REVIEW PAPER
Haematological abnormalities in systemic sclerosis
1
Department of Rheumatology and Connective Tissue Diseases, Medical University of Lublin, Poland
Submission date: 2020-03-20
Final revision date: 2020-05-10
Acceptance date: 2020-06-02
Online publication date: 2020-06-29
Publication date: 2020-06-29
Reumatologia 2020;58(3):162-166
KEYWORDS
TOPICS
ABSTRACT
Systemic sclerosis (SSc) is a connective tissue disease characterised by extremely high heterogeneity. This heterogeneity concerns the organ involvement, course of disease and prognosis. Unlike in some other systemic connective tissue diseases, especially systemic lupus erythematosus, in SSc haematological disorders occur rarely. When they develop, they affect erythrocytes, leucocytes and platelets. The most common cause of this pathology of erythrocyte abnormalities is microcytic anaemia resulting from micro-haemorrhages with telangiectasias within the digestive mucosa in patients with SSc. In SSc patients with severe haematological disturbances, the differential diagnosis should include overlapping with another systemic connective tissue disease or a haemato-oncological disease (lympho/myeloproliferative syndrome). In SSc patients with monoclonal proteins or cryoglobulins, it is essential to consider a haemato-oncological disease. In such cases, the differential diagnosis should be focused on a paraneoplastic syndrome, especially when the haematological symptoms develop shortly after the diagnosis of SSc and in the elderly.
REFERENCES (32)
1.
Valentini G, Iudici M, Walker UA, et al. The European Scleroderma Trials and Research group (EUSTAR) task force for the development of revised activity criteria for systemic sclerosis: derivation and validation of a preliminarily revised EUSTAR activity index. Ann Rheum Dis 2017; 76: 270-276, DOI: 10.1136/annrheumdis-2016-209768.
2.
González-Naranjo LA, Betancur OM, Alarcón GS, et al. Features associated with hematologic abnormalities and their impact in patients with systemic lupus erythematosus: data from a multiethnic Latin American cohort. Semin Arthritis Rheum 2016; 45: 675-678, DOI: 10.1016/j.semarthrit.2015.11.003.
3.
Hax V, de Conto Oliveira J. Gastric antral vascular ectasia causing refractory anemia in a patient with systemic sclerosis.
4.
J Clin Rheumatol 2019, DOI: 10.1097/RHU.0000000000001054 [Epub ahead of print].
5.
El-Gendy H, Shohdy KS, Maghraby GG, et al. Gastric antral vascular ectasia in systemic sclerosis: Where do we stand? Int J Rheum Dis 2017; 20: 2133-2139, DOI: 10.1111/1756-185X.13047.
6.
Turrión Nieves AI, Moruno Cruz H, Romero Bogado ML, Perez Gómez A. Upper gastrointestinal bleeding (watermelon stomach) in a patient with limited scleroderma (CREST syndrome). Reumatol Clin 2017; 13: 361-362, DOI: 10.1016/j.reumae.2016.05.012.
7.
Quigley EMM. The spectrum of small intestinal bacterial overgrowth (SIBO). Curr Gastroenterol Rep 2019; 21: 3, DOI: 10.1007/s11894-019-0671-z.
8.
Amiot A, Joly F, Alves A, et al. Long-term outcome of chronic intestinal pseudo-obstruction adult patients requiring home parenteral nutrition. Am J Gastroenterol 2009; 104: 1262-1270, DOI: 10.1038/ajg.2009.58.
9.
Gyger G, Baron M. Systemic sclerosis: gastrointestinal disease and its management. Rheum Dis Clin North Am 2015; 41: 459-473, DOI: 10.1016/j.rdc.2015.04.007.
10.
Marie I, Leroi A-M, Gourcerol G, et al. Lactose malabsorption in systemic sclerosis. Aliment Pharmacol Ther 2016; 44: 1123-1133, DOI: 10.1111/apt.13810.
11.
Kumar S, Singh J, Rattan S, et al. Review article: pathogenesis and clinical manifestations of gastrointestinal involvement in systemic sclerosis. Aliment Pharmacol Ther 2017, 45: 883-898, DOI: 10.1111/apt.13963.
12.
Gavriilaki E, Anagnostopoulos A, Mastellos DC. Complement in thrombotic microangiopathies: unraveling Ariadne’s thread into the labyrinth of complement therapeutics. Front Immunol 2019; 10: 337, DOI: 10.3389/fimmu.2019.00337.
13.
Frayha RA, Shulman LE, Stevens MB. Hematological abnormalities in scleroderma. A Study of 180 Cases. Acta Haematol 1980; 64: 25-30, DOI: 10.1159/000207206.
14.
Ruiter G, Lanser IJ, de Man FS, et al. Iron deficiency in systemic sclerosis patients with and without pulmonary hypertension. Rheumatology (Oxford) 2014; 53: 285-292, DOI: 10.1093/rheumatology/ket331.
15.
Sargın G, Şentürk T, Yavaşoğlu İ. Refractory anemia in systemic sclerosis: myelodisplastic syndrome. Eur J Rheumatol 2015; 2: 120-121, DOI: 10.5152/eurjrheum.2015.0100.
16.
