The presence of staphylococcal superantigens in nasal swabs and correlation with activity of granulomatosis with polyangiitis in own material.

OBJECTIVES: Nasal carriage of Staphylococcus aureus and its superantigens (SAg) seem to be a risk factor disease exacerbation in granulomatosis with polyangiitis (GPA). We investigated the association between the presence of SAg in nasal swabs and activity of disease in GPA patients also taking into account correlation with an antimicrobial treatment. METHODS: In a prospective study of a total of 150 GPA patients hospitalised in the period 2009-2016, nasal swabs were examined for the presence of Staphylococcus aureus and SAg. Subsequently, the association with disease activity was assessed. RESULTS: Of 362 Staphylococcus aureus-positive nasal swab cultures from 115 of the 150 patients, the presence of at least one SAg in 126 samples (34.8%) from 56 patients (48.7%) was found. Among the 17 patients with limited to subglottic stenosis (SGS) disease, SAg were detected in 6 cases (35.3%). We did not find a significant correlation between the presence of SAg and disease activity (p=0.986), although when individual SAg were analysed separatively, SED and TSST-1 were more frequently present in active disease. Additionally, the results of the analysis demonstrated a protective effect of trimethoprim/sulfamethoxazole (T/S) treatment (0R 0.52, p<0.0092) in GPA patients. Interestingly, GPA limited to SGS appeared as an unfavourable factor associated with disease activity (0R 1.84, p=0.05). CONCLUSIONS: The association between staphylococcal SAg in nasal swabs and GPA activity is not evident. Multiple mechanisms that may lead to disease activation still need to be investigated.

Progressive fibrosis in interstitial lung diseases - proposed definition and management.

Interstitial lung diseases may have an unpredictably progressive course, which is manifested as progression of pulmonary fibrosis, causing an increasing impairment of lung function affecting a poor prognosis. The possibility of an effective antifibrotic treatment is a chance for patients to slow down the progression of the disease, perhaps even extend their life. For this reason, standardization of the definition as well as identification criteria for progressive fibrosis interstitial lung disease is a method for optimizing the management in this group of patients.

Diagnosis of sarcoidosis - the updated ATS 2020 recommendations through the prism of everyday clinical practice.

Diagnosis of sarcoidosis is not standardized, but there is a general consensus that it should be based on the following criteria: consistent, adequate clinical presentation, demonstration of the presence of granulomatous lesions in pathomorphological examination (in one or more tissue samples) and the exclusion of alternative causes of granulomatous disease. In this article, the reader will find, briefly presented, the most important position of ATS experts regarding selected aspects of the diagnosis of sarcoidosis, with a comment from the authors of this editorial.

Diagnostic and treatment standards in idiopathic pulmonary fibrosis in the era of antifibrotic drugs in Poland: A real-world practice survey.

Background Currently, only two drugs have been shown to modify the inevitable natural history of idiopathic pulmonary fibrosis (IPF). Changes in the reimbursement policy for antifibrotic drugs in Poland have led to the availability of pirfenidone from January 2017 and nintedanib from March 2018 for the treatment of Polish patients with IPF. This study aimed to evaluate the possible changes and shortcomings in the clinical practice standards in IPF in the era of access to antifibrotic therapy in Poland. Methods A real-world data survey was performed among physicians attending the Polish Respiratory Society Congress held in May 2018. The present survey was a follow-up to the previous survey undertaken in 2016, before the availability of antifibrotics in Poland. Results A total of 99 physicians participated in the survey, among which 80% were pulmonologists. The majority of participants (83%) represented hospital-based clinicians and most of them (93%) were involved in interstitial lung diseases (ILD) management. As many as 63% of the respondents elaborate the final diagnosis of IPF working with the expert radiologist routinely, 47% do that in the cooperation with other pulmonologists, and if a biopsy was performed 39% discuss its results with the expert pathologist. Bronchoalveolar lavage (BAL) and surgical lung biopsy (SLB) would never be recommended in the differential diagnosis of IPF by 9% and 16% of the respondents, respectively. Corticosteroids (CS) or a combination of CS and immunosuppressants (IS) is still recommended by 22% of participants. Proton pump inhibitors (PPI) in the case of symptomatic GERD are prescribed by 44% of the respondents, and 12% prescribe PPI regardless of GERD symptoms. Pirfenidone is used by 70%, and nintedanib by 48% of the respondents. Only 39% of the respondents refer patients with IPF to professional rehabilitation centers. Conclusions The level of cooperation between pulmonologists and other specialists in the diagnostic workup of IPF is unsatisfactory. IPF treatment practices in the era of access to effective drugs in Poland require immediate improvement. There is an urgent need to develop the local Polish practical guidelines to improve the management of IPF.

"The significance of cardiac magnetic resonance imaging in detection and monitoring of the treatment efficacy of heart involvement in eosinophilic granulomatosis with polyangiitis patients".

BACKGROUND: Cardiac magnetic resonance imaging (CMRI) has emerged as a sensitive and non-invasive technique in the evaluation of cardiac lesions in eosinophilic granulomatosis with polyangiitis (EGPA) patients. OBJECTIVES: To evaluate the ability of CMRI to detection and monitoring of the treatment efficacy in EGPA patients with cardiac involvement. METHODS: To the retrospective-prospective study were enrolled 33 cardiac involvement EGPA patients. In 19 of them CMRI at the moment of diagnosis was performed, in 14 - CMRI after treatment was made, when this method was available - in this group the cardiac involvement was based on the clinical findings. All patients were treated with corticosteroids (CSs) and/or cyclophosphamide (CY). In the first group the control CMRI after one year of treatment was performed, but in the second group the time from the end of the treatment to execution of CMRI was 2-5 years. RESULTS: All patients had heart injury in CMRI. Myocardial edema was present in 87.8% cases, 54.5% of patients had perfusion defects and in all - late gadolinium enhancement was observed. Control CMRI was performed in 32 cases. Improvement was observed in 81% of patients - in 11% of them all lesions undergone completely remission and in 35% of them evolution to global fibrosis was found. In 7% of patients stabilization was achieved and in 12% - progression was observed. CONCLUSIONS: CMRI is a sensitive method detecting cardiac lesions in EGPA patients. It helps to detect patients, who need combined therapy and helps evaluate the therapeutic effect.

Intratracheal Dilation-injection Technique in the Treatment of Granulomatosis with Polyangiitis Patients with Subglottic Stenosis.

OBJECTIVE: An analysis of subglottic stenosis (SGS) occurrence frequency in patients with granulomatosis with polyangiitis (GPA) based on the time of appearance of clinical symptoms, and an assessment of treatment effectiveness, in particular with the intratracheal dilation-injection technique (IDIT). METHODS: Review and treatment with IDIT of 34 patients with SGS associated with GPA. RESULTS: SGS developed in 34 of 250 patients with GPA (13.6%) and was not reflective of disease activity in the organs in 15 of 34 patients (44%): 11 cases after and 4 cases during immunosuppressive therapy (IST) when patients did not have organ symptoms. All patients underwent IDIT and in total, the treatment resulted in immediate improvement. In addition, in 21 cases, IST was applied because of other organ involvement or of the lack of longterm efficacy of IDIT. The median time of response was 37 months and the median interval between sessions was 5 months. None of the patients required tracheostomy after beginning IDIT in our hospital. CONCLUSION: SGS often occurs independently of other features of active GPA. IDIT is a safe and effective technique in the treatment of GPA-related SGS. It should be performed in all patients with GPA who develop significant SGS and in those with multiorgan disease concomitantly with IST. In patients with isolated SGS, IDIT also makes IST and tracheostomy unnecessary.