die without bone marrow transplantation (summary by Dobbs et al., 2015).
Dyskeratosis congenita, autosomal dominant 1
MedGen UID:
1645250
Concept ID:
C4551974
Disease or Syndrome
Dyskeratosis congenita and related telomere biology disorders (DC/TBD) are caused by impaired telomere maintenance resulting in short or very short telomeres. The phenotypic spectrum of telomere biology disorders is broad and includes individuals with classic dyskeratosis congenita (DC) as well as those with very short telomeres and an isolated physical finding. Classic DC is characterized by a triad of dysplastic nails, lacy reticular pigmentation of the upper chest and/or neck, and oral leukoplakia, although this may not be present in all individuals. People with DC/TBD are at increased risk for progressive bone marrow failure (BMF), myelodysplastic syndrome or acute myelogenous leukemia, solid tumors (usually squamous cell carcinoma of the head/neck or anogenital cancer), and pulmonary fibrosis. Other findings can include eye abnormalities (epiphora, blepharitis, sparse eyelashes, ectropion, entropion, trichiasis), taurodontism, liver disease, gastrointestinal telangiectasias, and avascular necrosis of the hips or shoulders. Although most persons with DC/TBD have normal psychomotor development and normal neurologic function, significant developmental delay is present in both forms; additional findings include cerebellar hypoplasia (Hoyeraal Hreidarsson syndrome) and bilateral exudative retinopathy and intracranial calcifications (Revesz syndrome and Coats plus syndrome). Onset and progression of manifestations of DC/TBD vary: at the mild end of the spectrum are those who have only minimal physical findings with normal bone marrow function, and at the severe end are those who have the diagnostic triad and early-onset BMF.
Autoinflammation with pulmonary and cutaneous vasculitis
MedGen UID:
1841007
Concept ID:
C5830371
Disease or Syndrome
Autoinflammation with pulmonary and cutaneous vasculitis (AIPCV) is a disorder of immune dysregulation manifest as skin lesions (petechiae and purpura) appearing soon after birth followed by progressive pulmonary involvement causing restrictive lung disease and respiratory insufficiency. Other features may include hepatosplenomegaly and anemia (Kanderova et al., 2022).

Professional guidelines

PubMed

Guzik TJ, Mohiddin SA, Dimarco A, Patel V, Savvatis K, Marelli-Berg FM, Madhur MS, Tomaszewski M, Maffia P, D'Acquisto F, Nicklin SA, Marian AJ, Nosalski R, Murray EC, Guzik B, Berry C, Touyz RM, Kreutz R, Wang DW, Bhella D, Sagliocco O, Crea F, Thomson EC, McInnes IB
Cardiovasc Res 2020 Aug 1;116(10):1666-1687. doi: 10.1093/cvr/cvaa106. PMID: 32352535Free PMC Article
Wang H, Guo X, Zhou J, Li Y, Duan L, Si X, Zhang L, Liu X, Wang M, Shi J, Zhang L
Thorac Cancer 2020 Jan;11(1):191-197. Epub 2019 Nov 24 doi: 10.1111/1759-7714.13240. PMID: 31762218Free PMC Article
Fajgenbaum DC, Uldrick TS, Bagg A, Frank D, Wu D, Srkalovic G, Simpson D, Liu AY, Menke D, Chandrakasan S, Lechowicz MJ, Wong RS, Pierson S, Paessler M, Rossi JF, Ide M, Ruth J, Croglio M, Suarez A, Krymskaya V, Chadburn A, Colleoni G, Nasta S, Jayanthan R, Nabel CS, Casper C, Dispenzieri A, Fosså A, Kelleher D, Kurzrock R, Voorhees P, Dogan A, Yoshizaki K, van Rhee F, Oksenhendler E, Jaffe ES, Elenitoba-Johnson KS, Lim MS
Blood 2017 Mar 23;129(12):1646-1657. Epub 2017 Jan 13 doi: 10.1182/blood-2016-10-746933. PMID: 28087540Free PMC Article

Recent clinical studies

Etiology

Shakil F, Snijder J, Salvatore MM
Expert Rev Respir Med 2022 Aug;16(8):907-915. Epub 2022 Sep 6 doi: 10.1080/17476348.2022.2119131. PMID: 36066423
Musk AW, de Klerk N, Reid A, Hui J, Franklin P, Brims F
Int J Tuberc Lung Dis 2020 Jun 1;24(6):562-567. doi: 10.5588/ijtld.19.0645. PMID: 32553000
Dawod YT, Cook NE, Graham WB, Madhani-Lovely F, Thao C
Expert Rev Respir Med 2020 Aug;14(8):825-834. Epub 2020 May 22 doi: 10.1080/17476348.2020.1766971. PMID: 32379511
Oshima Y, Tanimoto T, Yuji K, Tojo A
JAMA Oncol 2018 Aug 1;4(8):1112-1115. doi: 10.1001/jamaoncol.2017.4526. PMID: 29327061Free PMC Article
Flament T, Bigot A, Chaigne B, Henique H, Diot E, Marchand-Adam S
Eur Respir Rev 2016 Jun;25(140):110-23. doi: 10.1183/16000617.0011-2016. PMID: 27246587Free PMC Article

