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Desquamative interstitial pneumonia(DIP)

MedGen UID:
65962
Concept ID:
C0238378
Disease or Syndrome
Synonyms: Desquamative interstitial pneumonitis; DIP; ILD, DESQUAMATIVE; INTERSTITIAL LUNG DISEASE, DESQUAMATIVE; INTERSTITIAL PNEUMONITIS, DESQUAMATIVE, FAMILIAL; PNEUMONIA, DESQUAMATIVE INTERSTITIAL, FAMILIAL
SNOMED CT: DIP - Desquamative interstitial pneumonitis (8549006); Desquamative interstitial pneumonia (8549006); Desquamative interstitial pneumonitis (8549006)
 
HPO: HP:0005942
Monarch Initiative: MONDO:0009887
OMIM®: 263000
Orphanet: ORPHA98852

Definition

Interstitial lung disease (ILD), or pneumonitis, is a heterogeneous group of disorders characterized pathologically by expansion of the interstitial compartment of the lung by inflammatory cells. Fibrosis occurs in many cases (Visscher and Myers, 2006). See also interstitial lung disease-1 (ILD1; 619611). Desquamative interstitial pneumonitis (DIP) was originally described as a pathologic entity by Liebow et al. (1965). Lung biopsy shows diffuse and uniform filling of alveoli by clusters of cells which Liebow et al. (1965) speculated to be 'desquamated pneumocytes.' Since then, these cells have been shown primarily to be pigmented alveolar macrophages. Other features include thickened alveolar septa with an infiltrate of inflammatory cells and plump, cuboidal type II pneumocytes. Mild collagen deposition without architectural distortion or honeycombing may be present. Different forms of ILD represent pathologic classifications based on histologic patterns rather than clinical diagnoses and may occur in a variety of clinical contexts (Visscher and Myers, 2006). Although DIP occurs most often as a sporadic disorder in adults during the third to fifth decade of life and is highly associated with smoking (Carrington et al., 1978), reports of a familial form with onset in infancy and early death suggest a genetic basis (Sharief et al., 1994). Cases of DIP reported in infants are often more severe and refractory to treatment than those reported in adults (Nogee et al., 2001). [from OMIM]

Clinical features

From HPO
Tubulointerstitial fibrosis
MedGen UID:
370652
Concept ID:
C1969372
Disease or Syndrome
A progressive detrimental connective tissue deposition (fibrosis) on the kidney parenchyma involving the tubules and interstitial tissue of the kidney. Tubulointerstitial injury in the kidney is complex, involving a number of independent and overlapping cellular and molecular pathways, with renal interstitial fibrosis and tubular atrophy (IF/TA) as the final common pathway. However, IF and TA are separable, as shown by the profound TA in renal artery stenosis, which characteristically has little or no fibrosis (or inflammation). For new annotations it is preferable to annotate to the specific HPO terms for Renal interstitial fibrosis and/or Renal tubular atrophy.
Cor pulmonale
MedGen UID:
18765
Concept ID:
C0034072
Disease or Syndrome
Right-sided heart failure resulting from chronic hypertension in the pulmonary arteries and right ventricle.
Failure to thrive
MedGen UID:
746019
Concept ID:
C2315100
Disease or Syndrome
Failure to thrive (FTT) refers to a child whose physical growth is substantially below the norm.
Cough
MedGen UID:
41325
Concept ID:
C0010200
Sign or Symptom
A sudden, audible expulsion of air from the lungs through a partially closed glottis, preceded by inhalation.
Tachypnea
MedGen UID:
66669
Concept ID:
C0231835
Finding
Very rapid breathing.
Desquamative interstitial pneumonia
MedGen UID:
65962
Concept ID:
C0238378
Disease or Syndrome
Interstitial lung disease (ILD), or pneumonitis, is a heterogeneous group of disorders characterized pathologically by expansion of the interstitial compartment of the lung by inflammatory cells. Fibrosis occurs in many cases (Visscher and Myers, 2006). See also interstitial lung disease-1 (ILD1; 619611). Desquamative interstitial pneumonitis (DIP) was originally described as a pathologic entity by Liebow et al. (1965). Lung biopsy shows diffuse and uniform filling of alveoli by clusters of cells which Liebow et al. (1965) speculated to be 'desquamated pneumocytes.' Since then, these cells have been shown primarily to be pigmented alveolar macrophages. Other features include thickened alveolar septa with an infiltrate of inflammatory cells and plump, cuboidal type II pneumocytes. Mild collagen deposition without architectural distortion or honeycombing may be present. Different forms of ILD represent pathologic classifications based on histologic patterns rather than clinical diagnoses and may occur in a variety of clinical contexts (Visscher and Myers, 2006). Although DIP occurs most often as a sporadic disorder in adults during the third to fifth decade of life and is highly associated with smoking (Carrington et al., 1978), reports of a familial form with onset in infancy and early death suggest a genetic basis (Sharief et al., 1994). Cases of DIP reported in infants are often more severe and refractory to treatment than those reported in adults (Nogee et al., 2001).
Respiratory distress
MedGen UID:
96907
Concept ID:
C0476273
Sign or Symptom
Respiratory distress is objectively observable as the physical or emotional consequences from the experience of dyspnea. The physical presentation of respiratory distress is generally referred to as labored breathing, while the sensation of respiratory distress is called shortness of breath or dyspnea.
Recurrent upper respiratory tract infections
MedGen UID:
154380
Concept ID:
C0581381
Disease or Syndrome
An increased susceptibility to upper respiratory tract infections as manifested by a history of recurrent upper respiratory tract infections (running ears - otitis, sinusitis, pharyngitis, tonsillitis).
Respiratory failure
MedGen UID:
257837
Concept ID:
C1145670
Disease or Syndrome
A severe form of respiratory insufficiency characterized by inadequate gas exchange such that the levels of oxygen or carbon dioxide cannot be maintained within normal limits.
Type II pneumocyte hypertrophy
MedGen UID:
1785168
Concept ID:
C5539455
Finding
Increase in size of type II pneumocytes, characterized by qualitative morphologic alterations, including cuboidal shapes, increased nucleocytoplasmic ratio, enlarged nuclei, prominent nucleoli, and various alterations in their nuclear chromatin.
Abnormality of metabolism/homeostasis
MedGen UID:
867398
Concept ID:
C4021768
Finding
Cyanosis
MedGen UID:
1189
Concept ID:
C0010520
Sign or Symptom
Bluish discoloration of the skin and mucosa due to poor circulation or inadequate oxygenation of arterial or capillary blood.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDesquamative interstitial pneumonia
Follow this link to review classifications for Desquamative interstitial pneumonia in Orphanet.

