[The Diagnosis is Obvious]. / Die Diagnose liegt auf der Hand.

Clinically amyopathic dermatomyositis (CADM) is a rare entity of dermatomyositis. As a rule, CADM presents without muscular involvement. Thus, the level of creatine kinase is most commonly within the normal range. Dermal manifestations of CADM are Gottron's papules and mechanic's hands. In the case of melanoma differentiation-associated gene 5 intracellular pathogen sensor (MDa5 antibodies), CADM is often associated with a rapidly progressive and severe form of interstitial lung disease. Pulmonary function tests reveal restriction and hypoxemia of varying degree. Features of ground-glass opacities, reticulations and consolidations are detected in high-resolution CT scan. Lymphocytes are sometimes predominant in bronchioloalveolar lavage. Pathologists see a picture similar to non-specific interstitial lung disease or organizing pneumonia. Pronounced immunosuppression is the most common therapy. However, sometimes a combination of different immunosuppressive therapies is necessary. A novel strategy to treat CADM with rapidly progressive interstitial lung disease is the Janus kinase inhibitor tofacitinib. Treatment can be monitored with the level of ferritin and MDa5-antibody titer. Mortality is as high as 84 %.

[Crazy Paving Pattern of the Lung]. / Pflastersteine in der Lunge.

Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease. PAP results from impaired surfactant clearance. In adults, autoimmune pulmonary alveolar proteinosis is present in 90 - 95 % of the cases. In 5 - 10 %, other etiologies such as toxins and dust exposure, hematological disorders and infections have to be considered. Men between 30 - 60 years are commonly affected. Typical symptoms are cough, dyspnea and alteration in ventilatory function. CT scan of the lung is characterised by a crazy paving pattern. In serological testing, granulocyte macrophage colony-stimulation factor can be identified in most patients with autoimmune pulmonary alveolar proteinosis. Whole-lung lavage remains the therapy of choice. In the current case, treatment with whole-lung lavage resulted in clinical and functional improvement.

Whom are we treating with adaptive servo-ventilation? A clinical post hoc analysis.

BACKGROUND: Recent evidence has shown that adaptive servo-ventilation (ASV) is contraindicated in patients with predominant central sleep apnea (CSA) and reduced left ventricular ejection fraction (LVEF ≤45%). The objective of this study was to assess the clinical usage of ASV in patients at the time-point of the release of a safety warning by type of SDB, breathing pattern and LVEF. METHODS: Patients of a cardiac and a respirology sleep center, both in Germany, who received ASV therapy were contacted between May and October 2015. Retrospective analyses included diagnostic polysomnography, polysomnography with continuous positive airway pressure prior to ASV initiation and echocardiography. Treatment emergent CSA was diagnosed after an appropriate treatment period on CPAP. RESULTS: 285 patients receiving ASV therapy (91 in the cardiac and 194 in the respirology setting) underwent diagnostic polysomnography. 233 (82%) patients had severe SDB, 94 (33%) predominant CSA, and 185 (65%) periodic breathing. 20% (n = 52) of patients had an LVEF of ≤45%. The most common indications for ASV were CSA in heart failure (41%) in the cardiac setting and treatment emergent CSA (80%) diagnosed after an appropriate period on CPAP in the respirology setting. The proportion of patients in whom ASV was contraindicated (CSA and LVEF ≤45%) was 16% in the cardiac setting and 9% in the respirology setting. CONCLUSION: Clinical usage of ASV changed for a small subgroup of patients after release of the SERVE-HF results. Nevertheless, ASV treatment should be monitored and evaluated with diligence in the reminder indications.