Granulomatous lymphocytic interstitial lung disease in a patient with Sjögren’s Disease
case report
DOI:
https://doi.org/10.47456/rbps.v26i1.51215Keywords:
Common Variable Immunodeficiencies, Sjogren’s Disease, Interstitial Lung DiseasesAbstract
Introduction: Sjögren’s Disease (SjD) is a multisystem autoimmune disease that can affect the lungs, leading to interstitial lung disease (ILD). Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) is a rare form of ILD, commonly associated with Common Variable Immunodeficiency (CVID), but rarely described in cases of secondary immunodeficiency. Case report: We present the case of a 51-year-old female patient diagnosed with SD in 2021, who developed Nonspecific Interstitial Pneumonia (NSIP). After treatment with Cyclophosphamide and Mycophenolate Mofetil, the patient developed hypogammaglobulinemia, without fulfilling CVID criteria, suggesting secondary immunodeficiency. In 2024, decreased pulmonary function and nodular consolidations appeared in the lung bases. Pulmonary biopsy revealed lymphocytic and granulomatous infiltration, confirming GLILD. Following treatment with corticosteroids and Rituximab, the patient showed a good initial response. Discussion: Although the overlap of SjD and CVID has been described, no reports of GLILD in patients with SjD, with or without CVID, have been identified to date. Hypogammaglobulinemia in SjD is recognized as a clinical manifestation and a marker of disease activity. Pulmonary biopsy plays a key role in differentiating forms of ILD in patients with autoimmune diseases and immunodeficiencies. Conclusion: This case highlights the importance of considering GLILD as a differential diagnosis of ILD in patients with SjD and hypogammaglobulinemia, even in the absence of CVID. A multidisciplinary approach and histological investigation were essential for appropriate management and contributed to the favorable outcome of the patient.
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Negrini S, Emmi G, Greco M, Borro M, Sardanelli F, Murdaca G, et al. Sjögren’s syndrome: a systemic autoimmune disease. Clin Exp Med [Internet]. 2022;22(1):9–25. Disponível em: http://dx.doi.org/10.1007/s10238-021-00728-6
Valim V, Zandonade E, Pereira AM, de Brito Filho OH, Serrano EV, Musso C, et al. Primary Sjögren’s syndrome prevalence in a major metropolitan area in Brazil. Rev Bras Reumatol [Internet]. 2013;53(1):24–34. Disponível em: http://dx.doi.org/10.1016/s2255-5021(13)70003-8
Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, et al. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren’s syndrome: A consensus and data-driven methodology involving three international patient cohorts. Arthritis Rheumatol [Internet]. 2017;69(1):35–45. Disponível em: http://dx.doi.org/10.1002/art.39859
Seror R, Ravaud P, Bowman SJ, Baron G, Tzioufas A, Theander E, et al. EULAR Sjögren’s syndrome disease activity index: development of a consensus systemic disease activity index for primary Sjögren’s syndrome. Ann Rheum Dis [Internet]. 2010;69(6):1103–9. Disponível em: http://dx.doi.org/10.1136/ard.2009.110619
Serrano EV, Valim V, Miyamoto ST, Giovelli RA, Paganotti MA, Cadê NV. Transcultural adaptation of the “EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI)” into Brazilian Portuguese. Rev Bras Reumatol [Internet]. 2013;53(6):483–
93. Disponível em: http://dx.doi.org/10.1016/j.rbr.2013.04.003
Ramos-Casals M, Font J, Garcia-Carrasco M, Brito M-P, Rosas J, Calvo-Alen J, et al. Primary Sjögren syndrome: hematologic patterns of disease expression. Medicine (Baltimore) [Internet]. 2002;81(4):281–92. Disponível em: http://dx.doi.org/10.1097/00005792-200207000-00004
Flament T, Bigot A, Chaigne B, Henique H, Diot E, Marchand-Adam S. Pulmonary manifestations of Sjögren’s syndrome. Eur Respir Rev [Internet]. 2016;25(140):110–23. Disponível em: http://dx.doi.org/10.1183/16000617.0011-2016
Lamers OAC, Smits BM, Leavis HL, de Bree GJ, Cunningham-Rundles C, Dalm VASH, et al. Treatment strategies for GLILD in common variable immunodeficiency: A systematic review. Front Immunol [Internet]. 2021;12:606099. Disponível em: http://dx.doi.org/10.3389/fimmu.2021.606099
