Background: Systemic sclerosis (SSc) treatment landscape has seen novel developments in the last decade in lung and skin domains of early disease. However, disease heterogeneity and fluctuating health infrastructure demand regional adaptation of high-quality trial data. We conducted a survey among Rheumatologists in India to assess the prevalent trends in managing systemic sclerosis. Objectives: •To explore the difference in trends in the assessment of Systemic sclerosis •To evaluate real-world trends in the treatment of different organ manifestations of SSc among rheumatologists in India. Methods: A questionnaire was designed by members of Indian Systemic Sclerosis Special Interest Group including a trial run, to assess the following aspects of SSc: •Disease trends- The frequency of organ-specific clinical features and the level of difficulty in treatment. •Assessment trends- The patterns of evaluation for organ involvement •Prescription trends- The practice pattern in the treatment of major SSc domains The survey was conducted in a bimodal manner. The Rheumatologists who attended a recent three-day national conference were invited to complete the questionnaire. Other practicing rheumatologists, including trainees, were contacted electronically and through local rheumatology networks to participate in the survey. Results: The participants in the survey were 210 (of 400 invited), with a response rate of 52.5%. Among them 30.5%(n=64) were trainees. Corporate hospitals were the most common practice setting (67.8%, n=129/190), 48% of responders worked in teaching hospitals. The most common source of patient referral was other specialists(n=186,97.4%), followed by self-referral (n=64,48.8%). A mean of 6 new patients per month were seen, with an annual follow-up of 68 patients per participant. The number of responses ranged from 195-206. The heterogeneity of SSc features encountered is summarized in Figure 1a. Puffy hands, arrhythmias, SIBO and constipation emerged as rare features. Calcinosis was the most difficult to treat(n=132/197,67%). Rectal incontinence was least targeted by participants (n=70/191, 36.6%). In disease assessment (Figure 1b), 55.7% agreed to recording mRSS; patient self-reported new areas of skin tightening (10%, n=21) and new skin symptoms (3.8%, n=8) were considered as worsening disease by those who did not perform mRSS. Utilization of NFC and endoscopy were less among the participants. Use of lung imaging, PFT and 6MWT were prevalent. 2DECHO with a focus on PASP and TAPSE was the most used for evaluation of pulmonary hypertension while Right heart catheterization and biomarkers were the least ordered. The treatment trends showed use of glucocorticoids by 82%; with majority prescribing less than 10 mg/day prednisolone equivalent (n=139, 66.1%) for 3 months or less (n=48, 22.8%) (Figure 2a). Most common indications for steroids were myositis (n=148, 34%), arthritis (n=141, 32.4%) and ILD (n=110, 25.3%). Early skin involvement, without other internal organ involvement was treated with immunosuppression (IS) by majority (n=179, 85.2%,) which was switched to another IS (n=149, 70.9%,) in the absence of improvement. Methotrexate (n=100, 47.6%) was preferred for skin tightening, with switching to rituximab (n=44, 20.9%). For ILD treatment, majority (99%) preferred Immunosuppression over antifibrotics, with 28.8% using it as add-on, preferably nintedanib, if ILD progresses. Mycophenolate was the most favored drug for ILD (62.6%, n=151 responses). In RP without gangrene, CCB with PDE5I addition upon worsening was the preferred strategy. For non-digital ulcers, steroids/DMARDs were most prescribed; an upfront combination of ERA+PDE5I was favored for PH by the majority. Pre-IS vaccination was favored by 90%. Conclusion: This nationwide survey to explore scleroderma practice trends among rheumatologists sheds light into domain-specific assessment and treatment preferences. Heterogeneity in disease encounter and practice patterns can significantly affect multicenter clinical trials and usage of outcome measures. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests: None declared.
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Shobha et al. (2024) studied this question.
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