Akciğer Tutulumu Olan Sistemik Skleroz Hastalarında Sınırlı Eklem Hareketliliğinin Değerlendirilmesi

Abstract

Giriş ve Amaç: Bozulmuş el fonksiyonu, sistemik skleroz (SSk) hastaların işlevselliği ile ilgili yaşam kalitesini bozar. Çalışmanın amacı, akciğer tutulumu olan SSk hastalarında elin sınırlı eklem hareketliliğini (LJM) değerlendirmektir. Gereç ve Yöntemler: 42 SSk hastası, LJM "dua işareti" testi kullanılarak değerlendirildi ve LJM pozitif ve LJM negatif hastaların demografik özellikleri (cinsiyet, yaş ve hastalık süresi), laboratuvar sonuçları (ESH, CRP, ANA, anti-topoizomeraz I ve anti-sentromer) ve modifiye Rodnan cilt skoru (mRss) sonuçları karşılaştırıldı. Bulgular: Toplam 42 (K=37, E=5) hasta (sınırlı kutanöz SSk (skSSk=12), diffüz kutanöz SSk (dkSSk=30)) bu çalışmaya dahil edildi ve %59,5'inde (skSSk =3, dkSSk =22) LJM pozitifliği saptanmıştır. LJM varlığı, skSSk ve dkSSk hastaları arasında istatistiksel olarak anlamlı bir farklılık gösterdi (p=0,006). LJM (+) hastaların mRss ortalaması LJM (-) hastalara göre anlamlı derecede yüksekti (p<0,001). Ayrıca, LJM (+) hastalarda DLCO oranları ve 6 dakika yürüme testi mesafeleri anlamlı derecede düşüktü (sırasıyla; p=0,021 ve p=0,004). Sonuç: Çalışmamızda, akciğer tutulumu olan SSk hastalarında LJM'nin mRss ile korele olduğu ve dkSSk hastaların daha yüksek LJM oranına sahip olduğu sonucuna vardık. "Dua işareti" testi günlük pratikte kolaylıkla uygulanabilir.

Keywords

• Sistemik skleroz
• dua işareti
• sınırlı eklem hareketliliği
• interstisyel akciğer hastalığı

Evaluation of Limited Joint Mobility in Systemic Sclerosis Patients with Pulmonary Involvement

Abstract

Aim: Systemic sclerosis (SSc) patients' functional quality of life is negatively impacted by impaired hand function. The aim of the study was to evaluate limited joint mobility (LJM) of the hand in SSc patients with lung involvement. Method: The research comprised 42 participants with SSc. LJM had an evaluation using the "prayer sign" exam. The demographic information (gender, age, and length of disease) as well as the laboratory results (ESR, CRP, anti-nuclear antibody (ANA), anti-topoisomerase I, and anti-centromer) and results of the modified Rodnan skin score (mRss) were compared between LJM (+) and LJM (-) patients. Results: This study comprised 42 (F=37, M=5) individuals with limited cutaneous SSc (lcSSc=12) and diffuse cutaneous SSc (dcSSc=30). Of them, 59.5% (lcSSc=3, dcSSc=22) tested positive for LJM. Depending on whether LJM was present, there was a statistically significant difference (p=0.006) between individuals with lcSSc and dcSSc. Patients with LJM+ had greater mean mRss (p<0.001). DLCO ratios and 6-minute walk test distances were significantly lower in LJM (+) patients (p=0.021 and p=0.004, respectively). Conclusion: In our study, we concluded that LJM correlated with mRss in SSc patients with lung involvement and that patients with dcSSc had a higher rate of LJM. The "prayer sign" test can be easily applied in daily practice.

