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Sarcoidosis is a granulomatous systemic disease of unknown etiology most commonly affecting the lungs and thoracic lymph nodes. The diagnosis is based on typical clinical radiologic appearance and histology with evidence of noncaseating epithelioid cell granulomas without central necrosis. In the acute form, Löfgren's syndrome, histologic confirmation may not be necessary. Approximately half of patients may develop a chronic form, and extrathoracic organ involvement should be investigated during the course. Indications for therapy are based on functional limitations, marked organ-related or systemic symptoms, and life-threatening organ manifestations (cardiac, central nervous system, renal, and ocular sarcoidosis). To date, there is no approved drug therapy for sarcoidosis. Administration of immunosuppressants such as glucocorticosteroids and as add-on or sequential, methotrexate, azathioprine or mycophenolate mofetil is recommended in the currently published international guideline.

IL-9, mainly produced by T helper 9 (Th9) cells, promotes allergic airway inflammation and remodeling through the interaction with its receptor (IL-9R). Th9 cells and IL-9 have also been implicated in tissue fibrosis and autoimmunity pathways. However, the role of IL-9/IL-9R in the pathogenesis of interstitial lung disease (ILD) is unknown.To evaluate IL-9/IL-9R expression in bronchoalveolar lavage fluid (BALF) lymphocytes of patients with various ILDs.Consecutive patients with ILD, who underwent BAL for diagnostic purposes, were studied. As control group, consecutive patients without evidence of ILD were included. Immunocytochemical staining of BALF lymphocytes for IL-9 and IL-9R was performed and evaluated by two independent readers.45 patients, of them 8 had idiopathic pulmonary fibrosis (IPF), 12 nonspecific interstitial pneumonia (NSIP), 10 sarcoidosis, 9 hypersensitivity pneumonitis (HP), 6 cryptogenic organizing pneumonia (COP), and 24 controls were studied. In the ILD group, the highest BALF lymphocyte count was seen in HP followed by NSIP, COP, sarcoidosis, and IPF (p 0.05 for HP vs IPF). The highest percentages of IL-9 and IL-9R positive lymphocytes were seen in COP. Conversely, NSIP showed the lowest rate of IL-9, and sarcoidosis the lowest rate of IL-9R positive lymphocytes. Only in NSIP, a direct correlation between IL and 9 and IL-9R positive lymphocytes was seen (r = 0.639, p = 0.025).BALF lymphocytes IL-9 and IL-9R expression differs between various ILDs and could reflect different pathogenetic mechanisms.

Eine 46 Jahre alte Patientin stellte sich mit dem Verdacht auf eine interstitielle Lungenerkrankung (externe Verdachtsdiagnose exogen-allergische Alveolitis) vor. Sie klagte über Belastungsluftnot nach Steigen von einer Etage Treppen. Die Patientin war seit 1 Jahr Exraucherin mit einem geschätzten Tabakkonsum von > 30 Packungsjahren („pack years“). Bei der gezielten Anamnese auf eine mögliche Allergenexposition war möglicherweise Schimmel im Keller vorhanden, außerdem war vor 3 Jahren eine Vogelvoliere entfernt worden. Die medizinische Vorgeschichte war ansonsten im Wesentlichen unauffällig, die Patientin war überwiegend als Hausfrau tätig gewesen.

Poster-Abstract

In den letzten Jahren haben sich die Klassifikationen der interstitiellen Lungenerkrankungen (ILD) und die Therapie der idiopathischen Lungenfibrose (IPF) grundlegend gewandelt. Da sich die Therapie der IPF von der anderer ILDs abgrenzt, ist es umso wichtiger, andere fibrosierende ILDs richtig zu diagnostizieren. Die Bezeichnung „Interstitial pneumonia with autoimmune features“ (IPAF) und deren Klassifikationskriterien wurden 2015 von einer ERS/ATS-Task-Force im Rahmen einer Konsensusfindung vorgeschlagen, um Patienten mit interstitiellen Pneumonien und Eigenschaften einer Autoimmunerkrankung zu beschreiben, die nicht die definitiven Kriterien einer Kollagenose erfullen. Die Therapie ist aktuell weiterhin in erster Linie immunsuppressiv; prospektive Daten hierzu fehlen jedoch.

