Swallowing difficulties with medication intake assessed with a novel self-report questionnaire in patients with systemic sclerosis – a cross-sectional population study

Markus Messerli,1,2 Rebecca Aschwanden,1 Michael Buslau,2 Kurt E Hersberger,1 Isabelle Arnet1 1Pharmaceutical Care Research Group, Department of Pharmaceutical Sciences, University of Basel, Basel, Switzerland; 2European Centre for the Rehabilitation of Scleroderma, Reha Rheinfelden, Rheinfelden, Switzerland Objectives: To assess subjective swallowing difficulties (SD) with medication intake and their practical consequences in patients suffering from systemic sclerosis (SSc) with a novel self-report questionnaire.Design and setting: Based on a systematic literature review, we developed a self-report questionnaire and got it approved by an expert panel. Subsequently, we sent the questionnaire by post mail to SSc patients of the European Center for the Rehabilitation of Scleroderma Rheinfelden, Switzerland.Participants: Patients were eligible if they were diagnosed with SSc, treated at the center, and were of age ≥18 years at the study start.Main outcome measures: Prevalence and pattern of SD with oral medication intake, including localization and intensity of complaints.Results: The questionnaire consisted of 30 items divided into five sections Complaints, Intensity, Localization, Coping strategies, and Adherence. Of the 64 SSc patients eligible in 2014, 43 (67%) returned the questionnaire. Twenty patients reported SD with medication intake (prevalence 47%), either currently (11; 26%) or in the past that had been overcome (9; 21%). Self-reported SD were localized mostly in the larynx (43%) and esophagus (34%). They were of moderate (45%) or strong to unbearable intensity (25%). Modification of the dosage form was reported in 40% of cases with SD. Adherence was poor for 20 (47%) patients and was not associated with SD (p=0.148).Conclusion: Our novel self-report questionnaire is able to assess the pattern of complaints linked to medication intake, that is, localization and intensity. It may serve as a guide for health care professionals in selecting the most suitable therapy option, enabling tailored counseling to reduce inappropriate medication modifications. Keywords: swallowing difficulties, medication intake, systemic sclerosis, coping behavior, self-report questionnaire, deglutition disorder

Vasoactive Therapy in Systemic Sclerosis: Real-life Therapeutic Practice in More Than 3000 Patients

Objective: Vasculopathy is a key factor in the pathophysiology of systemic sclerosis (SSc) and the main cause for Raynaud phenomenon (RP), digital ulcers (DU), and/or pulmonary arterial hypertension (PAH). It is so far unknown how patients with SSc are treated with vasoactive agents in daily practice. To determine to which extent patients with SSc were treated with different vasoactive agents, we used data from the German Network for Systemic Scleroderma registry. Methods: The data of 3248 patients with SSc were analyzed. Results: Patients were treated with vasoactive drugs in 61.1% of cases (1984/3248). Of these, 47.6% received calcium channel inhibitors, followed by 34.2% treated with angiotensin-converting enzyme (ACE) inhibitors, 21.1% treated with intravenous (IV) prostanoids, 10.1% with pentoxifylline, 8.8% with angiotensin 1 receptor antagonists (AT1RA), 8.7% with endothelin 1 receptor antagonists (ET1RA), 4.1% with phosphodiesterase type 5 (PDE5) inhibitors, and 5.3% with others. Patients with RP received vasoactive therapy in 63.3% of cases, with DU in 70.1%, and with PAH in 78.2% of cases. Logistic regression analysis revealed that patients with PAH were significantly more often treated with PDE5 inhibitors and ET1RA, and those with DU with ET1RA and IV prostanoids. In addition, 41.8% of patients were treated with ACE inhibitors and/or AT1RA. Patients registered after 2009 received significantly more often ET1RA, AT1RA, and IV prostanoids compared with patients registered prior to 2005. Conclusion: These data clearly indicate that many patients with SSc do not yet receive sufficient vasoactive therapy. Further, in recent years, a marked change of treatment regimens can be observed