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Prevalence and characteristics of inflammatory rheumatic diseases in patients with thalassemia

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Abstract

Reports of inflammatory rheumatic diseases (IRD) in thalassemia are limited. This study aimed to determine the prevalence and clinical characteristics of IRD in patients with thalassemia disease. Consecutive adult patients with thalassemia disease, confirmed by hemoglobin typing, attending the Hematology Clinic between June 2019 and May 2021 were invited to join this study. All of them had their history taken and a physical examination for IRD. Those with IRD had their medical records reviewed. Sixty-three patients (transfusion-dependent in 50) were included in this study. There was α-, β-, and co-inheritance of α- and β-thalassemia in 22.22%, 73.02%, and 4.76% of the patients, respectively, with β-thalassemia/Hb E disease in 53.97%. Twenty-three patients had IRD (rheumatoid arthritis in 9, gout in 6, systemic lupus erythematosus in 3, spondyloarthropathy in 2, and one patient each with dermatomyositis, overlap syndrome, and unclassified polyarthralgia). Clinical manifestations and laboratory findings were similar to IRD patients in general. In 40 patients without IRD, direct and indirect Coombs tests and antinuclear antibody (ANA) were positive in 51.72%, 27.59%, and 10.26%, respectively. When comparing among these 40 patients, between those with non-transfusion-dependent thalassemia (n = 10) and those with transfusion-dependent thalassemia (n = 30), the latter had non-significantly more positive direct Coombs (60.87% vs. 16.67%), indirect Coombs (30.43% vs. 16.67%), and ANA tests (13.33% vs. 0%). The prevalence of IRD in patients with thalassemia disease was rather high. Positive direct Coombs test and ANA were common in transfusion-dependent patients.

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References

  1. Viprakasit V, Ekwattanakit S (2018) Clinical classification, screening and diagnosis for thalassemia. Hematol Oncol Clin North Am 32(2):193–211. https://doi.org/10.1016/j.hoc.2017.11.006

    Article  PubMed  Google Scholar 

  2. Noureldine MHA, Taher AT, Haydar AA, Berjawi A, Khamashta MA, Uthman I (2018) Rheumatological complications of beta-thalassaemia: an overview. Rheumatology (Oxford) 57(1):19–27. https://doi.org/10.1093/rheumatology/kex058

    Article  CAS  Google Scholar 

  3. Chaloemwong J, Tantiworawit A, Rattanathammethee T, Chai-Adisaksopha C, Rattarittamrong E, Norasetthada L et al (2019) Hyperuricemia, urine uric excretion, and associated complications in thalassemia patients. Ann Hematol 98(5):1101–1110. https://doi.org/10.1007/s00277-019-03630-0

    Article  CAS  PubMed  Google Scholar 

  4. Kanthawang T, Pattamapaspong N, Louthrenoo W (2016) Acute bone infarction: a rare complication in thalassemia. Skeletal Radiol 45(7):1013–1016. https://doi.org/10.1007/s00256-016-2387-6

    Article  PubMed  Google Scholar 

  5. Altinoz MA, Gedikoglu G, Deniz G (2012) beta-Thalassemia trait association with autoimmune diseases: beta-globin locus proximity to the immunity genes or role of hemorphins? Immunopharmacol Immunotoxicol 34(2):181–190. https://doi.org/10.3109/08923973.2011.599391

    Article  CAS  PubMed  Google Scholar 

  6. Modell B, Darlison M (2008) Global epidemiology of haemoglobin disorders and derived service indicators. Bull World Health Organ 86(6):480–487. https://doi.org/10.2471/blt.06.036673

    Article  PubMed  PubMed Central  Google Scholar 

  7. Goh LPW, Chong ETJ, Lee PC (2020) Prevalence of Alpha(alpha)-Thalassemia in Southeast Asia (2010–2020): a meta-analysis involving 83,674 subjects. Int J Environ Res Public Health 17(20). https://doi.org/10.3390/ijerph17207354

  8. Kattamis A, Forni GL, Aydinok Y, Viprakasit V (2020) Changing patterns in the epidemiology of beta-thalassemia. Eur J Haematol 105(6):692–703. https://doi.org/10.1111/ejh.13512

