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Pulmonary interstitial lymphocyte infiltration

MedGen UID:
1785260
•Concept ID:
C5539701
•
Finding
HPO: HP:0033582

Definition

Abnormal accumulation of lymphocytes in the interstitium of the lung. [from HPO]

Conditions with this feature

Immunodeficiency due to CD25 deficiency
MedGen UID:
377894
•Concept ID:
C1853392
•
Disease or Syndrome
Immunodeficiency-41 is an autosomal recessive complex disorder of immune dysregulation. Affected individuals present in infancy with recurrent viral, fungal, and bacterial infections, lymphadenopathy, and variable autoimmune features, such as autoimmune enteropathy and eczematous skin lesions. Immunologic studies show a defect in T-cell regulation (summary by Goudy et al., 2013).
See: Condition Record
Immunodeficiency due to CD25 deficiency

Professional guidelines

PubMed

Differential diagnosis of IgG4-positive plasma cells in the lung.
Mukhopadhyay S
Semin Diagn Pathol 2024 Mar;41(2):72-78. Epub 2023 Nov 15 doi: 10.1053/j.semdp.2023.11.006. PMID: 37993385
Pulmonary Manifestations of Primary Sjögren's Syndrome: Underlying Immunological Mechanisms, Clinical Presentation, and Management.
Gupta S, Ferrada MA, Hasni SA
Front Immunol 2019;10:1327. Epub 2019 Jun 12 doi: 10.3389/fimmu.2019.01327. PMID: 31249572Free PMC Article
British Lung Foundation/United Kingdom Primary Immunodeficiency Network Consensus Statement on the Definition, Diagnosis, and Management of Granulomatous-Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency Disorders.
Hurst JR, Verma N, Lowe D, Baxendale HE, Jolles S, Kelleher P, Longhurst HJ, Patel SY, Renzoni EA, Sander CR, Avery GR, Babar JL, Buckland MS, Burns S, Egner W, Gompels MM, Gordins P, Haddock JA, Hart SP, Hayman GR, Herriot R, Hoyles RK, Huissoon AP, Jacob J, Nicholson AG, Rassl DM, Sargur RB, Savic S, Seneviratne SL, Sheaff M, Vaitla PM, Walters GI, Whitehouse JL, Wright PA, Condliffe AM
J Allergy Clin Immunol Pract 2017 Jul-Aug;5(4):938-945. Epub 2017 Mar 25 doi: 10.1016/j.jaip.2017.01.021. PMID: 28351785

Recent clinical studies

Etiology

Single-cell profiling reveals distinct adaptive immune hallmarks in MDA5+ dermatomyositis with therapeutic implications.
Ye Y, Chen Z, Jiang S, Jia F, Li T, Lu X, Xue J, Lian X, Ma J, Hao P, Lu L, Ye S, Shen N, Bao C, Fu Q, Zhang X
Nat Commun 2022 Oct 29;13(1):6458. doi: 10.1038/s41467-022-34145-4. PMID: 36309526Free PMC Article
Therapeutic options for CTLA-4 insufficiency.
Egg D, Rump IC, Mitsuiki N, Rojas-Restrepo J, Maccari ME, Schwab C, Gabrysch A, Warnatz K, Goldacker S, Patiño V, Wolff D, Okada S, Hayakawa S, Shikama Y, Kanda K, Imai K, Sotomatsu M, Kuwashima M, Kamiya T, Morio T, Matsumoto K, Mori T, Yoshimoto Y, Dybedal I, Kanariou M, Kucuk ZY, Chapdelaine H, Petruzelkova L, Lorenz HM, Sullivan KE, Heimall J, Moutschen M, Litzman J, Recher M, Albert MH, Hauck F, Seneviratne S, Pachlopnik Schmid J, Kolios A, Unglik G, Klemann C, Snapper S, Giulino-Roth L, Svaton M, Platt CD, Hambleton S, Neth O, Gosse G, Reinsch S, Holzinger D, Kim YJ, Bakhtiar S, Atschekzei F, Schmidt R, Sogkas G, Chandrakasan S, Rae W, Derfalvi B, Marquart HV, Ozen A, Kiykim A, Karakoc-Aydiner E, Králíčková P, de Bree G, Kiritsi D, Seidel MG, Kobbe R, Dantzer J, Alsina L, Armangue T, Lougaris V, Agyeman P, Nyström S, Buchbinder D, Arkwright PD, Grimbacher B
J Allergy Clin Immunol 2022 Feb;149(2):736-746. Epub 2021 Jun 7 doi: 10.1016/j.jaci.2021.04.039. PMID: 34111452
Ultra-rare cystic disease.
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
Lung complications of Sjogren syndrome.
Luppi F, Sebastiani M, Sverzellati N, Cavazza A, Salvarani C, Manfredi A
Eur Respir Rev 2020 Sep 30;29(157) Epub 2020 Aug 18 doi: 10.1183/16000617.0021-2020. PMID: 32817113Free PMC Article
Pulmonary manifestations of Sjögren's syndrome.
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

