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Lymphocytic interstitial pneumonia(LIP)

MedGen UID:
82682
Concept ID:
C0264511
Disease or Syndrome
Synonym: Lymphoid interstitial pneumonia
SNOMED CT: Idiopathic lymphocytic interstitial pneumonia (44274007); Lymphoid interstitial pneumonitis (44274007); LIP - Lymphoid interstitial pneumonitis (44274007); Lymphoid interstitial pneumonia (44274007); Lymphocytic interstitial pneumonia (44274007)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
HPO: HP:0006527
Monarch Initiative: MONDO:0009537
OMIM®: 247610
Orphanet: ORPHA79128

Definition

Lymphocytic interstitial pneumonitis is a benign lymphoproliferative disorder of the lung that is characterized by the presence of a dense, predominantly lymphocytic interstitial infiltrate (lymphocytes, plasma cells, other elements of the lymphoreticular system) that expands the alveolar septa. [from HPO]

Clinical features

From HPO
Clubbing
MedGen UID:
57692
Concept ID:
C0149651
Anatomical Abnormality
Broadening of the soft tissues (non-edematous swelling of soft tissues) of the digital tips in all dimensions associated with an increased longitudinal and lateral curvature of the nails.
Aortic valve stenosis
MedGen UID:
1621
Concept ID:
C0003507
Pathologic Function
The presence of a stenosis (narrowing) of the aortic valve.
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.
Lymphocytic interstitial pneumonia
MedGen UID:
82682
Concept ID:
C0264511
Disease or Syndrome
Lymphocytic interstitial pneumonitis is a benign lymphoproliferative disorder of the lung that is characterized by the presence of a dense, predominantly lymphocytic interstitial infiltrate (lymphocytes, plasma cells, other elements of the lymphoreticular system) that expands the alveolar septa.

Term Hierarchy

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

Conditions with this feature

Lymphocytic interstitial pneumonia
MedGen UID:
82682
Concept ID:
C0264511
Disease or Syndrome
Lymphocytic interstitial pneumonitis is a benign lymphoproliferative disorder of the lung that is characterized by the presence of a dense, predominantly lymphocytic interstitial infiltrate (lymphocytes, plasma cells, other elements of the lymphoreticular system) that expands the alveolar septa.
Immunodeficiency 63 with lymphoproliferation and autoimmunity
MedGen UID:
1682943
Concept ID:
C5193126
Disease or Syndrome
Immunodeficiency-63 with lymphoproliferation and autoimmunity (IMD63) is an autosomal recessive disorder characterized by immune dysregulation. Affected individuals present in infancy with features of both abnormal activation of certain immune signaling pathways, resulting in lymphoid proliferation, dermatitis, enteropathy, and hypergammaglobulinemia, as well as features of immunodeficiency, such as recurrent infections and increased susceptibility to viral infections, especially CMV. Laboratory studies show increased NK cells that show impaired differentiation, as well as abnormal T cell populations or responses. Some patients may die in childhood; hematopoietic bone marrow transplantation is curative (summary by Zhang et al., 2019).
Growth hormone insensitivity with immune dysregulation 1, autosomal recessive
MedGen UID:
1734133
Concept ID:
C5435698
Disease or Syndrome
Autosomal recessive growth hormone insensitivity syndrome with immune dysregulation-1 (GHISID1) is a congenital disorder characterized by short stature due to insensitivity to growth hormone (GH1; 139250). Affected individuals usually have failure to thrive, delayed bone age, and delayed puberty associated with decreased serum IGF1 (147440), IGFBP3 (146732), and ALS (601489). Some patients may have dysmorphic features. Most, but not all, patients have features of immune dysregulation, including chronic pulmonary disease, interstitial pneumonitis, recurrent or severe infections, eczema, and autoimmune arthritis. The immune features are highly variable (summary by Kofoed et al., 2003; Vidarsdottir et al., 2006). See 262500 for a form of growth hormone insensitivity caused by mutation in the growth hormone receptor gene (GHR; 600946).

