Interstitial lung disease (ILD) is a serious complication of rheumatoid arthritis (RA) contributing to significantly increased morbidity and mortality. Other respiratory complications, such as chronic obstructive pulmonary disease and bronchiectasis, are frequent in RA. Infections and drug toxicity are important differential diagnoses and should be considered in the diagnostic work-up of patients with RA presenting with respiratory symptoms. This review provides an overview of the epidemiology and pathogenesis of RA-ILD, the radiological and histopathological characteristics of the disease as well as the current and future treatment options. Currently, there is no available evidence-based therapy for RA-ILD, and immunosuppressants are the mainstay of therapy. Ongoing studies are exploring the role of antifibrotic therapy in patients with progressive fibrotic ILD, which may lead to a new treatment approach for subgroups of patients with RA-ILD.
Purpose The clinical manifestations of sarcoidosis vary widely, depending on the intensity of the inflammation and the organ systems affected. Hence, sarcoidosis patients may suffer from a great variety of symptoms. The aim of this study was to compare the self-reported burden of sarcoidosis patients in Denmark, Germany and the Netherlands, especially the prevalence of fatigue and small fiber neuropathy (SFN)-related symptoms, as well as differences in treatment strategies. Methods A cross-sectional web-based anonymous survey about complaints was conducted among sarcoidosis patients. Patients were invited to take part through the sarcoidosis patient societies as well as through outpatient sarcoidosis clinics in these countries. Results The questionnaire was completed by 1072 sarcoidosis patients (152 Danish, 532 German and 388 Dutch). Almost all patients reported having sarcoidosis-associated symptoms (organ-related as well as non-specific, non-organ related). Fatigue was reported by almost all respondents (90%), followed by pulmonary symptoms (72.4%). More than 50% of the respondents were being treated with prednisone, which was comparable in all three countries. In contrast, second- and third-line treatment differed substantially between Denmark, Germany and the Netherlands. Conclusion Sarcoidosis patients in Denmark, Germany and the Netherlands present with similar self-reported symptoms, organ-related as well as non-specific, non-organ related. Fatigue (90%) and symptoms associated with SFN (86%) were highly prevalent in all three countries. Electronic supplementary material The online version of this article (10.1007/s00408-019-00206-7) contains supplementary material, which is available to authorized users.
Two cases: A 66-year-old woman was referred to the hospital due to dyspnoea and cough. Seven months prior to referral, the patient had choked on a chunk of nut and grain-filled bread. She had daily cough and dyspnoea. The patient was convinced of an airway foreign body and she contacted her general practitioner and the emergency service several times; they all found this unlikely. Fibre optic bronchoscopy revealed two obstructing nut-like foreign bodies in the right upper and lower lobe, respectively. A 77-year-old man with sarcoidosis developed increased dyspnoea and sputum production. Three weeks earlier, the patient had choked on a magnesium tablet. Everyone was convinced that the tablet had dissolved. Infection was suspected. Chest CT scan was performed showing no obvious signs of infection or progression in sarcoidosis. After the CT scan, the patient coughed up the remains of the tablet and his symptoms resolved. Retrospective evaluation of the CT scan revealed the tablet.
Hypersensitivity pneumonitis (HP) is a complex pulmonary disorder mediated by the immune system and caused by various inhaled antigens against which the subject has previously been sensitized. In about 50% of the cases, the antigen is not identified. Identification and removal of the eliciting antigen is important for the prognosis.We report two cases of HP caused by molds and atypical mycobacteria isolated from wind instruments. We present the first case of HP caused by bassoon playing and another case of HP caused by molds in a trombone. HP caused by fungi and bacteria in wind instruments may be much more common than previously thought.HP caused by fungi and bacteria in wind instruments is probably underdiagnosed; this calls for more clinical attention when HP is suspected.
Background: Idiopathic pulmonary fibrosis (IPF) is a serious interstitial lung disease (ILD) with a median survival of 3-5 years. The aim of the present study was to evaluate disease severity and survival in patients diagnosed with IPF in the era of antifibrotic therapies compared with an earlier IPF cohort. Methods: We identified all patients with fibrotic ILD in the hospital electronic case record system between 2011 and 2016, and reviewed each case in order to identify incident patients with IPF. We used the GAP-index to compare disease severity and mortality to previous findings in patients with IPF diagnosed at our center between 2003 and 2009. Results: 260 patients were diagnosed with IPF between 2011 and 2016. Mean age was 72.6 years, 79% were male, mean forced vital capacity (FVC) was 80%, and mean diffusing capacity for carbon monoxide (DLco) was 44%. Age, FVC and DLco were significant predictors of mortality, but the presence of a typical usual interstitial pneumonia pattern on HRCT was not. Eighty percent of patients in GAP stage I received antifibrotic therapy, 73% in GAP stage II, and 29% in GAP stage III. The median survival was four years in the 2011-2016 cohort compared with three years in the 2003-2009 cohort. The distribution of patients between GAP stages was unchanged in 2011-2016 compared with 2003-2009, (stage I 34% vs. 32%, stage II 49% vs. 48% and stage III 20% vs. 16%). One-year mortality was 13% in 2011-2016 and 26% in 2003-2009. In severe disease (GAP stage III), one-year mortality was 26% and 54%, respectively, (p=0.019). Conclusion: Short-term mortality was significantly lower in the 2011-2016 cohort compared with 2003-2009. This improvement may be linked to changes in treatment strategies towards limited use of corticosteroids. Although early diagnosis of IPF still needs increased focus, the improvement is encouraging.
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