Immunodeficiency in the differential diagnosis of interstitial lung diseases
Authors:
Martina Doubková 1; Jana Špeldová 1; Zita Chovancová 2
Authors‘ workplace:
Klinika nemocí plicních a tuberkulózy LF MU a FN Brno, pracoviště Bohunice
1; Ústav klinické imunologie a alergologie LF MU a FN u sv. Anny v Brně
2
Published in:
Vnitř Lék 2019; 65(11): 685-693
Category:
Overview
Interstitial lung processes (IPP), or diffuse parenchymal lung diseases, are a broad group of diseases characterized by varying degrees of pulmonary fibrosis and inflammation affecting predominantly, but not exclusively, pulmonary interstitium. IPP mostly occur in adulthood with maximum manifestation between 40 and 70 years of age. Although IPP mostly present as a primary diagnosis, they also belong to the portfolio of pulmonary disorders in patients with primary immunodeficiencies. The authors present case reports of patients with interstitial lung involvement and primary immunodeficiencies [particularly those manifesting also in adulthood, such as common variable immunodeficiency (CVID), and cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4) deficiency]. In addition, we report the case of silicosis patient with severe lymphopenia. Therefore, in patients with newly diagnosed interstitial lung disease, congenital immune system disorder should be considered. Basic immunological laboratory examination of humoral and cellular immunity should be an essential part of the differential diagnosis algorithm for interstitial lung disease.
Keywords:
common variable immunodeficiency – cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4) deficiency – immunology laboratory investigation – interstitial lung disease – primary immunodeficiencies
Sources
- [American Thoracic Society; European Respiratory Society]. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med 2002; 165(2): 277–304. Dostupné z DOI: <http://dx.doi.org/10.1164/ajrccm.165.2.ats01>.
- Bienenstock J, Johnston N, Perey DY. Bronchial lymphoid tissue. I. Morphologic characteristics. Lab Invest 1973; 28(6): 686–692.
- Bienenstock J, Johnston N, Perey DY. Bronchial lymphoid tissue. II. Functional characteristics. Lab Invest 1973; 28(6): 693–698.
- Pabst R, Tschernig T. Bronchus-associated lymphoid tissue: an entry site for antigens for successful mucosal vaccinations? Am J Respir Cell Mol Biol 2010; 43(2): 137–141. Dostupné z DOI: <http://dx.doi.org/10.1165/rcmb.2010–0152RT>.
- Neyt K, Perros F, Geurtsvan Kessel CH et al. Tertiary lymphoid organs in infection and autoimmunity. Trends Immunol 2012; 33(6): 297–305. Dostupné z DOI: <http://dx.doi.org/10.1016/j.it.2012.04.006>.
- Rangel-Moreno J, Hartson L, Navarro C et al. Inducible bronchus-associated lymphoid tissue (iBALT) in patients with pulmonary complications of rheumatoid arthritis. J Clin Invest 2006; 116(12): 3183–3194. Dostupné z DOI: <http://dx.doi.org/10.1172/JCI28756>.
- Jordan M, Haczku A. Autoreactive bronchus-associated lymphoid tissue in interstitial lung disease: friend or foe? Am J Respir Cell Mol Biol 2013; 48(4): 397–398.
- Saikia B, Gupta S. Common Variable Immunodeficiency. Indian J Pediatr 2016; 83: 338–344. Dostupné z DOI: <http://dx.doi.org/10.1007/s12098–016–2038-x>.
- Bonilla FA, Barlan I, Chapel H et al. International Consensus Document (ICON): Common Variable Immunodeficiency Disorders. J Allergy Clin Immunol Pract 2016; 4(1): 38–59. Dostupné z DOI: <http://dx.doi.org/10.1016/j.jaip.2015.07.025>.
- Gathmann B, Mahlaoui N, Gérard L et al. Clinical picture and treatment of 2212 patients with common variable immunodeficiency. J Allergy Clin Immunol 2014; 134(1): 116–126. Dostupné z DOI: <http://dx.doi.org/10.1016/j.jaci.2013.12.1077>.
- Abbott JK, Gelfand EW. Common Variable Immunodeficiency: Diagnosis, Management, and Treatment. Immunol Allergy Clin North Am 2015; 35(4): 637–658. Dostupné z DOI: <http://dx.doi.org/10.1016/j.iac.2015.07.009>.