Professional guidelines

PubMed

Simchoni N, Vogel TP, Shum AK
Rheum Dis Clin North Am 2023 Nov;49(4):789-804. Epub 2023 Jul 21 doi: 10.1016/j.rdc.2023.06.005. PMID: 37821196Free PMC Article
Baldi BG, Carvalho CRR, Dias OM, Marchiori E, Hochhegger B
J Bras Pneumol 2017 Mar-Apr;43(2):140-149. doi: 10.1590/S1806-37562016000000341. PMID: 28538782Free PMC Article
Park S, Lee EJ
Korean J Intern Med 2017 Mar;32(2):229-238. Epub 2017 Feb 28 doi: 10.3904/kjim.2016.242. PMID: 28264540Free PMC Article

Recent clinical studies

Etiology

Cabeza Martínez B, Giménez Palleiro A, Mazzini Florindez SP
Radiologia (Engl Ed) 2022 Dec;64 Suppl 3:265-276. doi: 10.1016/j.rxeng.2022.09.005. PMID: 36737165
Lin W, Xin Z, Zhang J, Liu N, Ren X, Liu M, Su Y, Liu Y, Yang L, Guo S, Yang Y, Li Y, Cao J, Ning X, Li J, Xue H, Niu N, Chen Y, Li F, Sun L, Zhang X, Zhang F, Zhang W
BMC Pulm Med 2022 Feb 27;22(1):73. doi: 10.1186/s12890-022-01868-5. PMID: 35220958Free PMC Article
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 CT, Strek ME
Curr Opin Pulm Med 2021 Sep 1;27(5):388-395. doi: 10.1097/MCP.0000000000000791. PMID: 34127620Free PMC Article
Elia D, Torre O, Cassandro R, Caminati A, Harari S
Eur Respir Rev 2020 Sep 30;29(157) Epub 2020 Sep 2 doi: 10.1183/16000617.0163-2019. PMID: 32878971Free PMC Article

Diagnosis

Cabeza Martínez B, Giménez Palleiro A, Mazzini Florindez SP
Radiologia (Engl Ed) 2022 Dec;64 Suppl 3:265-276. doi: 10.1016/j.rxeng.2022.09.005. PMID: 36737165
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
Luppi F, Sebastiani M, Silva M, Sverzellati N, Cavazza A, Salvarani C, Manfredi A
Clin Exp Rheumatol 2020 Jul-Aug;38 Suppl 126(4):291-300. Epub 2020 Oct 23 PMID: 33095142
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
Panchabhai TS, Farver C, Highland KB
Clin Chest Med 2016 Sep;37(3):463-74. Epub 2016 Jun 25 doi: 10.1016/j.ccm.2016.04.009. PMID: 27514593

Therapy

Figuerêdo Moreira I, Marcelino Alvares-Saraiva A, Cristina Pérez E, Guilherme Xavier J, Denelle Spadacci-Morena D, Silva de Araújo R, Ricardo Dell'Armelina Rocha P, Anete Lallo M
Immunobiology 2022 May;227(3):152194. Epub 2022 Mar 6 doi: 10.1016/j.imbio.2022.152194. PMID: 35278762
Luppi F, Sebastiani M, Silva M, Sverzellati N, Cavazza A, Salvarani C, Manfredi A
Clin Exp Rheumatol 2020 Jul-Aug;38 Suppl 126(4):291-300. Epub 2020 Oct 23 PMID: 33095142
Panchabhai TS, Farver C, Highland KB
Clin Chest Med 2016 Sep;37(3):463-74. Epub 2016 Jun 25 doi: 10.1016/j.ccm.2016.04.009. PMID: 27514593
Ghrenassia E, Martis N, Boyer J, Burel-Vandenbos F, Mekinian A, Coppo P
J Autoimmun 2015 May;59:19-25. Epub 2015 Feb 7 doi: 10.1016/j.jaut.2015.01.010. PMID: 25660200
Della Latta V, Cabiati M, Rocchiccioli S, Del Ry S, Morales MA
Pharmacol Res 2013 Oct;76:182-9. Epub 2013 Aug 28 doi: 10.1016/j.phrs.2013.08.004. PMID: 23994158