Anti-[programmed cell death protein 1 (PD-1)] antibodies nivolumab and pembrolizumab were approved for adjuvant treatment of melanoma as they demonstrated improved relapse-free survival. Currently, combined anti-PD-1 plus anti-[cytotoxic T-lymphocyte-associated protein 4 (CTLA4)] blockade is being investigated in adjuvant and neoadjuvant trials. Sarcoidosis-like reactions have been described for immune checkpoint inhibitors and are most likely drug-induced. The reported rate of sarcoidosis/sarcoidosis-like reactions within clinical melanoma trials is2%. We observed that a remarkably higher number of melanoma patients (10/45 patients, 22%) treated with immune checkpoint inhibitor (ICI) within an adjuvant clinical trial-developed drug induced sarcoidosis-like reaction (DISR) mimicking metastasis.Of 45 stage III melanoma patients who were treated at our institute with adjuvant ICI (either nivolumab alone or in combination with ipilimumab) within a two-armed, blinded clinical trial, ten developed a DISR. Three of the ten patients were men, median age was 52 years (range, 32-70 years). DISRs were asymptomatic and generally detected radiographically at first radiographic imaging after the start of therapy (median time, 2.8 months) and described as a differential diagnosis to tumour progression. In one patient, DISR was only apparent 13.1 months after start of therapy and 4 weeks after the end of ICI treatment. DISR presented as mediastinal/hilar lymphadenopathy in 8/10 patients (as only site or in addition to lung, skin and/or bone involvement), one patient had only lung and cutaneous, one patient only cutaneous DISR. Biopsies from lymph nodes, skin and bone were taken in 8/10 patients, and histology confirmed sarcoidosis-like reactions (SLRs). As patients were asymptomatic, no treatment for DISR was required, and study treatment was stopped for DISR in only one patient due to bone involvement. DISRs have resolved or are in remission in all patients. At a median follow-up time of 15.3 months (range, 12-17.6 months), two patients experienced melanoma relapse.In most cases, sarcoidosis could only be differentiated from melanoma progression on biopsy. Treating physicians as well as radiologists have to be aware of the potentially higher rate of DISR in patients receiving adjuvant ICI. A thorough interdisciplinary workup is required to discriminate from true melanoma progression and to decide on continuation of adjuvant ICI treatment.

ZusammenfassungDie Sarkoidose ist eine Systemerkrankung unbekannter Ätiologie, die histologisch durch epitheloidzellige Granulome charakterisiert ist und sich immunpathologisch durch eine gesteigerte zelluläre Immunantwort vom Th1-Typ in den betroffenen Organen auszeichnet. Die Krankheit kommt weltweit vor, in nördlichen Breiten häufiger als im Süden. In Deutschland liegt die Inzidenz bei 10 pro 100 000 und die Prävalenz bei 44–48 pro 100 000. Die Erkrankung tritt meistens vor dem 50. Lebensjahr auf, kann aber prinzipiell in jedem Lebensalter, auch bei Kindern und Jugendlichen sowie im höheren Lebensalter in Erscheinung treten. Der Erkrankungsgipfel liegt zwischen 20 und 39 Jahren, Frauen sind in den meisten ethnischen Gruppen häufiger betroffen als Männer. Eine familiäre Häufung ist bekannt. Das klinische Erscheinungsbild bei Sarkoidose ist sehr vielfältig und von den einzelnen Organmanifestationen abhängig. Allgemeinsymptome sind Müdigkeit, Nachtschweiß, Gewichtsverlust, seltener auch Fieber, sowie Arthralgien und Myalgien. Organspezifische Symptome sind z. B. Husten und Luftnot beim Lungenbefall, Kopfschmerzen sowie Nervenlähmungen bei Neurosarkoidose, Herzrhythmusstörungen und Herzinsuffizienz bei kardialer Sarkoidose, vielgestaltige Hauterscheinungen bei Hautmanifestation, eine einseitige oder beidseitige Parotisschwellung bei Beteiligung der Speicheldrüsen. Man unterscheidet 2 Manifestationsformen: Eine akute, selten rezidivierende Form und eine chronische, häufiger rezidivierende Erkrankung. Das Löfgren-Syndrom, als Sonderform der akuten Sarkoidose, ist durch die Trias aus bihilärer Lymphadenopathie, Sprunggelenksarthritis und Erythema nodosum gekennzeichnet. Die chronische Sarkoidose tritt bei der Mehrzahl der Betroffenen auf, sie kann zunächst symptomarm verlaufen, trotz z. T. ausgeprägter Röntgenbildveränderungen. Man spricht von chronischer Sarkoidose, wenn nach einer Krankheitsdauer von über 2 Jahren die Krankheit noch aktiv ist. Die Prognose ist mit einer hohen Rate an Spontanremissionen insgesamt gut und hängt von den Organkomplikationen ab.

