Background: Chronic pain is a healthcare problem that significantly affects the physical and mental health of patients and impact their quality of life. It can complicate many disorders and represents a common symptom of rheumatologic diseases. Patients with Scleroderma report pulmonary and gastrointestinal symptoms as the limiting factor on their daily functioning, however pain remains the main challenging and ever-present problem in Scleroderma. Although Scleroderma-related pain has been studied and are considered a symptom caused by the disease activity, this symptom in Scleroderma has not yet been investigated in detail and data on the prevalence and management of pain in patients with Scleroderma is lacking. The goal of our study was to 1. evaluate the prevalence of pain, skin involvement, vascular, pulmonary, and gastrointestinal systems and 2. assess the efficacy of the symptomatic treatments used to palliate these symptoms among patients with Scleroderma in the outpatient rheumatology clinic of a tertiary care center.

Methods: Clinical data from January 2014 to January 2016 from outpatient rheumatology clinic of a tertiary care hospital (Mount Sinai Hospital) was reviewed. Cases were included in our study if they had documented diagnosis of Scleroderma by a rheumatologist and seen for at least 2 visits. 29 cases were reviewed for presence of pain, and involvement of skin, vascular, pulmonary, and gastrointestinal systems were extracted for each patient in the study.

Results: Twenty-nine cases were reviewed in detail. 55% of patient were diagnosed with diffuse and 45% with limited scleroderma. Most were women (86%), average age at diagnosis was 50 (range 21-77 years), and they had been followed at the clinic on average for 4.2 years. Seventy six percent of patients reported having some degree of pain (mild, moderate and severe); 28 patients had documented skin symptoms including digital ulcers, itching and infections; 24 had developed Raynaud’s phenomenon, 19 pulmonary symptoms, and all 29 patients had documented gastrointestinal symptoms. Of the patients with Raynaud’s phenomenon 46% were on CCBs (Calcium Channel Blockers). All patients with gastrointestinal involvement were on Proton Pump Inhibitors, however 55% continued to have gastrointestinal symptoms including nausea, poor appetite, dysphagia, odynophagia, abdominal pain, and/or abdominal bloating. Eleven patients were found to have Interstitial Lung Disease, and ten of them despite being on Disease Modifying Antirheumatic Drugs (DMARD) therapy were reporting varying degree of shortness of breath. Seven patients reported depressive symptoms, however none of them were on anti-depressants at the time of visits.

Conclusions: This retrospective chart review suggests that patients with Scleroderma who are under care of a multidisciplinary team consisting of expert rheumatologist, pulmonary, and gastrointestinal specialists at our tertiary center continue to have disturbing symptoms although on symptomatic treatment for many of these symptoms. We suggest frequent assessment of symptoms and quality of life and escalation of symptomatic treatment for pain and disturbing symptoms. Although palliative care teams are trained to address persistent pain and the resultant psychosocial stressors, more studies are needed to understand the best management for these symptoms in patients with Scleroderma and the need to involve palliative care as part of the multidisciplinary approach of this serious illness.