Conditions with this feature

Desquamative interstitial pneumonia
MedGen UID:
65962
Concept ID:
C0238378
Disease or Syndrome
Interstitial lung disease (ILD), or pneumonitis, is a heterogeneous group of disorders characterized pathologically by expansion of the interstitial compartment of the lung by inflammatory cells. Fibrosis occurs in many cases (Visscher and Myers, 2006). See also interstitial lung disease-1 (ILD1; 619611). Desquamative interstitial pneumonitis (DIP) was originally described as a pathologic entity by Liebow et al. (1965). Lung biopsy shows diffuse and uniform filling of alveoli by clusters of cells which Liebow et al. (1965) speculated to be 'desquamated pneumocytes.' Since then, these cells have been shown primarily to be pigmented alveolar macrophages. Other features include thickened alveolar septa with an infiltrate of inflammatory cells and plump, cuboidal type II pneumocytes. Mild collagen deposition without architectural distortion or honeycombing may be present. Different forms of ILD represent pathologic classifications based on histologic patterns rather than clinical diagnoses and may occur in a variety of clinical contexts (Visscher and Myers, 2006). Although DIP occurs most often as a sporadic disorder in adults during the third to fifth decade of life and is highly associated with smoking (Carrington et al., 1978), reports of a familial form with onset in infancy and early death suggest a genetic basis (Sharief et al., 1994). Cases of DIP reported in infants are often more severe and refractory to treatment than those reported in adults (Nogee et al., 2001).
Surfactant metabolism dysfunction, pulmonary, 1
MedGen UID:
368844
Concept ID:
C1968602
Disease or Syndrome
Inborn errors of pulmonary surfactant metabolism are genetically heterogeneous disorders resulting in severe respiratory insufficiency or failure in full-term neonates or infants. These disorders are associated with various pathologic entities, including pulmonary alveolar proteinosis (PAP), desquamative interstitial pneumonitis (DIP), or cellular nonspecific interstitial pneumonitis (NSIP) (Clark and Clark, 2005). A clinically similar disorder characterized by respiratory distress (267450) can affect preterm infants, who show developmental deficiency of surfactant. Acquired PAP (610910) is an autoimmune disorder characterized by the presence of autoantibodies to CSF2 (138960). Genetic Heterogeneity of Pulmonary Surfactant Metabolism Dysfunction See also SMDP2 (610913), caused by mutation in the SPTPC gene (178620) on 8p21; SMDP3 (610921), caused by mutation in the ABCA3 gene (601615) on 16p13; SMDP4 (300770), caused by mutation in the CSF2RA gene (306250) on Xp22; and SMDP5 (614370), caused by mutation in the CSF2RB gene (138981) on 22q12.
Interstitial lung disease due to ABCA3 deficiency
MedGen UID:
410074
Concept ID:
C1970456
Disease or Syndrome
For a general phenotypic description and a discussion of genetic heterogeneity of pulmonary surfactant metabolism dysfunction, see SMDP1 (265120).
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).
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.