Background: Nailfold capillary microscopy (NC) changes can be predictive of connective tissue disease (CTD), especially if associated with ANA positivity and Raynaud phenomenon. Interstitial lung disease (ILD) can be the first manifestation of CTD, before specific clinical signs or ANA positivity become evident. There are limited data on NC changes in patients with ILD as yet. Aim: to investigate NC changes in patients with ILD and no known CTD, and to correlate with ANA status and clinical variables. Patients and methods: 74 patients (27 F, 46 M) with interstitial lung disease (ILD) at first diagnosis were studied. ANA, detected by IIF on Hep2 cells, were considered positive at a titer >160. NC was performed by using a light microscope (Di-Li 2100, Distelkamp Elektronic, Germany). As comparison groups 116 patients with systemic lupus erythematosus (SLE) and 754 controls with normal pattern (NP) were included. Results: Among ILD patients, the most common diagnoses, confirmed by the ILD board, were IPF (32), idiopathic NSIP (19), IPAF (10) and COP (4). Pathological NC findings were seen in 30 (40%) ILD patients, 13 of them (43%) had NSIP pattern at HRCT. Frequency and intensity of NC changes in ILD patients were not associated with ANA status. Like SLE patients, ILD patients had more frequently impaired capillary filling and hemorrhage compared to NP controls. Conclusion: Patients with ILD seem to have NC changes with a similar morphology to SLE patients. NC alterations appear to be independent from ANA status and slightly more prevalent in NSIP patients.

Background: Anti-DFS70 antibody, corresponding to the dense fine ANA speckled pattern at indirect immunofluorescence (IIF) in HEp-2 substrates, have been detected in a variety of chronic inflammatory conditions, cancer and in healthy individuals, but only in a small percentage of systemic ANA- associated rheumatic disease (SARD). In a previous study we found that anti-DFS70 positivity can be used to largely exclude SARD in patients with ILD and ANA positivity (IPAF). Aim of this study: To investigate whether serum anti-DFS70 levels correlate with clinical parameters in ILD. Patients and Methods: 260 patients with ILD (100 NSIP and 160 IPF), 49 healthy controls (HC) and 35 scleroderma-ILD (SSc-ILD) patients as negative control were studied. Serum anti-DFS70 at baseline was measured by ELISA (MBL Co., Ltd., Japan). Results: During 24 months of follow-up, 37 ILD patients (34 NSIP and 3 IPF) developed connective tissue disease (CTD). Anti-DFS70 levels in the ILD group were significantly lower than in HC (119 vs 320 U/ml, p In patients with idiopathic NSIP (iNSIP) anti-DFS70 titers did not differ from those of IPF patients or CTD-NSIP (119 vs 123 vs 101 U/ml, respectively). In iNSIP patients, anti-DFS70 titers inversely correlated with FVC (%pred.) (r=-0.320, p Conclusions: iNSIP patients with higher anti-DFS70 levels showed more impairment of lung function and gas exchange than CTD-NSIP.

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) was recognized as a rare new entity. We report the case of a 63 years old female suffering from progressive dyspnea and dry cough for three years. Two years before admission to our hospital, idiopathic pulmonary fibrosis (IPF) was diagnosed in another hospital and treatment with prednisolone and N-acetylcysteine (NAC) was commenced. At admission HRCT showed upper lobe dominant fibrosis and associated pleural thickening. Surgical biopsies were re-evaluated and revealed fibroelastosis with pleural thickening and a probable UIP pattern, consistent with idiopathic PPFE. Treatment with pirfenidone was initiated due to progression under prednisolone and NAC. Upper lobe predominant pleural thickening with associated subpleural fibrotic changes should raise suspicion of PPFE. OA gold

