Background: The major reasons to treat sarcoidosis are to lower the morbidity and mortality risk or to improve quality of life (QoL). The indication for treatment varies depending on which manifestation is the cause of symptoms: lungs, heart, brain, skin or other manifestations. While glucocorticoids remain the first choice for initial treatment of symptomatic disease, prolonged use is associated with significant toxicity. Glucocorticoid-sparing alternatives are available. The presented treatment guidelines aim to provide guidance to physicians treating the very heterogenous sarcoidosis manifestations. Methods: A European Respiratory Society Task Force committee composed of clinicians, methodologists and patients with experience in sarcoidosis developed recommendations based on the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) methodology. The committee developed eight PICO (Patients, Intervention, Comparison, Outcomes) questions and these were used to make specific evidence-based recommendations. Results: The Task Force committee delivered 12 recommendations for seven PICOs. These included treatment of pulmonary, cutaneous, cardiac and neurologic disease as well as fatigue. One PICO question regarding small-fibre neuropathy had insufficient evidence to support a recommendation. In addition to the recommendations, the committee provided information on how they use alternative treatments, when there was insufficient evidence to support a recommendation. Conclusions: There are many treatments available to treat sarcoidosis. Given the diverse nature of the disease, treatment decisions require an assessment of organ involvement, risk for significant morbidity, and impact on QoL of the disease and treatment.

Several months into the COVID-19 pandemic, safe and effective treatments against this global health disaster have yet to be identified. Clinical research trials around the world are underway testin...

Pulmonary involvement is the leading cause of death in systemic sclerosis (SSc) and may manifest as interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), or in combination of both (ILD-pulmonary hypertension (PH)). Aim of this analysis was to determine prevalence, clinical characteristics, and survival of these different forms within the registry of the German Network for Systemic Sclerosis (DNSS). Does SSc-associated ILD with or without PH affect survival differently and are there any risk factors that have an additional impact? Clinical data of 5,831 SSc patients were collected in the DNSS registry, Kaplan-Meier estimates were used to compare overall survival (OS) in patients with SSc-ILD with PH (ILD-PH) and without PH (ILD-w/o-PH) to patients without pulmonary involvement and those with PAH. The Cox proportional hazard model was used to analyze the influence of pulmonary involvement and other potential predictors on patient survival. Clinical data of 3,257 patients with a mean follow-up years of 3.45±1.63 have been included in our analysis. At baseline, ILD was present in 34.5%, while PH (w/o-ILD) had a lower prevalence with 4.5%. At the end of follow-up, 47.6% of SSc patients had ILD, 15.2% ILD-PH, and 6.5% PAH. ILD was more frequent in the diffuse cutaneous form (57.3%), while PAH did not differ significantly between SSc subtypes. Significant differences in baseline characteristics between PAH vs. ILD-PH vs. ILD-w/o-PH were found for age at diagnosis, gender, SSc-subsets, antibody-status, FVC, DLCO and therapy. OS at 5 years was 96.4% for patients without pulmonary involvement and differed significantly between patients with ILD-w/o-PH, PAH, being worst in patients with ILD-PH. Female gender (HR 0.3), a higher BMI (HR 0.9) and higher DLCO values (HR 0.98) were associated with a lower mortality risk. ILD is the most prevalent pulmonary involvement in SSc, while the combination of ILD and PH is associated with the most detrimental survival.