Professional guidelines

PubMed

Kumar A, Cherian SV, Vassallo R, Yi ES, Ryu JH
Chest 2018 Aug;154(2):394-408. Epub 2017 Dec 5 doi: 10.1016/j.chest.2017.11.023. PMID: 29222007
Vassallo R, Thomas CF
Curr Opin Rheumatol 2004 May;16(3):186-91. doi: 10.1097/00002281-200405000-00004. PMID: 15103243
Jindal SK, Gupta D, Aggarwal AN
Curr Opin Pulm Med 1999 Sep;5(5):287-92. doi: 10.1097/00063198-199909000-00004. PMID: 10461532

Recent clinical studies

Etiology

Alarcon-Calderon A, Vassallo R, Yi ES, Ryu JH
Immunol Allergy Clin North Am 2023 May;43(2):273-287. Epub 2023 Mar 1 doi: 10.1016/j.iac.2023.01.007. PMID: 37055089
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Radiologia (Engl Ed) 2022 Dec;64 Suppl 3:277-289. doi: 10.1016/j.rxeng.2022.10.008. PMID: 36737166
Samarelli AV, Tonelli R, Marchioni A, Bruzzi G, Gozzi F, Andrisani D, Castaniere I, Manicardi L, Moretti A, Tabbì L, Cerri S, Beghè B, Dominici M, Clini E
Int J Mol Sci 2021 Aug 19;22(16) doi: 10.3390/ijms22168952. PMID: 34445658Free PMC Article
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Eur Respir Rev 2020 Jun 30;29(156) Epub 2020 Jun 23 doi: 10.1183/16000617.0183-2020. PMID: 32581141Free PMC Article
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Drug Saf 2010 Jul 1;33(7):539-58. doi: 10.2165/11532320-000000000-00000. PMID: 20553056

Diagnosis

Alarcon-Calderon A, Vassallo R, Yi ES, Ryu JH
Immunol Allergy Clin North Am 2023 May;43(2):273-287. Epub 2023 Mar 1 doi: 10.1016/j.iac.2023.01.007. PMID: 37055089
Serrano Gotarredona MP, Navarro Herrero S, Gómez Izquierdo L, Rodríguez Portal JA
Radiologia (Engl Ed) 2022 Dec;64 Suppl 3:277-289. doi: 10.1016/j.rxeng.2022.10.008. PMID: 36737166
Samarelli AV, Tonelli R, Marchioni A, Bruzzi G, Gozzi F, Andrisani D, Castaniere I, Manicardi L, Moretti A, Tabbì L, Cerri S, Beghè B, Dominici M, Clini E
Int J Mol Sci 2021 Aug 19;22(16) doi: 10.3390/ijms22168952. PMID: 34445658Free PMC Article
Lee KC, Kang EY, Yong HS, Kim C, Lee KY, Hwang SH, Oh YW
Korean J Radiol 2019 Sep;20(9):1368-1380. doi: 10.3348/kjr.2019.0057. PMID: 31464115Free PMC Article
Raoof S, Bondalapati P, Vydyula R, Ryu JH, Gupta N, Raoof S, Galvin J, Rosen MJ, Lynch D, Travis W, Mehta S, Lazzaro R, Naidich D
Chest 2016 Oct;150(4):945-965. Epub 2016 May 13 doi: 10.1016/j.chest.2016.04.026. PMID: 27180915Free PMC Article

Therapy

Alarcon-Calderon A, Vassallo R, Yi ES, Ryu JH
Immunol Allergy Clin North Am 2023 May;43(2):273-287. Epub 2023 Mar 1 doi: 10.1016/j.iac.2023.01.007. PMID: 37055089
Serrano Gotarredona MP, Navarro Herrero S, Gómez Izquierdo L, Rodríguez Portal JA
Radiologia (Engl Ed) 2022 Dec;64 Suppl 3:277-289. doi: 10.1016/j.rxeng.2022.10.008. PMID: 36737166
Margaritopoulos GA, Harari S, Caminati A, Antoniou KM
Respirology 2016 Jan;21(1):57-64. Epub 2015 Jul 2 doi: 10.1111/resp.12576. PMID: 26138798
Tazelaar HD, Wright JL, Churg A
Histopathology 2011 Mar;58(4):509-16. Epub 2010 Sep 21 doi: 10.1111/j.1365-2559.2010.03649.x. PMID: 20854463
Papiris SA, Triantafillidou C, Kolilekas L, Markoulaki D, Manali ED
Drug Saf 2010 Jul 1;33(7):539-58. doi: 10.2165/11532320-000000000-00000. PMID: 20553056