There is increasing evidence that sleep disorders are associated with cognitive decline. We, therefore, examined the cross-sectional association of sleep-disordered breathing (SDB), sleep quality, and three types of sleep complaints (difficulties initiating sleep, difficulties maintaining sleep, and early morning awakening) with mild cognitive impairment (MCI) and its subtypes. A group of 1,793 participants (51% men; 63.8 ± 7.5 years) of the population-based Heinz Nixdorf Recall study (total sample n = 4,157) received a screening for SDB and self-report measures of sleep complaints. Group comparisons were used to compare performances among five cognitive subtests. Multivariate logistic regression models were calculated to determine the association of MCI (n = 230) and MCI subtypes (amnestic MCI, n = 120; non-amnestic MCI, n = 110) with SDB severity levels, poor sleep quality, and sleep complaints. Severe SDB (apnea-hypopnea index ≥30/h, n = 143) was not associated with MCI, amnestic MCI, or non-amnestic MCI. Poor sleep quality was associated with MCI (Odds ratio (OR) = 1.40, 95% confidence interval (CI) = 1.02-2.03; fully adjusted) as well as frequently reported difficulties initiating sleep (OR = 1.94, 1.20-3.14), difficulties maintaining sleep (OR = 2.23, 1.27-4.63), and early morning awakening (OR = 2.30, 1.32-4.00). Severe difficulties initiating sleep (OR = 2.23, 1.21-4.13) and early morning awakening (OR = 2.88, 1.45-5.73) were solely associated with the amnestic MCI subtype, whereas, severe difficulties maintaining sleep (OR = 3.84, 1.13-13.08) were associated with non-amnestic MCI. Our results suggest that poor sleep quality, rather than SDB, is associated with MCI. The selective association of difficulties initiating sleep and early morning awakening with amnestic MCI and of difficulties maintaining sleep with non-amnestic MCI might serve as a marker to improve diagnostic accuracy in the earliest stages of cognitive impairment and will be further investigated in our longitudinal examination.

Hintergrund und Fragestellung: Die idiopathische Lungenfibrose (IPF) ist eine seltene, chronisch-progrediente Erkrankung mit einer mittleren Lebenserwartung nach Diagnosestellung von nur 3 Jahren. Pirfenidon ist bislang das einzige Medikament, das in der Europaischen Union zur Behandlung der leichten bis mittelschweren IPF bei Erwachsenen zugelassen ist. Ziel unserer Untersuchung war es, bisher erhobene Daten zur Sicherheit und Vertraglichkeit sowie zur Wirksamkeit von Pirfenidon in der Behandlung von IPF-Patienten in unserem Zentrum zu analysieren. Patienten und Methodik: Zwischen 2006 und 2012 wurden 45 Patienten (28 in Studien, 17 auserhalb) mit leichter bis mittelschwerer IPF mit Pirfenidon behandelt. Klinische Daten, lungenfunktionelle und radiologische Befunde sowie unerwunschte Arzneimittelwirkungen wurden erfasst. Ergebnisse: Die durchschnittliche Therapiedauer mit Pirfenidon betrug 48 Wochen (Bereich [3–321] Wochen). 16 Patienten (35 %) erhielten Pirfenidon allein und 29 in Kombination (65 %) mit Steroiden und/oder N-Acetylcystein (NAC). Am Ende der Beobachtungszeit zeigten 28 der 40 Patienten mit uber 3-monatiger Therapiedauer (70 %) einen stabilen Befund. Insgesamt traten bei 26 Patienten (58 %) Nebenwirkungen auf; diese waren am haufigsten (n = 17 [38 %]) gastrointestinaler Natur. Pirfenidon wurde wegen Nebenwirkungen von 6 Patienten (13 %) abgesetzt. Die mediane Uberlebenszeit nach Beginn der Therapie lag bei 3,8 Jahren. Folgerung: Pirfenidon als Monotherapie sowie in Kombination mit NAC und/oder Kortikosteroiden war im Allgemeinen gut vertraglich. Schwere Nebenwirkungen traten selten auf. Bei zwei Drittel der Patienten stabilisierte sich die IPF unter der Therapie. Unsere Untersuchungsergebnisse entsprechen den bisher vorliegenden Daten uber die Wirksamkeit und Vertraglichkeit von Pirfenidon in der Therapie der idiopathischen Lungenfibrose.

