PRACA ORYGINALNA
Prevalence and severity of complicated Raynaud’s phenomenon in limited and diffuse systemic sclerosis: a multicenter study in Iraq
Więcej
Ukryj
1
Rheumatology Unit, Department of Medicine, College of Medicine, University of Mustansiriyah, Iraq
2
Rheumatology Unit, Department of Medicine, College of Medicine, University of Babylon, Iraq
3
Rheumatology Unit, Department of Medicine, College of Medicine, University of Baghdad, Iraq
Data nadesłania: 07-03-2025
Data ostatniej rewizji: 10-06-2025
Data akceptacji: 27-06-2025
Data publikacji online: 04-12-2025
Autor do korespondencji
Farah Jaafar
Rheumatology Unit, Department of Medicine, College of Medicine, University of Mustansiriyah, Alkarkh St., postcode: 10021, Iraq
Reumatologia 2025;63(6):383-388
SŁOWA KLUCZOWE
DZIEDZINY
STRESZCZENIE
Introduction:
Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease with multiple internal organ involvement, vasculopathy, and fibrosis. Two major types are present, limited systemic sclerosis (lSSc) and diffuse systemic sclerosis (dSSc), according to the limit of skin fibrosis, with variability in internal organ involvement. Raynaud’s phenomenon (RP) is almost always present in either type as a presenting feature; it may precede the onset by years. It affects the quality of life for the patient and has a variable range of complications as well, with the most severe being tissue gangrene and finger amputation. The aim of the study was to investigate the prevalence of RP complications and predictors of outcome in lSSc and dSSc.
Material and methods:
Patients diagnosed with SSc were included in the study at 3 rheumatology centers in Iraq over a 3-year period. Data collection was conducted through questionnaires and interviews. All patients underwent clinical assessments to determine the presence or absence of RP complications, including pit scars, ulcers, ischemia, and amputated digits at the time of interview as well as the previous records. Subjects with concomitant autoimmune diseases were excluded from the study.
Results:
Of the 105 patients, 55 (52%) had dSSc, and 92% of those were female. The mean age was in the fourth decade. Digital complications were recorded. Ischemia was the most frequently
observed complication in limited scleroderma, while amputation was the least common. These complications exhibited a significant association with the duration of the disease, with the highest prevalence occurring within the first 10 years of diagnosis. Cardiac complications were associated with RP. Notably, 27% of patients with dSSc had hypertension, while 64% of patients with limited scleroderma did not have cardiac complications.
Conclusions:
Raynaud’s phenomenon is a defining characteristic of both limited and diffuse scleroderma. It is particularly complex in the diffuse form, underscoring the necessity for aggressive treatment to prevent debilitating complications. It is crucial to educate patients about the significance of adhering to treatment regimens and cessation of smoking.
REFERENCJE (26)
1.
Adigun R, Goyal A, Hariz A. Systemic sclerosis (scleroderma). In: StatPearls Publishing LLC, Treasure Island (FL) 2024.
2.
Maciejewska M, Sikora M, Maciejewski C, et al. Raynaud’s Phenomenon with focus on systemic sclerosis. J Clin Med 2022; 11: 2490, DOI: 10.3390/jcm11092490.
3.
Hakami LM, Forster GL, Jones MK, DeGeorge BRJ. Scleroderma and Raynaud phenomenon: the cold truth regarding the use of operative management. Plast Reconstr Surg 2022; 150: 105e–114e, DOI: 10.1097/PRS.0000000000009187.
4.
McMahan ZH, Wigley FM. Raynaud’s phenomenon and digital ischemia: a practical approach to risk stratification, diagnosis and management. Int J Clin Rheumtol 2010; 5: 355–370, DOI: 10.2217/ijr.10.17.
5.
van den Hoogen F, Khanna D, Fransen J, et al. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis 2013; 72: 1747–1755, DOI: 10.1136/annrheumdis-2013-204424.
6.
Pope JE, Denton CP, Johnson SR, et al. State-of-the-art evidence in the treatment of systemic sclerosis. Nat Rev Rheumatol 2023; 19: 212–226, DOI: 10.1038/s41584-023-00909-5.
7.
Poormoghim H, Andalib E, Jalali A, et al. Foot pain and lesions in systemic sclerosis: prevalence and association with organ involvement. J Rheum Dis Treat 2019; 5, DOI: 10.23937/2469-5726/1510076.