Thoracic manifestations of IgG4-related disease.
Muller R, Ebbo M, Habert P, Daniel L, Briantais A, Chanez P, Gaubert JY, Schleinitz N
Respirology 2023 Feb;28(2):120-131. Epub 2022 Nov 27 doi: 10.1111/resp.14422. PMID: 36437514Free PMC Article
Therapeutic options for CTLA-4 insufficiency.
Egg D, Rump IC, Mitsuiki N, Rojas-Restrepo J, Maccari ME, Schwab C, Gabrysch A, Warnatz K, Goldacker S, Patiño V, Wolff D, Okada S, Hayakawa S, Shikama Y, Kanda K, Imai K, Sotomatsu M, Kuwashima M, Kamiya T, Morio T, Matsumoto K, Mori T, Yoshimoto Y, Dybedal I, Kanariou M, Kucuk ZY, Chapdelaine H, Petruzelkova L, Lorenz HM, Sullivan KE, Heimall J, Moutschen M, Litzman J, Recher M, Albert MH, Hauck F, Seneviratne S, Pachlopnik Schmid J, Kolios A, Unglik G, Klemann C, Snapper S, Giulino-Roth L, Svaton M, Platt CD, Hambleton S, Neth O, Gosse G, Reinsch S, Holzinger D, Kim YJ, Bakhtiar S, Atschekzei F, Schmidt R, Sogkas G, Chandrakasan S, Rae W, Derfalvi B, Marquart HV, Ozen A, Kiykim A, Karakoc-Aydiner E, Králíčková P, de Bree G, Kiritsi D, Seidel MG, Kobbe R, Dantzer J, Alsina L, Armangue T, Lougaris V, Agyeman P, Nyström S, Buchbinder D, Arkwright PD, Grimbacher B
J Allergy Clin Immunol 2022 Feb;149(2):736-746. Epub 2021 Jun 7 doi: 10.1016/j.jaci.2021.04.039. PMID: 34111452
Lung complications of Sjogren syndrome.
Luppi F, Sebastiani M, Sverzellati N, Cavazza A, Salvarani C, Manfredi A
Eur Respir Rev 2020 Sep 30;29(157) Epub 2020 Aug 18 doi: 10.1183/16000617.0021-2020. PMID: 32817113Free PMC Article
Lymphocytic Interstitial Pneumonia.
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
Pulmonary manifestations of Sjögren's syndrome.
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

Therapy

Efficacy, safety, and biomarker analysis of nivolumab in combination with abemaciclib plus endocrine therapy in patients with HR-positive HER2-negative metastatic breast cancer: a phase II study (WJOG11418B NEWFLAME trial).
Masuda J, Sakai H, Tsurutani J, Tanabe Y, Masuda N, Iwasa T, Takahashi M, Futamura M, Matsumoto K, Aogi K, Iwata H, Hosonaga M, Mukohara T, Yoshimura K, Imamura CK, Miura S, Yamochi T, Kawabata H, Yasojima H, Tomioka N, Yoshimura K, Takano T
J Immunother Cancer 2023 Sep;11(9) doi: 10.1136/jitc-2023-007126. PMID: 37709297Free PMC Article
Lung complications of Sjogren syndrome.
Luppi F, Sebastiani M, Sverzellati N, Cavazza A, Salvarani C, Manfredi A
Eur Respir Rev 2020 Sep 30;29(157) Epub 2020 Aug 18 doi: 10.1183/16000617.0021-2020. PMID: 32817113Free PMC Article
Lymphocytic Interstitial Pneumonia.
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
Pulmonary manifestations of Sjögren's syndrome.
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
Diffuse panbronchiolitis.
Poletti V, Casoni G, Chilosi M, Zompatori M
Eur Respir J 2006 Oct;28(4):862-71. doi: 10.1183/09031936.06.00131805. PMID: 17012632