Background Video-assisted thoracotomy is the recommended tool for differential diagnosis of Idiopathic Interstitial Pneumonias (IIPs) when clinical, radiological and BAL data are inconclusive after multidisciplinary team discussion (MDTD). However, in the last years transbronchial cryobiopsy has gained importance as a new diagnostic tool. Aims and Objectives The aim of this study was to analyze diagnostic yield, safety and complications of transbronchial cryobiopsies performed in our centre from January 2014 till December 2015. Methods All procedures were performed in rigid bronchoscopy. Freezing time was three to five seconds. The biopsy was manually separated from the probe within a few seconds using warm water. The flexible bronchoscope was immediately reintroduced into the airways to control a possible bleeding. A Fogarty balloon was not implemented. Data on transbronchial fluoroscopy-guided cryobiopsies in rigid bronchoscopy was reviewed retrospectively. Demographics, clinical variables, intra-procedural and post-procedural complications and histological results were recorded. Results 54 patients (32 male, 22 female) with IIPs underwent transbronchial cryobiopsy. The histological diagnosis was achieved in 79.6% of cases. Final diagnosis was made after MDTD. The most frequently identified pattern was non-specific interstitial pneumonia. Intrabronchial bleeding occurred in 32 cases, none required interventional procedures. Pneumothorax occurred in eight cases (14.8%), six required a chest tube for less than seven days, none required surgical intervention. Conclusion Transbronchial cryobiopsy is a safe alternative to surgical lung biopsies and provides a high diagnostic yield in ILDs.

Erratum zu: Pneumologe 2018 https://doi.org/10.1007/s10405-018-0205-7 Aufgrund eines Produktionsfehlers wurde in der ursprunglich veroffentlichten Version dieses Beitrags sowohl in der deutschen als auch in der englischen Uberschrift der Untertitel „Interstitial pneumonia with autoimmune features …

Background: Hypnic headache (HH) is a rare primary headache disorder characterized by strictly sleep-related headache attacks. Most patients are over the age of 50 and usually awake at the same time at night with dull bilateral head pain. The pathophysiology of this headache disorder is still enigmatic but association with rapid eye movement (REM) sleep and sleep-disordered breathing (SDB) has been suggested. Methods: Six patients with HH according to the current International Classification of Headache Disorders (ICHD-II) criteria (code 4.5) were investigated. Serial polysomnography (PSG) was performed in each patient for four consecutive nights. Results: A total of 22 HH attacks were recorded from all patients during PSG. Six of the monitored headache attacks arose from REM sleep; 16 attacks, however, arose from different non-REM (NREM) sleep stages. Five patients showed an increased apnoea/hypopnoea index (>5), indicating obstructive sleep apnoea (OSA) on some but not the majority of nights. Headache onset and occurrence of SDB were not temporally connected. Conclusions: This prospective study shows that the onset of HH was not associated with sleep stage. These results contradict the current belief that REM sleep and SDB play a crucial role in the pathophysiology of HH.

Background Non-specific interstitial pneumonia (NSIP) is a rare lung disease mostly associated with connective tissue diseases (CTD), hypersensitivity pneumonitis, acute lung injury or drugs. After exclusion of known causes, NSIP can be defined as idiopathic. Familial occurrence has been described anecdotally. Case Presentation: We here report the presentation of a 38-year old monozygotic, female pair of twins with simultaneous diagnosis of interstitial lung disease in our institution as a hint for a probable genetic association. Two 38-year-old monozygotic twin sisters, living in two different cities in Germany, presented with a 3 years history of breathlessness and cough after exertion. Rheumatologic laboratory investigations only revealed positivity of antinuclear antibodies (1:80) in one of the two patients. Lung function tests showed mild restriction and reduced diffusion capacity in both cases. High-resolution-chest-CT-scans showed reticulation and ground-glass opacities. Transbronchial cryo-biopsies were performed. Histopathological findings were typical for NSIP. On the basis of a multidisciplinary discussion the diagnosis of idiopathic NSIP could be confirmed in both cases. Conclusion: The simultaneous occurrence of NSIP in these twins, although living in different cities, having different jobs and a negative family history for pulmonary fibrosis strongly suggests that genetic susceptibility is more important than environmental factors in triggering sporadic idiopathic NSIP.