Prognosis

Alarcon-Calderon A, Vassallo R, Yi ES, Ryu JH
Immunol Allergy Clin North Am 2023 May;43(2):273-287. Epub 2023 Mar 1 doi: 10.1016/j.iac.2023.01.007. PMID: 37055089
Cottin V
Eur Respir Rev 2020 Jun 30;29(156) Epub 2020 Jun 23 doi: 10.1183/16000617.0183-2020. PMID: 32581141Free PMC Article
Hellemons ME, Moor CC, von der Thüsen J, Rossius M, Odink A, Thorgersen LH, Verschakelen J, Wuyts W, Wijsenbeek MS, Bendstrup E
Eur Respir Rev 2020 Jun 30;29(156) Epub 2020 Jun 23 doi: 10.1183/16000617.0181-2019. PMID: 32581140Free PMC Article
Margaritopoulos GA, Vasarmidi E, Jacob J, Wells AU, Antoniou KM
Eur Respir Rev 2015 Sep;24(137):428-35. doi: 10.1183/16000617.0050-2015. PMID: 26324804Free PMC Article
Tazelaar HD, Wright JL, Churg A
Histopathology 2011 Mar;58(4):509-16. Epub 2010 Sep 21 doi: 10.1111/j.1365-2559.2010.03649.x. PMID: 20854463

Clinical prediction guides

Li Y, Seidl E, Knoflach K, Gothe F, Forstner ME, Michel K, Pawlita I, Gesenhues F, Sattler F, Yang X, Kroener C, Reu-Hofer S, Ley-Zaporozhan J, Kammer B, Krüger-Stollfuß I, Dinkel J, Carlens J, Wetzke M, Moreno-Galdó A, Torrent-Vernetta A, Lange J, Krenke K, Rumman N, Mayell S, Sismanlar T, Aslan A, Regamey N, Proesmans M, Stehling F, Naehrlich L, Ayse K, Becker S, Koerner-Rettberg C, Plattner E, Manali ED, Papiris SA, Campo I, Kappler M, Schwerk N, Griese M
Thorax 2023 Jun;78(6):587-595. Epub 2023 Feb 20 doi: 10.1136/thorax-2022-219434. PMID: 36808083Free PMC Article
Cottin V
Eur Respir Rev 2020 Jun 30;29(156) Epub 2020 Jun 23 doi: 10.1183/16000617.0183-2020. PMID: 32581141Free PMC Article
Bak SH, Lee HY
Int J Chron Obstruct Pulmon Dis 2017;12:3221-3229. Epub 2017 Nov 1 doi: 10.2147/COPD.S146899. PMID: 29138550Free PMC Article
Godbert B, Wissler MP, Vignaud JM
Eur Respir Rev 2013 Jun 1;22(128):117-23. doi: 10.1183/09059180.00005812. PMID: 23728865Free PMC Article
Papiris SA, Triantafillidou C, Kolilekas L, Markoulaki D, Manali ED
Drug Saf 2010 Jul 1;33(7):539-58. doi: 10.2165/11532320-000000000-00000. PMID: 20553056

Recent systematic reviews

Hochhegger B, Zanon M, Altmayer S, Mandelli NS, Stüker G, Mohammed TL, Verma N, Meirelles GSP, Marchiori E
Br J Radiol 2021 Feb 1;94(1118):20200703. Epub 2020 Dec 9 doi: 10.1259/bjr.20200703. PMID: 33296607Free PMC Article
Hellemons ME, Moor CC, von der Thüsen J, Rossius M, Odink A, Thorgersen LH, Verschakelen J, Wuyts W, Wijsenbeek MS, Bendstrup E
Eur Respir Rev 2020 Jun 30;29(156) Epub 2020 Jun 23 doi: 10.1183/16000617.0181-2019. PMID: 32581140Free PMC Article
Polosa R, Simidchiev A, Walters EH
Cochrane Database Syst Rev 2002;2002(3):CD002872. doi: 10.1002/14651858.CD002872. PMID: 12137662Free PMC Article

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