Cinetto F, Scarpa R, Carrabba M, Firinu D, Lougaris V, Buso H, et al. Granulomatous Lymphocytic interstitial lung disease (GLILD) in Common Variable Immunodeficiency (CVID): A multicenter retrospective study of patients from Italian PID referral centers. Front Immunol [Internet]. 2021;12:627423. Disponível em: http://dx.doi.org/10.3389/fimmu.2021.627423
Galant-Swafford J, Catanzaro J, Achcar RD, Cool C, Koelsch T, Bang TJ, et al. Approach to diagnosing and managing granulomatous-lymphocytic interstitial lung disease. EClinicalMedicine [Internet]. 2024;75(102749):102749. Disponível em: http://dx.doi.org/10.1016/j.eclinm.2024.102749
Mucke J, Cornet A, Witte T, Schneider M. Association of common variable immunodeficiency and rare and complex connective tissue and musculoskeletal diseases. A systematic literature review. Clin Exp Rheumatol [Internet]. 2022;40 Suppl 134(5):40–5. Disponível em: http://dx.doi.org/10.55563/clinexprheumatol/bbuvih
Agarwal S, Cunningham-Rundles C. Autoimmunity in common variable immunodeficiency. Ann Allergy Asthma Immunol [Internet]. 2019;123(5):454–60. Disponível em: http://dx.doi.org/10.1016/j.anai.2019.07.014
Quartuccio L, De Marchi G, Longhino S, Manfrè V, Rizzo MT, Gandolfo S, et al. Shared pathogenetic features between common variable immunodeficiency and Sjögren’s syndrome: Clues for a personalized medicine. Front Immunol [Internet]. 2021;12:703780. Disponível em: http://dx.doi.org/10.3389/fimmu.2021.703780
Akagi K, Yamamoto K, Umemura A, Ide S, Hirayama T, Takazono T, et al. Human immunodeficiency virus-associated vacuolar encephalomyelopathy with granulomatous-lymphocytic interstitial lung disease improved after antiretroviral therapy: a case report. AIDS Res Ther [Internet]. 2020;17(1):38. Disponível em: http://dx.doi.org/10.1186/s12981-020-00295-y
Gao X, Michel K, Griese M. Interstitial lung disease in immunocompromised children. Diagnostics (Basel) [Internet]. 2022;13(1):64. Disponível em: http://dx.doi.org/10.3390/diagnostics13010064
Sasaki J, Tominaga M, Sudou M, Tokisawa S, Nishii Y, Zaizen Y, et al. Granulomatous Lymphocytic interstitial lung disease in multiple myeloma. Intern Med [Internet]. 2023;62(3):439–44. Disponível em: http://dx.doi.org/10.2169/internalmedicine.9758-22
Hurst JR, Verma N, Lowe D, Baxendale HE, Jolles S, Kelleher P, et al. 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. J Allergy Clin Immunol Pract [Internet]. 2017;5(4):938–45. Disponível em: http://dx.doi.org/10.1016/j.jaip.2017.01.021
Cottin V. Lung biopsy in interstitial lung disease: balancing the risk of surgery and diagnostic uncertainty. Eur Respir J [Internet]. 2016;48(5):1274–7. Disponível em: http://dx.doi.org/10.1183/13993003.01633-2016
Tessarin G, Baronio M, Gazzurelli L, Rossi S, Chiarini M, Moratto D, et al. Rituximab monotherapy is effective as first-line treatment for granulomatous Lymphocytic interstitial lung disease (GLILD) in CVID patients. J Clin Immunol [Internet]. 2023;43(8):2091–103. Disponível em: http://dx.doi.org/10.1007/s10875- 023-01587-4
Bucciol G, Petrone A, Putti MC. Efficacy of mycophenolate on lung disease and autoimmunity in children with immunodeficiency. Pediatr Pulmonol [Internet]. 2017;52(10):E73–6. Disponível em: http://dx.doi.org/10.1002/ppul.23757
Ramos-Casals M, Brito-Zerón P, Bombardieri S, Bootsma H, De Vita S, Dörner T, et al. EULAR recommendations for the management of Sjögren’s syndrome with topical and systemic therapies. Ann Rheum Dis [Internet]. 2020;79(1):3–18. Disponível em: http://dx.doi.org/10.1136/annrheumdis-2019-216114
Johnson SR, Bernstein EJ, Bolster MB, Chung JH, Danoff SK, George MD, et al. 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline for the treatment of interstitial lung disease in people with systemic autoimmune rheumatic diseases. Arthritis Care Res (Hoboken) [Internet]. 2024;76(8):1051–69. Disponível em: http://dx.doi.org/10.1002/acr.25348
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