- Lo B, Abdel-Motal UM. Lessons from CTLA-4 deficiency and checkpoint inhibition. Curr Opin Immunol 2017; 49: 14–19. Dostupné z DOI: <http://dx.doi.org/10.1016/j.coi.2017.07.014>.
- Rowshanravan B, Halliday N, Sansom D. CTLA-4: a moving target in immunotherapy. Blood 2018; 131(1): 58–67. Dostupné z DOI: <http://dx.doi.org/10.1182/blood-2017–06–741033>.
- Králíčková P, Kubcová Š, Kočová E et al. Successful rituximab treatment of granulomatous/lymphocytic interstitial lung disease in common variable immunodeficiency. Epidemiol Mikrobiol Imunol 2018; 67(3): 142–148.
- Verma N, Burns SO, Walker LSK et al. Immune deficiency and autoimmunity in patients with CTLA-4 (CD152) mutations. Clin Exp Immunol 2017; 190(1): 1–7. Dostupné z DOI: <http://dx.doi.org/10.1111/cei.12997>.
- Chitale S, Moots R. Abatacept: the first T lymphocyte co-stimulation modulator, for the treatment of rheumatoid arthritis. Expert Opin Biol Ther 2008; 8(1): 115–122. Dostupné z DOI: <http://dx.doi.org/10.1517/14712598.8.1.115>.
- [Centers for Disease Control (CDC)]. Unexplained CD4+ T-lymphocyte depletion in persons without evident HIV infection – United States. MMWR Morb Mortal Wkly Rep 1992; 41(30): 541–545.
- Yarmohammadi H, Cunningham-Rundles C. Idiopathic CD4 lymphocytopenia: Pathogenesis, etiologies, clinical presentations and treatment strategies. Ann Allergy Asthma Immunol 2017; 119(4): 374–378. Dostupné z DOI: <http://dx.doi.org/10.1016/j.anai.2017.07.021>.
- Asher I, Mahlab-Guri K, Elbirt D et al. Idiopathic CD4 Lymphopenia: Severe CD4 Lymphopenia in the Absence of Human Immunodeficiency Virus Infection. Isr Med Assoc J 2016; 18(10): 627–629.
- Walker UA, Warnatz K. Idiopathic CD4 lymphocytopenia. Curr Opin Rheumatol 2006; 18(4): 389–395. Dostupné z DOI: <http://dx.doi.org/10.1097/01.bor.0000231908.57913.2f>.
- Vašáková M, Polák J, Matěj R. Intersticiální plicní procesy: od etiopatogeneze přes radiologický obraz k histopatologické diagnóze. Maxdorf: Praha 2016. ISBN 978–80–7345–488–3.
- Chapel H, Lucas M, Lee M et al. Common variable immunodeficiency disorders: division into distinct clinical phenotypes. Blood 2008; 112(2): 277–286. Dostupné z DOI: <http://dx.doi.org/10.1182/blood-2007–11–124545>.
- Prasse A, Kayser G, Warnatz K. Common variable immunodeficiency-associated granulomatous and interstitial lung disease. Curr Opin Pulm Med 2013; 19(5): 503–509. Dostupné z DOI: <http://dx.doi.org/10.1097/MCP.0b013e3283642c47>.
- Maglione PJ, Ko HM, Beasley MB et al. Tertiary lymphoid neogenesis is a component of pulmonary lymphoid hyperplasia in patients with common variable immunodeficiency. J Allergy Clin Immunol 2014; 133(2): 535–542. Dostupné z DOI: <http://dx.doi.org/10.1016/j.jaci.2013.08.022>.
- Giovannetti A, Pierdominici M, Mazzetta F et al. Unravelling the complexity of T cell abnormalities in common variable immunodeficiency. J Immunol 2007; 178(6): 3932–3943. Dostupné z DOI: <http://dx.doi.org/10.4049/jimmunol.178.6.3932>.
- Warnatz K, Voll RE. Pathogenesis of autoimmunity in common variable immunodeficiency. Front Immunol 2012; 3: 210. Dostupné z DOI: <http://dx.doi.org/10.3389/fimmu.2012.00210>.