Introduction: Data on efficacy of immunosuppressive treatment with azathioprine (AZA) in CTD-ILD are still poor. KL-6 is an established biomarker for interstitial lung disease but its use to evaluate treatment response is still unknown. Aim of Study: To investigate the role of serum KL-6 as a biomarker to assess response to AZA in CTD-ILD patients. Patients and Methods: 54 patients with CTD-ILD (19 rheumatoid arthritis, 15 systemic sclerosis, 8 polymyositis/antisynthetase syndrome, 4 undifferentiated connective tissue disease, 3 sjogren syndrome, 3 mixed connective tissue disease, 1 psoriatic arthritis, 1 polymyalgia rheumatica) treated with AZA were studied. Progression was defined as decrease of FVC ≥5% or DLco ≥10% pred and worsening of clinical symptoms or new radiological findings. KL-6 was measured in serum at start of therapy and at last visit by NANOPIA. Results: Median follow up time was 21 months (range: 2- 105 months) and mean treatment duration was 35 months. 15 patients (28%) discontinued AZA within 3 months mostly due to increased liver enzymes. 39 patients (72%) received AZA longer than 3 months. Among these, 25 (64%) patients responded to treatment according to the predefined criteria. Mean serum KL-6 level at start of treatment was 2247±1526 U/L and 2114±1574 at the end of follow up (p=0.067). No correlations were seen between serum KL-6 and baseline FVC and DLco and between changes of KL-6 and changes in FVC or DLco over time. Responders showed a decrease of KL-6 by 19% over time, and non-responders an increase by 20% (p=0.061). Conclusions: Serum KL-6 seems to contribute partly to assess response to AZA in CTD-ILD.

Die obstruktive Schlafapnoe und die arterielle Hypertonie sind sehr haufige, aber leider auch oft nicht erkannte Erkrankungen. Der ursachliche Zusammenhang von Schlafapnoe und Hypertonie ist gesichert. Wahrscheinlich ist die undiagnostizierte Schlafapnoe die haufigste Ursache der fruher so genannten „essentiellen“ Hypertonie. Besonders wichtig erscheint daher die Identifikation dieser Patienten. Bei allen Hypertonikern sollten nach Schnarchen, Atemstillstanden und Tagesmudigkeit gefragt werden, der Halsumfang gemessen und ggf. eine ambulante Messung der nachtlichen Atmung (kardiorespiratorische Polygraphie) erfolgen. Insbesondere sollten alle Patienten mit schwer einstellbarer Hypertonie oder fehlender Nachtabsenkung in der 24-h-Blutdruckmessung auf ein obstruktives Schlafapnoesyndrom gescreent werden. Umgekehrt sollten alle Patienten mit Schlafapnoe gezielt auf eine arterielle Hypertonie untersucht werden. Da bei diesen Patienten auch nur eine nachtliche arterielle Hypertonie vorliegen kann, reichen Einzelmessungen am Tage nicht aus, um einen Bluthochdruck sicher auszuschliesen. Die CPAP-Therapie („continuous positive airway pressure“) kann den Blutdruck bei hypertensiven Schlafapnoepatienten effektiv senken. Dies gilt besonders fur Patienten mit obstruktiver Schlafapnoe und therapierefraktarer Hypertonie. Fur nachtliche, durch Schlafapnoe induzierte Blutdruckspitzen scheint die CPAP-Therapie die zurzeit am besten dokumentierte Therapieform zu sein.

Introduction Polysomnography (PSG) is considered the gold standard in the diagnosis of sleep disordered breathing (SDB). Because of costs and labor-intensity it is, however, performed last in graded diagnostic protocols that often involve respiratory pressure measurements via nasal canula as an alternative sensitive method for SDB detection. MicroMESAM, a newly developed screening device based on this method, allows automated analysis of apnoeas, hypopnoeas and snoring. Aim and methods To validate the device, we first compared signal quality of MicroMESAM flow-time curves with those generated by a pneumotachograph. Then, in 50 patients suspected of having obstructive sleep apnoea, we compared MicroMESAM-generated automated analysis with manually scored results of simultaneously collected PSG data. Results MicroMESAM-generated flow-time curves correspond with pneumotachograph-generated curves in 95% of respiratory events, resulting in less 4 +/- 2% difference in respective area under the curves. MicroMESAM and PSG generated numbers of apnoeas (r = 0.99) and hypopnoea (r = 0.81), as well as AHI (r = 0.98) correlated highly, displaying mean differences in AHI of 3.8, and in 1.96 sigma interval of + 11.1 to - 3.5/h. Sensitivities and specificities for SDB were 97.3%, respective 46% at SDB-defining AHI of 5, and 100%, respective 87.5%, at SDB-defining AHI of 10. Summary MicroMESAM-generated flow-time curves correspond well with pneumotachograph generated curves, producing automated AHIs that are highly sensitive in detecting SDB. MicroMESAM, therefore, is suitable as a screening device for SDB.