Forestier A, Guerrier T, Jouvray M, et al. Altered B lymphocyte homeostasis and functions in systemic sclerosis. Autoimmun Rev 2018; 17: 244-255, DOI: 10.1016/j.autrev.2017.10.015.
17.
Giovannetti A, Rosato E, Renzi C, et al. Analyses of T cell phenotype and function reveal an altered T cell homeostasis in systemic sclerosis. Correlations with disease severity and phenotypes. Clin Immunol 2010; 137: 122-133, DOI: 10.1016/j.clim.2010.06.004.
18.
Reiff A, Weinberg KI, Triche T, et al. T lymphocyte abnormalities in juvenile systemic sclerosis patients. Clin Immunol 2013; 149: 146-155, DOI: 10.1016/j.clim.2013.07.005.
19.
Manzella DJ, Vicente L, Pérez de la Hoz AA, et al. Catastrophic antiphospholipid syndrome as a complication of systemic sclerosis. Reumatismo 2019; 71: 92-98, DOI: 10.4081/reumatismo.2019.1095.
20.
Merashli M, Alves JD, Ames PRJ. Clinical relevance of antiphospholipid antibodies in systemic sclerosis: A systematic review and meta-analysis. Semin Arthritis Rheum 2017; 46: 615-624, DOI: 10.1016/j.semarthrit.2016.10.004.
21.
Vavlukis M, Kotlar I, Chaparoska E, et al. Antiphospholipid syndrome – A case report of pulmonary thromboembolism, Followed with acute myocardial infarction in patient with systemic sclerosis. Open Access Maced J Med Sci 2015; 3: 705-709, DOI: 10.3889/oamjms.2015.114.
22.
Rossi FW, Lobasso A, Selleri C, et al. Autoimmune tautology in a complex case of polyautoimmunity: systemic sclerosis, autoimmune liver involvement, antiphospholipid syndrome and Hashimoto’s thyroiditis. Isr Med Assoc J 2017; 19: 193-195.
23.
Yang Y, Zhang L, Tang Y, et al. Systemic sclerosis with thrombotic thrombocytopenia purpura and malignant hypertension. Ren Fail 2012; 34: 1170-1172, DOI: 10.3109/0886022X.2012.712858.
24.
Wangkaew S, Lertthanaphok S, Puntana S, Noppakun K. Risk factors and outcome of Thai patients with scleroderma renal crisis: a disease duration-matched case control study. Int J Rheum Dis 2017; 20: 1562-1571, DOI: 10.1111/1756-185X.13145.
25.
Babar F, Cohen SD. Thrombotic microangiopathies with rheumatologic involvement. Rheum Dis Clin North Am 2018; 44: 635-649, DOI: 10.1016/j.rdc.2018.06.010.
26.
Keeler E, Fioravanti G, Samuel B, Longo S. Scleroderma renal crisis or thrombotic thrombocytopenic purpura: seeing through the masquerade. Lab Med 2015; 46: e39-44, DOI: 10.1309/LM72AM5XFHZYOQCB.
27.
Colaci M, Giuggioli D, Vacchi C, Ferri C. Hematological malignancies in systemic sclerosis patients: case reports and review of the world literature. Case Rep Rheumatol 2017; 6230138, DOI: 10.1155/2017/6230138.
28.
Vettori S, Staibano S, Mascolo M, et al. Non-Hodgkin’s lymphoma in systemic sclerosis: case and literature review. Clin Rheumatol 2010; 29: 1-6, DOI: 10.1007/s10067-009-1286-9.
29.
Partouche L, Goulabchand R, Maria ATJ, et al. Biphasic temporal relationship between cancers and systemic sclerosis: a clinical series from Montpellier University Hospital and review of the literature. J Clin Med 2020; 9: 853, DOI: 10.3390/jcm9030853.
30.
Trad S, Nosbaum A, Musset L, et al. Systemic sclerosis and prevalence of monoclonal immunoglobulin. Autoimmun Rev 2014; 13: 1189-1194, DOI: 10.1016/j.autrev.2014.08.016.
31.
Hasegawa M, Orito H, Yamamoto K, et al. Skin sclerosis as a manifestation of POEMS syndrome. J Dermatol 2012; 39: 922-926, DOI: 10.1111/j.1346-8138.2012.01609.x.
32.
Giuggioli D, Manfredi A, Colaci M, et al. Systemic sclerosis and cryoglobulinemia: our experience with overlapping syndrome of scleroderma and severe cryoglobulinemic vasculitis and review of the literature. Autoimmun Rev 2013; 12: 1058-1063, DOI: 10.1016/j.autrev.2013.06.013.
Copyright: © Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie. This is an Open Access journal, all articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0) License (https://creativecommons.org/licenses/by-nc-sa/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
Share
RELATED ARTICLE
| eISSN: | 2084-9834 |
| ISSN: | 0034-6233 |
We process personal data collected when visiting the website. The function of obtaining information about users and their behavior is carried out by voluntarily entered information in forms and saving cookies in end devices. Data, including cookies, are used to provide services, improve the user experience and to analyze the traffic in accordance with the Privacy policy. Data are also collected and processed by Google Analytics tool (more).
You can change cookies settings in your browser. Restricted use of cookies in the browser configuration may affect some functionalities of the website.
You can change cookies settings in your browser. Restricted use of cookies in the browser configuration may affect some functionalities of the website.