    Article  PubMed  PubMed Central  Google Scholar 

  9. Aletaha D, Neogi T, Silman AJ, Funovits J, Felson DT, Bingham CO 3rd et al (2010) 2010 Rheumatoid arthritis classification criteria: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Arthritis Rheum 62(9):2569–2581. https://doi.org/10.1002/art.27584

    Article  PubMed  Google Scholar 

  10. Neogi T, Jansen TL, Dalbeth N, Fransen J, Schumacher HR, Berendsen D et al (2015) 2015 Gout classification criteria: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Arthritis Rheumatol 67(10):2557–2568. https://doi.org/10.1002/art.39254

    Article  PubMed  PubMed Central  Google Scholar 

  11. Hochberg MC (1997) Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 40(9):1725. https://doi.org/10.1002/art.1780400928

    Article  CAS  PubMed  Google Scholar 

  12. Petri M, Orbai AM, Alarcon GS, Gordon C, Merrill JT, Fortin PR et al (2012) Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum 64(8):2677–2686. https://doi.org/10.1002/art.34473

    Article  PubMed  PubMed Central  Google Scholar 

  13. Rudwaleit M, van der Heijde D, Landewe R, Listing J, Akkoc N, Brandt J et al (2009) The development of Assessment of SpondyloArthritis international Society classification criteria for axial spondyloarthritis (part II): validation and final selection. Ann Rheum Dis 68(6):777–783. https://doi.org/10.1136/ard.2009.108233

    Article  CAS  PubMed  Google Scholar 

  14. Rudwaleit M, van der Heijde D, Landewe R, Akkoc N, Brandt J, Chou CT et al (2011) The Assessment of SpondyloArthritis International Society classification criteria for peripheral spondyloarthritis and for spondyloarthritis in general. Ann Rheum Dis 70(1):25–31. https://doi.org/10.1136/ard.2010.133645

    Article  CAS  PubMed  Google Scholar 

  15. Bohan A, Peter JB (1975) Polymyositis and dermatomyositis (first of two parts). N Engl J Med 292(7):344–347. https://doi.org/10.1056/NEJM197502132920706

    Article  CAS  PubMed  Google Scholar 

  16. Bohan A, Peter JB (1975) Polymyositis and dermatomyositis (second of two parts). N Engl J Med 292(8):403–407. https://doi.org/10.1056/NEJM197502202920807

    Article  CAS  PubMed  Google Scholar 

  17. van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A et al (2013) 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. Arthritis Rheum 65(11):2737–2747. https://doi.org/10.1002/art.38098

    Article  PubMed  PubMed Central  Google Scholar 

  18. Pengo V, Tripodi A, Reber G, Rand JH, Ortel TL, Galli M et al (2009) Update of the guidelines for lupus anticoagulant detection. Subcommittee on lupus anticoagulant/antiphospholipid antibody of the scientific and standardisation committee of the International Society on Thrombosis and Haemostasis. J Thromb Haemost 7(10):1737–40. https://doi.org/10.1111/j.1538-7836.2009.03555.x

    Article  CAS  PubMed  Google Scholar 

  19. Pliakou XI, Koutsouka FP, Damigos D, Bourantas KL, Briasoulis EC, Voulgari PV (2012) Rheumatoid arthritis in patients with hemoglobinopathies. Rheumatol Int 32(9):2889–2892. https://doi.org/10.1007/s00296-011-2125-2

    Article  CAS  PubMed  Google Scholar 

  20. Gorriz L, De Leon C, Herrero-Beaumont G, del Vallado PF (1983) Arthritis in beta-thalassemia minor. Arthritis Rheum 26(10):1292–1293. https://doi.org/10.1002/art.1780261023

    Article  CAS  PubMed  Google Scholar 

  21. Marcolongo R, Trotta F, Scaramelli M (1975) Letter: Beta-thalassaemic trait and rheumatoid arthritis. Lancet 1(7916):1141. https://doi.org/10.1016/s0140-6736(75)92530-1

