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Vnetne revmatične bolezni so heterogena skupina bolezni, ki lahko prizadenejo tudi pljučni intersticij. Tako kot je pogostnost intersticijske pljučne bolezni (IPB) v okviru vnetnih revmatičnih bolezni variabilna, je tudi klinična slika zelo raznolika: od omejene nenapredujoče prizadetosti pljučnega intersticija do fulminantno potekajoče življenje ogrožajoče bolezni. Podatki za diagnosticiranje in zdravljenje IPB v sklopu vnetnih revmatičnih bolezni so razmeroma skopi. Temeljno zdravljenje praviloma predstavljajo imunomodulacijska zdravila. Nedavno so se jim pri progresivni pljučni fibrozi pridružili še protifibrotiki. Odločitev pri kom, kdaj in kako intenzivno zdraviti IPB pogosto predstavlja izziv. V prispevku podajamo sklepe multidisciplinarnega posvetovalnega sestanka slovenskih revmatologov in pulmologov, ki po mnenju avtorjev glede na dostopne podatke pomenijo dobro prakso pri obravnavi tovrstnih bolnikov. Osredinjamo se na bolnike s sistemskimi vezivnotkivnimi boleznimi in revmatoidnim artritisom.

Pljučnica je najpogostejši vzrok za težji potek okužbe z virusom SARS-CoV-2 in s hospitalizacijo. Potek covidne pljučnice je lahko različen; infiltrati, vidni na rentgenski sliki, se lahko resorbirajo spontano, včasih pa je potrebno zdravljenje s sistemskimi glukokortikoidi. Ob odpustu iz bolnišnice zdravljenje običajno še ni končano, zato je Združenje pulmologov Slovenije v želji po enotnem obravnavanju bolnikov s covidno pljučnico izdelalo mnenje za obravnavo in sledenje bolnikov po odpustu iz bolnišnice. Zavedamo se, da ob novi bolezni ne gre za dokončno mnenje, saj bodo nova spoznanja o covidni pljučnici zanesljivo zahtevala obnavljanje mnenj.

Background The relationship between anti-SARS-CoV-2 humoral immune response, pathogenic inflammation, lymphocytes and fatal COVID-19 is poorly understood. Methods A longitudinal prospective cohort of hospitalised patients with COVID-19 (n=254) was followed up to 35 days after admission (median, 8 days). We measured early anti-SARS-CoV-2 S1 antibody IgG levels and dynamic (698 samples) of quantitative circulating T-, B- and natural killer lymphocyte subsets and serum interleukin-6 (IL-6) response. We used machine learning to identify patterns of the immune response and related these patterns to the primary outcome of 28-day mortality in analyses adjusted for clinical severity factors. Results Overall, 45 (18%) patients died within 28 days after hospitalisation. We identified six clusters representing discrete anti-SARS-CoV-2 immunophenotypes. Clusters differed considerably in COVID-19 survival. Two clusters, the anti-S1-IgGlowestTlowestBlowestNKmodIL-6mod, and the anti-S1-IgGhighTlowBmodNKmodIL-6highest had a high risk of fatal COVID-19 (HR 3.36–21.69; 95% CI 1.51–163.61 and HR 8.39–10.79; 95% CI 1.20–82.67; p≤0.03, respectively). The anti-S1-IgGhighestTlowestBmodNKmodIL-6mod and anti-S1-IgGlowThighestBhighestNKhighestIL-6low cluster were associated with moderate risk of mortality. In contrast, two clusters the anti-S1-IgGhighThighBmodNKmodIL-6low and anti-S1-IgGhighestThighestBhighNKhighIL-6lowest clusters were characterised by a very low risk of mortality. Conclusions By employing unsupervised machine learning we identified multiple anti-SARS-CoV-2 immune response clusters and observed major differences in COVID-19 mortality between these clusters. Two discrete immune pathways may lead to fatal COVID-19. One is driven by impaired or delayed antiviral humoral immunity, independently of hyper-inflammation, and the other may arise through excessive IL-6-mediated host inflammation response, independently of the protective humoral response. Those observations could be explored further for application in clinical practice.