Scarce evidence suggests that ambient air pollution and temperature might play a role in incidence and severity of sleep disordered breathing (SDB). We investigated the association of short-term exposure to fine particulate matter (particles with a 50% cut-off aerodynamic diameter of 10 μm (PM10)), ozone and temperature with SDB in the general population.Between 2006 and 2008, 1773 participants (aged 50–80 years) of the Heinz Nixdorf Recall study underwent screening for SDB, as defined by the apnoea–hypopnoea index (AHI). We assessed daily exposure to PM10, ozone, temperature and humidity. We used multiple linear regression to estimate associations of daily PM10, ozone levels and temperature on the day of screening, adjusting for relative humidity, season, age, sex, body mass index, education, smoking habits, alcohol consumption and physical activity.In the study population, the mean±sdAHI was 11.2±11.4 events·h−1. Over all seasons, an interquartile range increase in temperature (8.6°C) and ozone (39.5 µg·m−3) was associated with a 10.2% (95% CI 1.2–20.0%) and 10.1% (95% CI 2.0–18.9%) increase in AHI, respectively. Associations for temperature were stronger in summer, yielding a 32.4% (95% CI 0.0–75.3%) increase in AHI per 8.6°C (p-value for season–temperature interaction 0.08). We observed that AHI was not associated with PM10.This study suggests that short-term variations in ozone concentration and temperature are associated with SDB.

Introduction: Few studies have compared the diagnostic role of endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA), a minimally invasive technique used routinely for investigation of mediastinal and hilar lymphadenopathy, with traditional endoscopic diagnostic approaches in sarcoidosis. Aim of this study was to compare the diagnostic efficiency of mucosal biopsy, transbronchial biopsy (TBB) and EBUS-TBNA for confirming sarcoidosis. Patients and Methods: 58 consecutive patients (33 men, 25 women, 48 ± 16 years) in whom the suspected initial diagnosis of sarcoidosis had been supported by confirmation of granulomas, were retrospectively investigated for the diagnostic yield of the three bronchoscopic biopsy procedures. Radiological CXR stages were: stage I 36%, stage II 54%, stage III 10%. Bronchoscopy with mucosal biopsy was performed in 49/58, TBB in 33/58 and EBUS-TBNA of mediastinal and hilar lymph nodes in 49/58 cases in the same session. In 21 patients all diagnostic approaches were combined in one session. Results: Confirmation of granulomas was achieved in 43% of mucosal biopsies (regardless of macroscopic appearance), 82% of TBB and 76% of EBUS-TBNA. According to radiological stages (I vs. II) the diagnostic yield was: mucosal biopsy 42% vs. 48%, TBB 33% vs. 91%, EBUS-TBNA 85% vs. 73%. There were 3 postinterventional pneumothoraces. Conclusion: In this single center study, EBUS-TBNA achieved the best diagnostic yield in patients with sarcoidosis stage I but TBB seems to remain the better option for stage II disease.

Background: Nintedanib, a tyrosine kinase inhibitor approved for the treatment of idiopathic pulmonary fibrosis (IPF), exhibits antifibrotic and antiinflammatory activity. Th1 cytokines with IFN-γ inducing capacity have been shown to inhibit angiogenesis through up-regulation of ELR-CXC chemokines in mice. The aim of the study was to investigate the effect of nintedanib on the production of angiogenic/ angiostatic cytokines by AMs in ILD. Methods: AMs obtained by bronchoalveolar lavage fluid (BALF) from 9 ILD patients (5 IPF and 4 idiopathic nonspecific interstitial pneumonias (iNSIP)) were cultured with/without lipopolysaccharide (LPS). The effects of nintedanib at different concentrations (0, 0.1, 0.3, 1.0 μmol/L) on the production of Th1 cytokines (IL-12p40 and IL-18), angiogenic chemokines (IL-8, SDF-1α), and an angiostatic chemokine (MIG) were measured in the cell culture supernatants by Luminex bead based assay. Results: The spontaneous release of MIG by AMs was higher in IPF than in iNSIP (454.3±167.6 vs. 73.8±29.1 pg/mL/10 6 AMs, p 6 AMs, p Conclusions: Nintedanib seems to reduce the release of IL-12p40, IL-18 and MIG by AMs from patients with IPF and iNSIP in a similar way.