    Article  CAS  PubMed  Google Scholar 

  22. Montecucco C, Caporali R, Rossi S, Epis O (1999) Rheumatoid arthritis in beta-thalassaemia trait. Rheumatology (Oxford) 38(10):1021–1022. https://doi.org/10.1093/rheumatology/38.10.1021

    Article  CAS  Google Scholar 

  23. Castellino G, Govoni M, Trotta F (2000) Rheumatoid arthritis in beta-thalassaemia trait. Rheumatology (Oxford) 39(11):1286–1287. https://doi.org/10.1093/rheumatology/39.11.1286

    Article  CAS  Google Scholar 

  24. Caporali R, Bugatti S, Rossi S, Cavagna L, Bogliolo L, Montecucco C (2004) Rheumatoid arthritis in beta-thalassemic trait: clinical, serologic and immunogenetic profile. Joint Bone Spine 71(2):117–120. https://doi.org/10.1016/S1297-319X(03)00069-1

    Article  PubMed  Google Scholar 

  25. Keenan RT, Nowatzky J, Pillinger MH (2013) Eitology and pathogenesis of hyperuricemia and gout. In: Firestein GS, Budd R, C., Gabriel S, E., McInnes IB, O’Dell JR, eds. Kelley’s textbook of rheumatology. Philadelphia: Elavier Saunders 1533:53

    Google Scholar 

  26. Kumar V, Gruber B (2003) Tophaceous gout in a patient with thalassemia. J Clin Rheumatol 9(6):380–384. https://doi.org/10.1097/01.rhu.0000099746.74211.78

    Article  PubMed  Google Scholar 

  27. Lambeth JT, Burns-Cox CJ, MacLean R (1970) Sacroiliac gout associated with hemoglobin E and hypersplenism. Radiology 95(2):413–415. https://doi.org/10.1148/95.2.413

    Article  CAS  PubMed  Google Scholar 

  28. Paik CH, Alavi I, Dunea G, Weiner L (1970) Thalassemia and gouty arthritis. JAMA 213(2):296–297

    Article  CAS  Google Scholar 

  29. Mishra V, Sorabjee J, Mirgh S, Mishra M (2016) Tophaceous gout in thalassemia intermedia: a rare association. Oxf Med Case Reports 2016(5):105–106. https://doi.org/10.1093/omcr/omw030

    Article  PubMed  PubMed Central  Google Scholar 

  30. Agrawal BK, Marwaha S, Bhatnagar M, Parry SA, Agrawal U (2016) Association of systemic lupus erythematosus and beta thalassaemia trait- a case report. J Clin Diagn Res 10(6):OD03-4. https://doi.org/10.7860/JCDR/2016/19803.7933

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  31. Lin QY, Chen DY, Kong S, Liu WQ, Sun XF, Li DZ (2021) A rare case of Hb H disease and systemic lupus erythematosus. Hemoglobin 45(1):66–68. https://doi.org/10.1080/03630269.2021.1886945

    Article  CAS  PubMed  Google Scholar 

  32. Voulgari PV, Karali A, Alymara V, Chaidos A, Bourantas KL, Drosos AA (2005) Systemic lupus erythematosus in a patient with beta-thalassemia major. Clin Exp Rheumatol 23(2):278

    CAS  PubMed  Google Scholar 

  33. Castellino G, Govoni M, Padovan M, Rizzo N, Trotta F (2005) Beta thalassaemic trait and systemic lupus erythematosus. Ann Rheum Dis 64(4):653–654. https://doi.org/10.1136/ard.2004.023689

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  34. Castellino G, Govoni M, Trotta F (2004) Beta-thalassaemic trait and systemic lupus erythematosus. Clin Exp Rheumatol 22(3):382–383

    CAS  PubMed  Google Scholar 

  35. Berkovitch M, Laxer RM, Inman R, Koren G, Pritzker KP, Fritzler MJ et al (1994) Arthropathy in thalassaemia patients receiving deferiprone. Lancet 343(8911):1471–1472. https://doi.org/10.1016/s0140-6736(94)92585-2