Z dokumentom želimo smernice, ki jih predlaga GINA, vključiti v slovenski prostor. Želimo, da bi dokument služil enotnemu in dogovorjenemu pristopu k obravnavi bolnikov z astmo na primarni in specialistični pulmološki ravni.

Recent studies have suggested that causative variants in telomerase complex genes (TCGs) are present in around 10% of individuals with idiopathic pulmonary fibrosis (IPF) regardless of family history of the disease. However, the studies used a case-control rare variant enrichment study design which is not directly translatable to routine practice. To validate the prevalence results and to establish the individual level, routine clinical practice, and utility of those results we performed next generation sequencing of TCGs on a cohort of well-characterized consecutive individuals with IPF (diagnosis established according to ATS/ERS/JRS/ALAT guidelines). Of 27 IPF patients, three had a family history of idiopathic interstitial pneumonia (familial IPF) and 24 did not (sporadic IPF). Pathogenic/likely-pathogenic variants (according to American College of Medical Genetics criteria) in TCG were found in three individuals (11.1%) of the whole cohort; specifically, they were present in 2 out of 24 (8.3%) of the sporadic and in 1 out of 3 (33.3%) of the patients with familial IPF. Our results, which were established on an individual-patient level study design and in routine clinical practice (as opposed to the case-control study design), are roughly in line with the around 10% prevalence of causative TCG variants in patients with IPF.

The purpose of this paper is to implement the guidelines proposed by GOLD in the Slovenian healthcare system, and to describe the cornerstones of the management of this disease. Te document is meant to serve as an agreed approach to the management of COPD patients.

The purpose of this paper is to implement the guidelines proposed by GINA in the Slovenian healthcare system, and to describe the cornerstones of the management of this disease. The document is meant to serve as an agreed approach to the management of asthma patients.

Akutne in kronične okvare pljuč po zdravljenju raka v otroštvu so po sodobnem zdravljenju redke in običajno brez kliničnih znakov ter simptomov. Pri bolnici, ki je bila v otroštvu zdravljena zaradi Hodgkinovega limfoma z obsevanjem vratu, supraklavikularnih bezgavk in mediastinuma ter s kemoterapijo, so kvarne posledice verjetna kombinacija neugodnih učinkov tega zdravljenja na srce in pljuča. Pri naši bolnici je glede na potek in napredovanje pljučne prizadetosti v ospredju vaskularna prizadetost - pljučna vaskulopatija, ki je bila s histološkim pregledom bioptata pljučnega tkiva tudi potrjena. Pri opisu želimo opozoriti na kroničen ter progredienten potek bolezni, ki terja zahtevno obravnavo specialistov in 22 let po zdravljenju povzroča težko invalidnost.

Abstract: Bronchoscopy with convex probe ultrasound in combination with transbronchial fine-needle aspiration biopsy is a relatively new, minimally invasive method that can also be used in an outpatient setting. It is indicated for staging of the mediastinal and hilar lymph nodes in patients with lung cancer, for the diagnosis of lung and mediastinal tumors, for the diagnosis of mediastinal lymphadenopaty and for the diagnosis and aspiration of mediastinal cysts. The method has a high diagnostic accuracy, which makes it comparable to more invasive diagnostic methods. At the same time it is less invasive for patients and offers a better safety profile.

Background: Welders’ lung is a pneumoconiosis of benign clinical course following inhalation exposure to welding fumes. It represents the deposition of iron oxides in the lungs. h e purpose of this study was to highlight the occupational history, appropriate diagnostic work-up and proper action at er the established diagnosis. Methods: We investigated i ve patients, longterm welders who were admitted to our hospital in the last two years because of possible interstitial lung disease. h ey underwent bronchoscopy with transbronchial biopsy and bronchoalveolar lavage, high resolution computed tomography of chest and pulmonary function testing. Results: h e patients were male, non- or exsmokers, with a long history of occupational exposure to welding. h e most common symptom was dyspnea on ef ort, accompanied with productive cough. Pulmonary function tests showed no abnormalities. HRCT disclosed centrilobular ground glass nodular opacities. Macrophagic reaction with positive stainig for iron was present in bronchoalveolar lavage. In lung tissue, we observed intense iron deposition with no i - brosis. Patients were discussed together with occupational medical doctor. h ey were advised to stop the exposure. Conclusions: h is study coni rms that longterm welders may have symptoms with no functional disorders, but with prominent morphological changes. h e key to correct diagnosis is an occupational history of the patient. Diagnostic work-up includes funda-mental procedures in suspected interstitial lung disease. h e best therapy is cessation of exposure.