Keywords

• Systemic sclerosis
• prayer sign
• limited joint mobility
• interstitial lung disease

References

1. Marie I, Jouen F, Hellot MF, Levesque H. Anticardiolipin and anti-beta2 glycoprotein I antibodies and lupus-like anticoagulant: prevalence and significance in systemic sclerosis. Br J Dermatol. 2008;158(1):141-144.
2. 2. Thombs BD, Taillefer SS, Hudson M, Baron M. Depression in patients with systemic sclerosis: a systematic review of the evidence. Arthritis Rheum. 2007;57(6):1089-1097.
3. 3. Tyndall AJ, Bannert B, Vonk M, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010;69(10):1809-1815.
4. 4. Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis. 2007;66(7):940-944.
5. 5. Perelas A, Silver RM, Arrossi AV, Highland KB. Systemic sclerosis-associated interstitial lung disease. Lancet Respir Med. 2020;8(3):304-320.
6. 6. Steen VD, Medsger TA Jr. Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum. 2000;43(11):2437-2444.
7. 7. Ingegnoli F, Boracchi P, Ambrogi F, Gualtierotti R, Galbiati V, Meroni PL. Hand impairment in systemic sclerosis: association of different hand indices with organ involvement. Scand J Rheumatol. 2010;39(5):393-397. 8. Skare TL, Toebe BL, Boros C. Hand dysfunction in scleroderma patients. Sao Paulo Med J. 2011;129(5):357-360.
8. 9. Uslu S, Gülle S, Can G, Sarı İ, Önen F, Birlik M. Evaluation of limited hand mobility in systemic sclerosis patients by using "prayer sign" and "tabletop sign". Clin Rheumatol. 2021;40(7):2771-2777.

9. 10. Pardos-Gea J. Positive prayer sign in eosinophilic fasciitis. Rheumatology (Oxford). 2017;56(4):628.
10. 11. Uslu S. Positive Prayer Sign in Tophaceous Gout. Eur J Rheumatol. 2023;10(4):179.
11. 12. 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. Arthritis Rheum. 2013;65(11):2737-2747.
12. 13. LeRoy EC, Black C, Fleischmajer R, et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988;15(2):202-205.
13. 14. Low AHL, Ng SA, Berrocal V, et al. Evaluation of Scleroderma Clinical Trials Consortium training recommendations on modified Rodnan skin score assessment in scleroderma. Int J Rheum Dis. 2019;22(6):1036-1040. doi:10.1111/1756-185X.13523
14. 15. Denton CP, Khanna D. Systemic sclerosis. Lancet. 2017;390(10103):1685-1699. doi:10.1016/S0140-6736(17)30933-9
15. 16. Avouac J, Walker U, Tyndall A, et al. Characteristics of joint involvement and relationships with systemic inflammation in systemic sclerosis: results from the EULAR Scleroderma Trial and Research Group (EUSTAR) database. J Rheumatol. 2010;37(7):1488-1501.
16. 17. Avouac J, Clements PJ, Khanna D, Furst DE, Allanore Y. Articular involvement in systemic sclerosis. Rheumatology (Oxford). 2012;51(8):1347-1356.
17. 18. Poole JL, Watzlaf VJ, D'amico F. A five-year followup of hand function and activities of daily living in systemic sclerosis (scleroderma). J Hand Ther. 2004;17(4):407-411.
18. 19. Arkkila PE, Gautier JF. Musculoskeletal disorders in diabetes mellitus: an update. Best Pract Res Clin Rheumatol. 2003;17(6):945-970.
19. 20. Avouac J, Guerini H, Wipff J, et al. Radiological hand involvement in systemic sclerosis. Ann Rheum Dis. 2006;65(8):1088-1092.
20. 21. Bálint Z, Farkas H, Farkas N, et al. A three-year follow-up study of the development of joint contractures in 131 patients with systemic sclerosis. Clin Exp Rheumatol. 2014;32(6 Suppl 86):.
21. 22. Ashida R, Ihn H, Mimura Y, et al. Clinical features of scleroderma patients with contracture of phalanges. Clin Rheumatol. 2007;26(8):1275-1277.
22. 23. Eberhardt K, Sandqvist G, Geborek P. Hand function tests are important and sensitive tools for assessment of treatment response in patients with rheumatoid arthritis. Scand J Rheumatol. 2008;37(2):109-112.
23. 24. Merkel PA, Clements PJ, Reveille JD, et al. Current status of outcome measure development for clinical trials in systemic sclerosis. Report from OMERACT 6. J Rheumatol. 2003;30(7):1630-1647.
24. 25 Bouros D, Wells AU, Nicholson AG, et al. Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med. 2002;165(12):1581-1586.
25. 26. Jung E, Suh CH, Kim HA, Jung JY. Clinical Characteristics of Systemic Sclerosis With Interstitial Lung Disease. Arch Rheumatol. 2018;33(3):322-327