Prognosis

Lin W, Xin Z, Zhang J, Liu N, Ren X, Liu M, Su Y, Liu Y, Yang L, Guo S, Yang Y, Li Y, Cao J, Ning X, Li J, Xue H, Niu N, Chen Y, Li F, Sun L, Zhang X, Zhang F, Zhang W
BMC Pulm Med 2022 Feb 27;22(1):73. doi: 10.1186/s12890-022-01868-5. PMID: 35220958Free PMC Article
Lee CT, Strek ME
Curr Opin Pulm Med 2021 Sep 1;27(5):388-395. doi: 10.1097/MCP.0000000000000791. PMID: 34127620Free PMC Article
Dong X, Gao YL, Lu Y, Zheng Y
Clin Rheumatol 2021 Feb;40(2):601-612. Epub 2020 Jul 1 doi: 10.1007/s10067-020-05236-8. PMID: 32613392Free PMC Article
Luppi F, Sebastiani M, Silva M, Sverzellati N, Cavazza A, Salvarani C, Manfredi A
Clin Exp Rheumatol 2020 Jul-Aug;38 Suppl 126(4):291-300. Epub 2020 Oct 23 PMID: 33095142
Prenzel F, Harfst J, Schwerk N, Ahrens F, Rietschel E, Schmitt-Grohé S, Rubak SML, Poplawska K, Baden W, Vogel M, Hollizeck S, Ley-Zaporozhan J, Brasch F, Reu S, Griese M; LIP/FB-Kids-Lung-Registry Study Group
Pediatr Pulmonol 2020 Apr;55(4):909-917. Epub 2020 Feb 10 doi: 10.1002/ppul.24680. PMID: 32040879

Clinical prediction guides

Gonçalves CM, Cordeiro LR, de Andrade CAF, de Carvalho JF
Curr Rheumatol Rev 2023;19(1):1-6. doi: 10.2174/1573397118666220817091520. PMID: 35980075
Figuerêdo Moreira I, Marcelino Alvares-Saraiva A, Cristina Pérez E, Guilherme Xavier J, Denelle Spadacci-Morena D, Silva de Araújo R, Ricardo Dell'Armelina Rocha P, Anete Lallo M
Immunobiology 2022 May;227(3):152194. Epub 2022 Mar 6 doi: 10.1016/j.imbio.2022.152194. PMID: 35278762
Lin W, Xin Z, Zhang J, Liu N, Ren X, Liu M, Su Y, Liu Y, Yang L, Guo S, Yang Y, Li Y, Cao J, Ning X, Li J, Xue H, Niu N, Chen Y, Li F, Sun L, Zhang X, Zhang F, Zhang W
BMC Pulm Med 2022 Feb 27;22(1):73. doi: 10.1186/s12890-022-01868-5. PMID: 35220958Free PMC Article
Luppi F, Sebastiani M, Silva M, Sverzellati N, Cavazza A, Salvarani C, Manfredi A
Clin Exp Rheumatol 2020 Jul-Aug;38 Suppl 126(4):291-300. Epub 2020 Oct 23 PMID: 33095142
Prenzel F, Harfst J, Schwerk N, Ahrens F, Rietschel E, Schmitt-Grohé S, Rubak SML, Poplawska K, Baden W, Vogel M, Hollizeck S, Ley-Zaporozhan J, Brasch F, Reu S, Griese M; LIP/FB-Kids-Lung-Registry Study Group
Pediatr Pulmonol 2020 Apr;55(4):909-917. Epub 2020 Feb 10 doi: 10.1002/ppul.24680. PMID: 32040879

Recent systematic reviews

Ramzi N, Jamee M, Bakhtiyari M, Rafiemanesh H, Zainaldain H, Tavakol M, Rezaei A, Kalvandi M, Zian Z, Mohammadi H, Jadidi-Niaragh F, Yazdani R, Abolhassani H, Aghamohammadi A, Azizi G
Pediatr Pulmonol 2020 Feb;55(2):292-299. Epub 2019 Dec 13 doi: 10.1002/ppul.24599. PMID: 31833673

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