The aim of this study was to develop a Croatian Delphi-based expert consensus for screening interstitial lung disease (ILD) associated with connective tissue disease (CTD). A systematic literature review was conducted on risk factors for the development of ILD, prevalence and incidence of ILD, diagnostic and screening methods for ILD, and prognosis of ILD in idiopathic inflammatory myopathy (IIM), mixed connective tissue disease (MCTD), primary Sjögren’s syndrome (pSS), rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and systemic sclerosis (SSc) were performed. Based on the evidence found, experts developed questionnaires for screening and monitoring ILD in each CTD, which were provided via an online survey. Following the electronic survey, two screening algorithms were developed based on the consensus opinions. The detection strategy for ILD included high-resolution computed tomography (HRCT) in addition to pulmonary function testing for IIM, MCTD, and SSc. and pulmonary function testing for newly diagnosed pSS, RA and SLE. However, in patients with identified risk factors for ILD HRCT, these tests should also be performed. A screening strategy for early identification of patients with various CTD-ILD was first developed by a multidisciplinary team of rheumatologists, pulmonologists, and radiologists to identify early CTD patients at risk of ILD, a severe extra-articular manifestation of CTD.

Pulmonary hypertension (PH) is a chronic disease which severely impairs quality of life (QoL). The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) is the first disease-specific tool to assess patient-reported symptoms,...

Interstitial lung diseases (ILD) are a heterogeneous group of diseases and one of the differential diagnosis which have to be excluded during diagnostic procedures are malignancies. We will present four patients who were referred to our Department because of suspicion of interstitial lung diseases according to radiology finding. In one case only, one of the radiologist’s differential diagnosis was pulmonary lymphangitic carcinomatosis. All four patients had exertional dyspnea and dry cough which are nonspecific and can be first manifestation of ILD or obstructive lung diseases. After diagnostic evaluation in three cases, diagnosis was pulmonary lymphangitic carcinomatosis due to metastatic lung adenocarcinoma and in one due to metastatic adenocarcinoma of unknown primary origin. Patients with lymphangitic carcinomatosis have poor prognosis without treatment and usually die because of respiratory failure. With these four cases we want to highlight importance of thinking about malignancies when we have patients with suspicion of interstitial lung disease especially when reticular pattern is present on chest X ray. We also wanted to show how important is radiology finding and multidisciplinary approach, and how radiologist’s differential diagnosis can be very helpful in making decisions in further investigations and way of clinicians thinking.

Lung cancer incidence in heart transplant patients is higher than in general population and correlates with smoking history. EGFR-mutations are more frequent in adenocarcinoma and among non-smoking women but incidence in solid organ transplanted patients is still not known. We present case of a 65-year-old ex-smoker male with history of heart transplantation and EGFR positive metastatic lung adenocarcinoma. At admission he was in a severe clinical condition and treatment with erlotinib was started. Initially he had good clinical and radiologic response to treatment with only grade 1 side effects. Data about drug interactions between cyclosporine and erlotinib are insufficient but we have to take this interaction into consideration during treatment because both drugs are substrates and inhibitors of CYP34A. In our case erlotinib was safe and well tolerated drug, there were no relevant toxicity, but close monitoring and dose reduction of cyclosporine was needed.

Background: Sarcoidosis is a highly heterogeneous disease, leading to differences in clinical practices across countries. This study aimed to identify similarities and differences in diagnostic, therapeutic, and follow-up practices for sarcoidosis across European coun-tries. Methods: Within the Joint Action on REspiratory Diseases (JARED) project, we conducted a multinational, cross-sectional study using a key informant expert survey. The structured survey comprised 48 questions on adult sarcoidosis diagnostics and management. Rep-resentatives of all 15 JARED project countries completed the electronic survey. Results: Sarcoidosis and/or interstitial lung disease centres are present in 12 countries, but only 7 have national guidelines for diagnosis and treatment. In most countries, bron-choscopy with bronchoalveolar lavage and endobronchial ultrasound-guided lymph node biopsy are performed. The use of bronchoscopic lung biopsies, particularly cryo-biopsies, varies considerably. To assess the extent and activity of the disease, almost all countries perform extended pulmonary function testing, but there are variations in the use of hypercalcaemia and hypercalciuria tests. Positron emission tomography is used routinely only in 4 countries. Around half of the patients are treated with corticosteroids. In most cases, treatment is initiated when an organ other than the lungs is affected or when severe pulmonary function impairment is present. The follow-up intervals range from 1 to 12 months. The monitoring strategies vary based on the radiological stage of sarcoidosis or the severity of the disease. Conclusions: Our survey highlights substantial variation in sarcoidosis management across Europe, underscoring the need for updated, evidence-based recommendations on diagnostic strategies, treatment initiation and duration, and long-term follow-up.