- Sánchez-Ramón S, Radigan L, Yu JE et al. Memory B cells in common variable immunodeficiency: clinical associations and sex differences. Clin Immunol 2008; 128(3): 314–321. Dostupné z DOI: <http://dx.doi.org/10.1016/j.clim.2008.02.013>.
- Wehr C, Kivioja T, Schmitt C et al. The EUROclass trial: defining subgroups in common variable immunodeficiency. Blood 2008; 111(1): 77–85. Dostupné z DOI: <http://dx.doi.org/10.1182/blood-2007–06–091744>.
- Warnatz K, Wehr C, Dräger R et al. Expansion of CD19(hi)CD21(lo/neg) B cells in common variable immunodeficiency (CVID) patients with autoimmune cytopenia. Immunobiology 2002; 206(5): 502–513. Dostupné z DOI: <http://dx.doi.org/10.1078/0171–2985–00198>.
- Unger S, Seidl M, van Schouwenburg P et al. The TH1 phenotype of follicular helper T cells indicates an IFN-γ-associated immune dysregulation in patients with CD21low common variable immunodeficiency. J Allergy Clin Immunol 2018; 141(2): 730–740. Dostupné z DOI: <http://dx.doi.org/10.1016/j.jaci.2017.04.041>.
- Park J, Munagala I, Xu H et al. Interferon signature in the blood in inflammatory common variable immune deficiency. PLoS One 2013; 8(9): e74893. Dostupné z DOI: <http://dx.doi.org/10.1371/journal.pone.0074893>.
- Cols M, Rahman A, Maglione PJ et al. Expansion of inflammatory innate lymphoid cells in patients with common variable immune deficiency. J Allergy Clin Immunol 2016; 137(4): 1206–1215. Dostupné z DOI: <http://dx.doi.org/10.1016/j.jaci.2015.09.013>.
- Coraglia A, Galassi N, Fernández Romero DS et al. Common Variable Immunodeficiency and Circulating TFH. J Immunol Res 2016; 2016: 4951587. Dostupné z DOI: <http://dx.doi.org/10.1155/2016/4951587>.
- Wheat WH, Cool CD, Morimoto Y et al. Possible role of human herpesvirus 8 in the lymphoproliferative disorders in common variable immunodeficiency. J Exp Med 2005; 202(4): 479–484. Dostupné z DOI: <http://dx.doi.org/10.1084/jem.20050381>.
- Andiman WA, Eastman R, Martin K et al. Opportunistic lymphoproliferations associated with Epstein-Barr viral DNA in infants and children with AIDS. Lancet 1985; 2(8469–8470): 1390–1393. Dostupné z DOI: <http://dx.doi.org/10.1016/s0140–6736(85)92557–7>.
- Schussler E, Beasley MB, Maglione PJ. Lung Disease in Primary Antibody Deficiencies. J Allergy Clin Immunol Pract 2016; 4(6): 1039–1052. Dostupné z DOI: <http://dx.doi.org/10.1016/j.jaip.2016.08.005>.
- Régent A, Autran B, Carcelain G et al. Idiopathic CD4 lymphocytopenia: clinical and immunologic characteristics and follow-up of 40 patients. Medicine (Baltimore) 2014; 93(2): 61–72. Dostupné z DOI: <http://dx.doi.org/10.1097/MD.0000000000000017>.
- Cunningham-Rundles C, Bodian C. Common variable immunodeficiency: clinical and immunological features of 248 patients. Clin Immunol 1999; 92(1): 34–48. Dostupné z DOI: <http://dx.doi.org/10.1006/clim.1999.4725>.
- Ardeniz O, Cunningham-Rundles C. Granulomatous disease in common variable immunodeficiency. Clin Immunol 2009; 133(2): 198–207. Dostupné z DOI: <http://dx.doi.org/10.1016/j.clim.2009.05.001>.
- Park JH, Levinson AI. Granulomatous-lymphocytic interstitial lung disease (GLILD) in common variable immunodeficiency (CVID). Clin Immunol 2010; 134(2): 97–103. Dostupné z DOI: <http://dx.doi.org/10.1016/j.clim.2009.10.002>.