8.
Fernández-Codina A, Cañas-Ruano E, Pope JE. Management of Raynaud’s phenomenon in systemic sclerosis – a practical approach. J Scleroderma Relat Disord 2019; 4: 1020–1010, DOI: 10.1177/2397198318823951.
9.
Musa R, Qurie A. Raynaud Disease. In: StatPearls Publishing, Treasure Island (FL) 2025.
10.
Moinzadeh P, Nihtyanova SI, Howell K, et al. Impact of hallmark autoantibody reactivity on early diagnosis in scleroderma. Clin Rev Allergy Immunol 2012; 43: 249–255, DOI: 10.1007/s12016-012-8331-1.
11.
Nitsche A. Raynaud, digital ulcers and calcinosis in scleroderma. Reumatol Clin 2012; 8: 270–277, DOI: 10.1016/j.reuma.20212.02.006.
12.
Velásquez-Franco CJ, Rodríguez-Castrillón JC. Raynaud’s phenomenon, capillaroscopy, and digital ulcers as sentinel events in systemic sclerosis. Rev Colomb Reumatol 2020; 27: 170–174, DOI: 10.1016/j.rcreue.2020.07.001.
13.
Marvi U, Chung L. Digital ischemic loss in systemic sclerosis. Int J Rheumatol 2010; 2010: 130717, DOI: 10.1155/2010/130717.
14.
Hughes M, Allanore Y, Chung L, et al. Raynaud phenomenon and digital ulcers in systemic sclerosis. Nat Rev Rheumatol 2020; 16: 208–221, DOI: 10.1038/s41584-020-0386-4.
15.
Virgili-Gervais G, Matthews B, Nassar EL, et al. The association of outdoor temperature and self-reported Raynaud’s phenomenon severity among people with systemic sclerosis: a Scleroderma Patient-centered Intervention Network cohort study. Lancet Rheumatol 2024; 6: e684–e692, DOI: 10.1016/S2665-9913(24)00189-9.
16.
Pauling JD, Saketkoo LA, Matucci-Cerinic M, et al. The patient experience of Raynaud’s phenomenon in systemic sclerosis. Rheumatology 2019; 58: 18–26, DOI: 10.1093/rheumatology/key026.
17.
Williams AA, Carl HM, Lifchez SD. The Scleroderma hand: manifestations of disease and approach to management. J Hand Surg 2018; 43: 550–557, DOI: 10.1016/j.jhsa.2018.03.021.
18.
Foti R. Treatment with intravenous iloprost in patients with systemic sclerosis: a short review. J Rare Dis Res Treat 2017; 2: 6–13.
20.
Pope JE. The diagnosis and treatment of Raynaud’s phenomenon. Drugs 2007; 67: 517–525, DOI: 10.2165/00003495-200767040-00003.
21.
Silva C, Solanki KK, White DHN. The Relationship between smoking, Raynaud’s phenomenon, digital ulcers, and skin thickness in the Waikato Systemic Sclerosis Cohort. Rheumatol Immunol Res 2022; 3: 84–89, DOI: 10.2478/rir-2022-0014.
22.
Suter LG, Murabito JM, Felson DT, Fraenkel L. Smoking, alcohol consumption, and Raynaud’s phenomenon in middle age. Am J Med 2007; 120: 264–271, DOI: 10.1016/j.amjmed.2006.06.007.
23.
Chennakesavulu PV, Uppaluri S, Koyi J, et al. Pulmonary hypertension in scleroderma – evaluation and management. Dis Mon 2023; 69: 101468, DOI: 10.1016/j.disamonth.2022.101468.
24.
Chaisson NF, Hassoun PM. Systemic sclerosis-associated pulmonary arterial hypertension. Chest 2013; 144: 1346–1356, DOI: 10.1378/chest.12-2396.
25.
Launay D, Sobanski V, Hachulla E, Humbert M. Pulmonary hypertension in systemic sclerosis: different phenotypes. Eur Respir Rev 2017; 26: 170056, DOI: 10.1183/16000617.0056-2017.
26.
Morrisroe K, Huq M, Stevens W, et al. Risk factors for development of pulmonary arterial hypertension in Australian systemic sclerosis patients: results from a large multicenter cohort study. BMC Pulmonary Med 2016; 16: 134, DOI: 10.1186/s12890-016-0296-z.
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.