    Article  CAS  PubMed  Google Scholar 

  36. Al-Refaie FN, Wonke B, Hoffbrand AV (1994) Arthropathy in thalassaemia patients receiving deferiprone. Lancet 344(8917):262–3. https://doi.org/10.1016/s0140-6736(94)93029-5

    Article  CAS  PubMed  Google Scholar 

  37. Gratwick GM, Bullough PG, Bohne WH, Markenson AL, Peterson CM (1978) Thalassemic osteoarthropathy. Ann Intern Med 88(4):494–501. https://doi.org/10.7326/0003-4819-88-4-494

    Article  CAS  PubMed  Google Scholar 

  38. Kellenberger CJ, Schmugge M, Saurenmann T, Di Gennaro L, Eber SW, Willi UV et al (2004) Radiographic and MRI features of deferiprone-related arthropathy of the knees in patients with beta-thalassemia. AJR Am J Roentgenol 183(4):989–994. https://doi.org/10.2214/ajr.183.4.1830989

    Article  PubMed  Google Scholar 

  39. Jain A, Agnihotri A, Marwaha N, Sharma RR (2016) Direct antiglobulin test positivity in multi-transfused thalassemics. Asian J Transfus Sci 10(2):161–163. https://doi.org/10.4103/0973-6247.164268

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  40. Arinsburg SA, Skerrett DL, Kleinert D, Giardina PJ, Cushing MM (2010) The significance of a positive DAT in thalassemia patients. Immunohematology 26(3):87–91

    Article  CAS  Google Scholar 

  41. Agarwal MB, Viswanathan C, Gupte SS, Desai NG, Vasandani D, Bhave AA (1992) Anti-nuclear antibody positivity in multi-transfused thalassemia major. Indian Pediatr 29(5):607–610

    CAS  PubMed  Google Scholar 

  42. Winichakoon P, Tantiworawit A, Rattanathammethee T, Hantrakool S, Chai-Adisaksopha C, Rattarittamrong E et al (2015) Prevalence and risk factors for complications in patients with nontransfusion dependent alpha- and beta-thalassemia. Anemia 2015(793025. https://doi.org/10.1155/2015/793025

  43. Jensen CE, Tuck SM, Agnew JE, Koneru S, Morris RW, Yardumian A et al (1998) High prevalence of low bone mass in thalassaemia major. Br J Haematol 103(4):911–915. https://doi.org/10.1046/j.1365-2141.1998.01108.x

    Article  CAS  PubMed  Google Scholar 

  44. Vogiatzi MG, Autio KA, Mait JE, Schneider R, Lesser M, Giardina PJ (2005) Low bone mineral density in adolescents with beta-thalassemia. Ann N Y Acad Sci 1054(462–6. https://doi.org/10.1196/annals.1345.063

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Acknowledgements

The authors thank Mrs. Waraporn Sukitawut and Ms. Phimwalan Konkaeo for their secretarial assistance and Ms. Supparat Konsamun for her statistical assistance.

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Authors and Affiliations

Authors

Contributions

Conceptualization: P.P., A.T., and W.L.

Methodology: P.P., A.T., and W.L.

Data collection: P.P., N.K., and W.L.

Data analysis: P.P. and W.L.

Interpretation of the statistical results: P.P., A.T., N.K., and W.L.

Writing the first and final draft of the manuscript: P.P. and W.L.

All of the authors provided a critical review of intellectual content and approved the final version to be submitted for publication. Dr. Louthrenoo has full access to all of the data in this study and takes responsibility for its integrity and accuracy and also that of the analysis.

Corresponding author

Correspondence to Worawit Louthrenoo.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. This study was approved by the Ethics Committee of the Faculty of Medicine, Chiang Mai University (no. 072/2019).

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All of the patients provided their written informed consent to participate and publish before entering the study.

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The authors declare no competing interests.

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Piriyakhuntorn, P., Tantiworawit, A., Kasitanon, N. et al. Prevalence and characteristics of inflammatory rheumatic diseases in patients with thalassemia. Ann Hematol 101, 1667–1675 (2022). https://doi.org/10.1007/s00277-022-04870-3

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