Prognosis

Pulmonary Manifestations of Sjögren's Disease.
Byrne L, McCarthy C, Fabre A, Gupta N
Semin Respir Crit Care Med 2024 Jun;45(3):397-410. Epub 2024 Apr 15 doi: 10.1055/s-0044-1785675. PMID: 38621712
Single-cell profiling reveals distinct adaptive immune hallmarks in MDA5+ dermatomyositis with therapeutic implications.
Ye Y, Chen Z, Jiang S, Jia F, Li T, Lu X, Xue J, Lian X, Ma J, Hao P, Lu L, Ye S, Shen N, Bao C, Fu Q, Zhang X
Nat Commun 2022 Oct 29;13(1):6458. doi: 10.1038/s41467-022-34145-4. PMID: 36309526Free PMC Article
Lung complications of Sjogren syndrome.
Luppi F, Sebastiani M, Sverzellati N, Cavazza A, Salvarani C, Manfredi A
Eur Respir Rev 2020 Sep 30;29(157) Epub 2020 Aug 18 doi: 10.1183/16000617.0021-2020. PMID: 32817113Free PMC Article
Pulmonary manifestations of Sjögren's syndrome.
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
Sjögren's Syndrome.
Papiris SA, Tsonis IA, Moutsopoulos HM
Semin Respir Crit Care Med 2007 Aug;28(4):459-71. doi: 10.1055/s-2007-985667. PMID: 17764063

Clinical prediction guides

Single-cell profiling reveals distinct adaptive immune hallmarks in MDA5+ dermatomyositis with therapeutic implications.
Ye Y, Chen Z, Jiang S, Jia F, Li T, Lu X, Xue J, Lian X, Ma J, Hao P, Lu L, Ye S, Shen N, Bao C, Fu Q, Zhang X
Nat Commun 2022 Oct 29;13(1):6458. doi: 10.1038/s41467-022-34145-4. PMID: 36309526Free PMC Article
Extracellular HMGB1 blockade inhibits tumor growth through profoundly remodeling immune microenvironment and enhances checkpoint inhibitor-based immunotherapy.
Hubert P, Roncarati P, Demoulin S, Pilard C, Ancion M, Reynders C, Lerho T, Bruyere D, Lebeau A, Radermecker C, Meunier M, Nokin MJ, Hendrick E, Peulen O, Delvenne P, Herfs M
J Immunother Cancer 2021 Mar;9(3) doi: 10.1136/jitc-2020-001966. PMID: 33712445Free PMC Article
The pathogenicity of SARS-CoV-2 in hACE2 transgenic mice.
Bao L, Deng W, Huang B, Gao H, Liu J, Ren L, Wei Q, Yu P, Xu Y, Qi F, Qu Y, Li F, Lv Q, Wang W, Xue J, Gong S, Liu M, Wang G, Wang S, Song Z, Zhao L, Liu P, Zhao L, Ye F, Wang H, Zhou W, Zhu N, Zhen W, Yu H, Zhang X, Guo L, Chen L, Wang C, Wang Y, Wang X, Xiao Y, Sun Q, Liu H, Zhu F, Ma C, Yan L, Yang M, Han J, Xu W, Tan W, Peng X, Jin Q, Wu G, Qin C
Nature 2020 Jul;583(7818):830-833. Epub 2020 May 7 doi: 10.1038/s41586-020-2312-y. PMID: 32380511
British Lung Foundation/United Kingdom Primary Immunodeficiency Network Consensus Statement on the Definition, Diagnosis, and Management of Granulomatous-Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency Disorders.
Hurst JR, Verma N, Lowe D, Baxendale HE, Jolles S, Kelleher P, Longhurst HJ, Patel SY, Renzoni EA, Sander CR, Avery GR, Babar JL, Buckland MS, Burns S, Egner W, Gompels MM, Gordins P, Haddock JA, Hart SP, Hayman GR, Herriot R, Hoyles RK, Huissoon AP, Jacob J, Nicholson AG, Rassl DM, Sargur RB, Savic S, Seneviratne SL, Sheaff M, Vaitla PM, Walters GI, Whitehouse JL, Wright PA, Condliffe AM
J Allergy Clin Immunol Pract 2017 Jul-Aug;5(4):938-945. Epub 2017 Mar 25 doi: 10.1016/j.jaip.2017.01.021. PMID: 28351785
Pulmonary manifestations of Sjögren's syndrome.
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

Recent systematic reviews

Airway disorders associated with immune checkpoint inhibitor therapy: Two case reports and a systematic review.
Kawakami N, Saito H, Takahashi S, Kajie S, Kato R, Shimaya K, Wakai Y, Saito K, Sakashita M
Semin Oncol 2022 Dec;49(6):439-455. Epub 2023 Feb 2 doi: 10.1053/j.seminoncol.2023.01.003. PMID: 36759235

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