Einleitung: Ein groser Teil aller Patienten mit Schlaganfall leidet an einer obstruktiven Schlafapnoe (OSA), die zum Zeitpunkt des akuten Ereignisses nicht bekannt ist. Zwar gilt die Polysomnographie (PSG) bei Verdacht auf OSA als diagnostischer Goldstandard, die routinemasige Durchfuhrung ist jedoch nicht praktikabel. Eine Pulsoximetrie konnte jedoch ein einfach anwendbarer und kostengunstiger Screeningtest sein. Methodik: Bei 184 Schlaganfallpatienten wurde das wahrend der Polysomnographie (PSG) digital aufgezeichnetete pulsoximetrische Signal vollautomatisch mittels einer Software (AHDip) im Hinblick auf Desaturationen, definiert als Abnahme der Sauerstoffsattigung von ≥ 4 %, analysiert. Der Apnoe-Hypopnoe-Index (AHI) in der PSG wurde als Goldstandard definiert und mit der Anzahl der mit AHDip automatisch erkannten Sauerstoffentsattigungen (ODI) pro Stunde Aufzeichnungsdauer verglichen. Ergebnisse: Die Korrelation zwischen AHI in der PSG und Desaturationsindex (ODI) in der Pulsoximetrie betrug r = 0,84 (p < 0,001). In Abhangigkeit von der Definition einer relevanten schlafbezogenen Atmungsstorung (AHI 10, 15, 20 oder 30/h) und dem angewandten cut-off-Wert fur den ODI (z. B. 15/h) ergaben sich fur die Pulsoximetrie eine Sensitivitat von 32 - 83 % und eine Spezifitat von 99 - 96 %. Die diagnostische Genauigkeit (gemessen als area under the curve der ROC-Kurve) bei einem AHI ≥ 20 als Diagnose einer SBAS betrug 96 %. Schlussfolgerung: Bei Patienten mit Schlaganfall ermoglicht die automatische Analyse des pulsoximetrischen Signals mittels AHDip eine realistische Erkennung schlafbezogener Atmungsstorungen und konnte allgemein in Populationen mit hoher Vortestwahrscheinlichkeit eingesetzt werden. Background: The prevalence of sleep-disordered breathing, particularly obstructive sleep apnea, among stroke patients is high. Routine screening with the current diagnostic gold standard of polysomnography is not feasible. Pulse oximetry could be a simple screening test. Methods: The signal of pulse oximetry, recorded during full polysomnography in 184 stroke patients during neurological rehabilitation, was analyzed automatically by software for desaturations ≥ 4 %. The polysomnographic apnoea-hypopnoea-index (AHI) was used as the diagnostic gold standard and compared with the oxygen desaturation index (ODI). Results: Correlation between AHI of PSG and ODI of oximetry was r = 0.84 (p < 0.001). Dependent on the definition of SDB (AHI 10, 15, 20 or 30/h) and the cut-off-point for the ODI (e. g. 15/h) sensitivity was 32 - 83 % and specificity 99 - 96 %. The diagnostic accuracy as determined by the area under the ROC-curve was 96 %. Conclusion: Automated analysis of pulse oximetry gives reasonable results for screening for SDB in stroke patients and could be used in populations with high pre-test probability.

The prevalence of obstructive sleep apnoea (OSA) following stroke is high and OSA is associated with increased morbidity, mortality and poor functional outcome. Nasal continuous positive airway pressure (nCPAP) is the treatment of choice for OSA, but its effects in stroke patients are unknown.The effectiveness and acceptance of treatment with nCPAP in 105 stroke patients with OSA, admitted to rehabilitation was prospectively investigated. Subjective wellbeing was measured with a visual analogue scale in 41 patients and 24-;h blood pressure was determined in 16 patients before and after 10 days of treatment. Differences were compared between patients who did and did not accept treatment.There was an 80% reduction of respiratory events with concomitant increase in oxygen saturation and improvement in sleep architecture. No serious side-effects were noticed. Seventy-four patients (70.5%) continued treatment at home. Nonacceptance was associated with a lower functional status, as measured by the Barthel Index, and the presence of aphasia. Ten days after initiation of nCPAP, compliant users showed a clear improvement in wellbeing (differences in visual analogue scale (ΔVAS) mean±sd 26±26 mm)versusnoncompliant patients (ΔVAS 2±25 mm, p=0.021). Only the compliant group had a reduction in mean nocturnal blood pressure (ΔBP; −8±7.3 mmHgversus0.8±8.4 mmHg, p=0.037).Stroke patients with obstructive sleep apnoea can be treated effectively with nasal continuous positive airway pressure and show a similar improvement and primary acceptance to obstructive sleep apnoea patients without stroke. Continuous positive airway pressure acceptance is associated with improved wellbeing and decreased nocturnal blood pressure.