Diagnosis

Okor I, Bob-Manuel T, Price J, Sleem A, Amoran O, Kelly J, Ekerete MF, Bamgbose MO, Bolaji OA, Krim SR
Curr Probl Cardiol 2022 Sep;47(9):101268. Epub 2022 May 27 doi: 10.1016/j.cpcardiol.2022.101268. PMID: 35644500Free PMC Article
Jain P, Garg S, Sharma VK, Maheshwari S
J Assoc Physicians India 2019 Apr;67(4):87-88. PMID: 31299851
Morales AT, Cignarella AG, Jabeen IS, Barkin JS, Mirsaeidi M
Eur J Intern Med 2019 Aug;66:18-24. Epub 2019 Jun 19 doi: 10.1016/j.ejim.2019.06.010. PMID: 31227290
Salvatore M, Ishikawa G, Padilla M
J Am Board Fam Med 2018 Jan-Feb;31(1):151-162. doi: 10.3122/jabfm.2018.01.170288. PMID: 29330249
Javidan-Nejad C, Bhalla S
Radiol Clin North Am 2009 Mar;47(2):289-306. doi: 10.1016/j.rcl.2008.11.006. PMID: 19249457

Therapy

Morimoto Y, Nishida C, Tomonaga T, Izumi H, Yatera K, Sakurai K, Kim Y
J Occup Health 2021 Jan;63(1):e12240. doi: 10.1002/1348-9585.12240. PMID: 34128301Free PMC Article
Shafi A, Rehman JU, Nawaz A, Aljohani NI
J Coll Physicians Surg Pak 2020 Oct;30(10):1117-1118. doi: 10.29271/jcpsp.2020.10.1117. PMID: 33143845
Verriere B, Ferreira V, Denis E, Zahreddine K, Deletie E, Quinsat D, Re D
Am J Ther 2016 Sep-Oct;23(5):e1205-8. doi: 10.1097/MJT.0000000000000300. PMID: 26371947
Vourlekis JS
Clin Chest Med 2004 Dec;25(4):739-47, vii. doi: 10.1016/j.ccm.2004.07.001. PMID: 15564019
Rosenow EC 3rd
Dis Mon 1994 May;40(5):253-310. doi: 10.1016/0011-5029(94)90025-6. PMID: 8174459

Prognosis

Morales AT, Cignarella AG, Jabeen IS, Barkin JS, Mirsaeidi M
Eur J Intern Med 2019 Aug;66:18-24. Epub 2019 Jun 19 doi: 10.1016/j.ejim.2019.06.010. PMID: 31227290
Salvatore M, Ishikawa G, Padilla M
J Am Board Fam Med 2018 Jan-Feb;31(1):151-162. doi: 10.3122/jabfm.2018.01.170288. PMID: 29330249
Oshima Y, Tanimoto T, Yuji K, Tojo A
JAMA Oncol 2018 Aug 1;4(8):1112-1115. doi: 10.1001/jamaoncol.2017.4526. PMID: 29327061Free PMC Article
Flament T, Bigot A, Chaigne B, Henique H, Diot E, Marchand-Adam S
Eur Respir Rev 2016 Jun;25(140):110-23. doi: 10.1183/16000617.0011-2016. PMID: 27246587Free PMC Article
Klotman ME, Hamilton JD
Semin Respir Infect 1987 Jun;2(2):95-103. PMID: 2827281

Clinical prediction guides

Camard M, Besse B, Cariou PL, Massayke S, Laparra A, Noel N, Michot JM, Ammari S, Pavec JL, Lambotte O
Respir Med Res 2022 Nov;82:100969. Epub 2022 Nov 9 doi: 10.1016/j.resmer.2022.100969. PMID: 36370683
Guzik TJ, Mohiddin SA, Dimarco A, Patel V, Savvatis K, Marelli-Berg FM, Madhur MS, Tomaszewski M, Maffia P, D'Acquisto F, Nicklin SA, Marian AJ, Nosalski R, Murray EC, Guzik B, Berry C, Touyz RM, Kreutz R, Wang DW, Bhella D, Sagliocco O, Crea F, Thomson EC, McInnes IB
Cardiovasc Res 2020 Aug 1;116(10):1666-1687. doi: 10.1093/cvr/cvaa106. PMID: 32352535Free PMC Article
Jain P, Garg S, Sharma VK, Maheshwari S
J Assoc Physicians India 2019 Apr;67(4):87-88. PMID: 31299851
Flament T, Bigot A, Chaigne B, Henique H, Diot E, Marchand-Adam S
Eur Respir Rev 2016 Jun;25(140):110-23. doi: 10.1183/16000617.0011-2016. PMID: 27246587Free PMC Article
Klotman ME, Hamilton JD
Semin Respir Infect 1987 Jun;2(2):95-103. PMID: 2827281

Recent systematic reviews

Genestreti G, Di Battista M, Trisolini R, Denicolò F, Valli M, Lazzari-Agli LA, Dalpiaz G, De Biase D, Bartolotti M, Cavallo G, Brandes AA
Tumori 2015 Jun 25;101(3):e92-5. doi: 10.5301/tj.5000275. PMID: 25908033
Yalçin B, Kremer LC, Caron HN, van Dalen EC
Cochrane Database Syst Rev 2013 Aug 22;(8):CD006301. doi: 10.1002/14651858.CD006301.pub3. PMID: 23970444
Yalçin B, Kremer LC, Caron HN, van Dalen EC
Cochrane Database Syst Rev 2010 May 12;(5):CD006301. doi: 10.1002/14651858.CD006301.pub2. PMID: 20464740
Raanani P, Gafter-Gvili A, Paul M, Ben-Bassat I, Leibovici L, Shpilberg O
J Clin Oncol 2009 Feb 10;27(5):770-81. Epub 2008 Dec 29 doi: 10.1200/JCO.2008.16.8450. PMID: 19114702
Raanani P, Gafter-Gvili A, Paul M, Ben-Bassat I, Leibovici L, Shpilberg O
Cochrane Database Syst Rev 2008 Oct 8;(4):CD006501. doi: 10.1002/14651858.CD006501.pub2. PMID: 18843719

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