[CF Lung Disease - a German S3 Guideline: Pseudomonas aeruginosa]. / S3-Leitlinie: Lungenerkrankung bei Mukoviszidose ­ Pseudomonas aeruginosa.

Cystic Fibrosis (CF) is the most common autosomal recessive genetic multisystemic disease. In Germany, it affects at least 8000 people. The disease is caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene leading to dysfunction of CFTR, a transmembrane chloride channel. This defect causes insufficient hydration of the airway epithelial lining fluid which leads to reduction of the mucociliary clearance.Even if highly effective, CFTR modulator therapy has been available for some years and people with CF are getting much older than before, recurrent and chronic infections of the airways as well as pulmonary exacerbations still occur. In adult CF life, Pseudomonas aeruginosa (PA) is the most relevant pathogen in colonisation and chronic infection of the lung, leading to further loss of lung function. There are many possibilities to treat PA-infection.This is a S3-clinical guideline which implements a definition for chronic PA-infection and demonstrates evidence-based diagnostic methods and medical treatment in order to give guidance for individual treatment options.

Pain chronification and the important role of non-disease-specific symptoms in patients with systemic sclerosis.

BACKGROUND: Pain is a frequent, yet inadequately explored challenge in patients with systemic sclerosis (SSc). This study aimed to conduct an extensive pain assessment, examining pain chronification and its association with disease manifestations. METHODS: Consecutive SSc patients attending their annual assessment were included. SSc-specific features were addressed as defined by the European Scleroderma Trials and Research (EUSTAR) guidelines. Pain analysis included intensity, localization, treatment, chronification grade according to the Mainz Pain Staging System (MPSS), general well-being using the Marburg questionnaire on habitual health findings (MFHW) and symptoms of anxiety and depression using the Hospital Anxiety and Depression Scale (HADS). RESULTS: One hundred forty-seven SSc patients completed a pain questionnaire, and 118/147 patients reporting pain were included in the analysis. Median pain intensity was 4/10 on a numeric rating scale (NRS). The most frequent major pain localizations were hand and lower back. Low back pain as the main pain manifestation was significantly more frequent in patients with very early SSc (p = 0.01); those patients also showed worse HADS and MFHW scores. Regarding pain chronification, 34.8% were in stage I according to the MPSS, 45.2% in stage II and 20.0% in stage III. There was no significant correlation between chronification grade and disease severity, but advanced chronification was significantly more frequent in patients with low back pain (p = 0.024). It was also significantly associated with pathological HADS scores (p < 0.0001) and linked with decreased well-being and higher use of analgesics. CONCLUSIONS: Our study implies that also non-disease-specific symptoms such as low back pain need to be considered in SSc patients, especially in early disease. Since low back pain seems to be associated with higher grades of pain chronification and psychological problems, our study underlines the importance of preventing pain chronification in order to enhance the quality of life.