- Bates CA, Ellison MC, Lynch DA et al. Granulomatous-lymphocytic lung disease shortens survival in common variable immunodeficiency. J Allergy Clin Immunol 2004; 114(2): 415–421. Dostupné z DOI: <http://dx.doi.org/10.1016/j.jaci.2004.05.057>.
- Perez RL, Rivera-Marrero CA, Roman J. Pulmonary granulomatous inflammation: From sarcoidosis to tuberculosis. Semin Respir Infect 2003; 18(1): 23–32. Dostupné z DOI: <http://dx.doi.org/10.1053/srin.2003.50005>.
- Bouvry D, Mouthon L, Brillet PY et al. Granulomatosis-associated common variable immunodeficiency disorder: a case-control study versus sarcoidosis. Eur Respir J 2013; 41(1): 115–122. Dostupné z DOI: <http://dx.doi.org/10.1183/09031936.00189011>.
- Resnick ES, Moshier EL, Godbold JH et al. Morbidity and mortality in common variable immune deficiency over 4 decades. Blood 2012; 119(7): 1650–1657. Dostupné z DOI: <http://dx.doi.org/10.1182/blood-2011–09–377945>.
- Boursiquot JN, Gérard L, Malphettes M et al. Granulomatous disease in CVID: retrospective analysis of clinical characteristics and treatment efficacy in a cohort of 59 patients. J Clin Immunol 2013; 33(1): 84–95. Dostupné z DOI: <http://dx.doi.org/10.1007/s10875–012–9778–9>.
- Verbsky JW, Routes JM. Sarcoidosis and common variable immunodeficiency: similarities and differences. Semin Respir Crit Care Med 2014; 35(3): 330–335. <http://dx.doi.org/10.1055/s-0034–1376862>.
- Doubková M, Moulis M, Skřičková J. Intersticiální plicní procesy a granulomatózy asociované s běžným variabilním imunodeficitem. Vnitř Lék 2015; 61(2): 119–124.
- Quinti I, Soresina A, Spadaro G et al. Long-term follow-up and outcome of a large cohort of patients with common variable immunodeficiency. J Clin Immunol 2007; 27(3): 308–316. Dostupné z DOI: <http://dx.doi.org/10.1007/s10875–007–9075–1>.
- Hatab AZ, Ballas ZK. Caseating granulomatous disease in common variable immunodeficiency treated with infliximab. J Allergy Clin Immunol 2005; 116(5): 1161–1162. Dostupné z DOI: <http://dx.doi.org/10.1016/j.jaci.2005.08.041>.
- Chase NM, Verbsky JW, Hintermeyer MK et al. Use of combination chemotherapy for treatment of granulomatous and lymphocytic interstitial lung disease (GLILD) in patients with common variable immunodeficiency (CVID). J Clin Immunol 2013; 33(1): 30–39. Dostupné z DOI: <http://dx.doi.org/10.1007/s10875–012–9755–3>.
- Stasi R, Del Poeta G, Stipa E et al. Response to B-cell depleting therapy with rituximab reverts the abnormalities of T-cell subsets in patients with idiopathic thrombocytopenic purpura. Blood 2007; 110(8): 2924–2930. Dostupné z DOI: <http://dx.doi.org/10.1182/blood-2007–02–068999>.
- Doubková M, Pokorná J. Autoprotilátky u systémových onemocnění pojiva a ANCA asociovaných vaskulitid, jejich vztah k intersticiálním plicním procesům a prognóze. Vnitř Lék 2017; 63(2): 98–106.
- Boesecke C, Rockstroh JK, Thoden J. Opportunistic infections: What’s new?. Dtsch Med Wochenschr 2018; 143(24): 1755–1758. Dostupné z DOI: <http://dx.doi.org/10.1055/a-0641–9456>.
Labels
Diabetology Endocrinology Internal medicineArticle was published in
Internal Medicine
![Issue 11](https://www.prolekare.cz/media/cache/resolve/journal_issue/media/image/e5782888ebae9f107f4026ec96a2e5c2.png)
2019 Issue 11
Most read in this issue
- Functional gastrointestinal disorders and pain
- Interferon-alpha in the treatment of myeloproliferative diseases
- Vitamin D deficiency and its health effects
- Immunodeficiency in the differential diagnosis of interstitial lung diseases