Background Whipple’s disease is a chronic, multisystemic disease caused by a microorganism named Tropheryma whipplei. Disease is rare, so it presents a diagnostic problem to clinicians. Long-term treatment with antibiotics is successful. We report two patients with longstanding complaints and accurate histopathological diagnosis further on confirmed by molecular biology test. Methods Both patients presented with malaise, weight loss and laboratory parameters of chronic infection. Since rheumatological, haemathological and infective causes of complaints were ruled out, patients were admitted to our hospital. Histopathological examination in both cases confirmed Whipple’s disease and antibiotic treatment was started. Polymerase chain reaction using specific oligonucleotide primers for 16S rRNA was performed on formalin-fixed, paraffin-embedded tissue samples of both patients and peripheral blood of one patient. Results One patient presented with advanced disease and despite antibiotic treatment he died with signs of pulmonary arterial hypertension. The other patient was diagnosed early in the course of the disease and antibiotic treatment was effective. In tissue samples of both patients we found PAS-positive intracellular bacilli. By PCR method we detected a specific gene segment of Tropheryma whipplei in tissue samples and peripheral blood. Conclusions Presented two cases confirm the rarity of Whipple’s disease and illustrate the variety of nonspecific clinical findings which lead to late diagnosis. Histopathological changes are very specific on PAS staining. Molecular biology test aids in disease confirmation.

Astmo ima vsaj 5 % ljudi. Bolnike duši, ker se zaradi astmatskega vnetja zožijo dihalne poti. Ukrepi, ki zmanjšajo astmatsko vnetje, izboljšajo astmo. Vnetje zmanjšujemo z odstranjevanjem alergenov in dražljivcev ter predvsem z uporabo protivnetnih zdravil. Raziskave urejenosti astme po vsem svetu pokažejo, da v vsakdanjem življenju le malo bolnikov doseže tako dobro stanje urejenosti bolezni, kakršno je možno doseči v kliničnih raziskavah. Trenutno veljavne svetovne smernice za obravnavo astme imajo pomanjkljivost, da se osrediščajo v glavnem na predpisovanje zdravil. Dejstvo pa je, da na urejenost bolezni lahko vplivamo predvsem z zdravstveno vzgojo in večanjem zavzetosti bolnikov za zdravljenje.

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Priporočila prikazujejo, kakšna naj bo obravnava bolnikov s pljučnim rakom v Sloveniji v letu 2002. Predstavljajo osnovne postopke, ki jih želimo zagotoviti bolnikom s to boleznijo. Naslanjajo se na Smernice za internistično obravnavo bolnika s pljučnim rakom (1).

Background. We were interested to study the effectof different ways of administering topical lidocaine and thepossible benefit of using atropine as premedication for flexiblebronchoscopy.Patients and methods. 105 patients were randomised intofour groups. Lidocaine was administered either through thebronchoscope or by transcricoid injection. The use of atropinepremedication was also randomised. The duration of bronchoscopysessions, amount of lidocaine used, symptomes andsigns such as coughing, heart rate and oxygene saturationwere documented. Patients and medical staff graded their perceptionsof the procedure using a four-grade severity scale.Results. The duration of the bronchoscopy was not significantlyprolonged in the group with bronchoscopic lidocaine administrationcompared to the transcricoid group (p = 0.6). The patientswith bronchoscopic lidocaine administration requiredsignificantly more additional lidocaine than in the transcricoidgroup (p = 0.03). No significant difference was found inpatients who received atropine and those without it. Severecoughing recorded by bronchoscopists and nurses was significantlymore frequent in patients without atropine premedication(p = 0.006) but patient did not experience the bronchoscopyto be more unpleasant without atropine.Conclusions. Patients tolerated both variants of topical anaesthesiaequally well but less amount of additional lidocainewas used by transcricoid administration. The use of atropinewas of no benefit, but resulted in more tachycardia events.