Evaluation of prognostic factors for patients with eosinophilic granulomatosis with polyangiitis recruited at the pneumonological centre and mainly ANCA negativity: A retrospective analysis of a single cohort in Poland.

OBJECTIVES: The aim was to investigate the risk factors for relapse and death in patients with eosinophilic granulomatosis with polyangiitis (EGPA) recruited at the pneumonological centre and mainly antineutrophil cytoplasmic antibody negativity. METHODS: We retrospectively recruited 86 patients. Relapse was defined as the recurrence or appearance of new organ symptoms. The study end-point included the final examination. RESULTS: Relapses occurred in 34.9% of the patients, while 9.3% died. Immunosuppressive therapy (P = 0.042), prolonged low-dose corticosteroid treatments (mainly for asthma) (P = 0.006), and longer follow-up duration (P = 0.004) were associated with a higher relapse risk, while advanced EGPA severity (P = 0.0015) and activity (P = 0.044), older age of onset (P = 0.030), symptomatic cardiac involvement (P = 0.007), and postinflammatory cardiac fibrosis (P = 0.038) were associated with a higher risk of death. Sinusitis (P = 0.028) and prolonged low-dose corticosteroid treatments (P = 0.025) correlated with a better prognosis. Relapses did not have an impact on the mortality (P = 0.693). CONCLUSIONS: Relapses in EGPA remain frequent, although they do not impact mortality. Cardiac involvement is common, but clinically symptomatic cardiomyopathy is associated with a higher risk of death. Asthma requiring chronic corticosteroid treatments is associated with a lower risk of death, although the risk of EGPA recurrence is significantly higher.

Decreased Haemoglobin Level Measured at Admission Predicts Long Term Mortality after the First Episode of Acute Pulmonary Embolism.

Background: Decreased hemoglobin concentration was reported to predict long term prognosis in patients various cardiovascular diseases including congestive heart failure and coronary artery disease. We hypothesized that hemoglobin levels may be useful for post discharge prognostication after the first episode of acute pulmonary embolism. Therefore, the aim of the current study was to evaluate a potential prognostic value of a decreased hemoglobin levels measured at admission due to the first episode of acute PE for post discharge all cause mortality during at least 2 years follow up. Methods: This was a prospective, single-center, follow-up, observational, cohort study of consecutive survivors of the first PE episode. Patients were managed according to ESC current guidelines. After the discharge, all PE survivors were followed for at least 24 months in our outpatient clinic. Results: During 2 years follow-up from the group of 402 consecutive PE survivors 29 (7.2%) patients died. Non-survivors were older than survivors 81 years (40−93) vs. 63 years (18−97) p < 0.001 presented higher sPESI 2 (0−4) vs. 1 (0−5), p < 0.001 driven by a higher frequency of neoplasms (37.9% vs. 16.6%, p < 0.001); and had lower hemoglobin (Hb) level at admission 11.7 g/dL (6−14.8) vs. 13.1 g/dL (3.1−19.3), p < 0.001. Multivariable analysis showed that only Hb and age significantly predicted all cause post-discharge mortality. ROC analysis for all cause mortality showed AUC for hemoglobin 0.688 (95% CI 0.782−0.594), p < 0.001; and for age 0.735 (95% CI 0.651−0.819) p < 0.001. A group of 59 subjects with hemoglobin < 10.5 g/dL showed mortality rate of 16.9% (OR for mortality 4.19 (95% CI 1.82−9.65), p-value < 0.00, while among 79 patients with Hb > 14.3 g/dL only one death was detected. Interestingly, patients in age > 64 years hemoglobin levels < 13.2 g/dL compared to patients in the same age but with >13.2 g/dL showed OR 3.6 with 95% CI 1.3−10.1 p = 0.012 for death after the discharge. Conclusions: Lower haemoglobin measured in the acute phase especially in patients in age above 64 years showed significant impact on the prognosis and clinical outcomes in PE survivors.

Extrapulmonary Manifestations of SARS-CoV-2: A Report of 3 Cases and a Literature Review.

BACKGROUND COVID-19 disease, caused by the SARS-CoV-2 virus, has been one of the greatest challenges in modern medicine. It is mostly known to affect the pulmonary system, leading to pneumonia and acute respiratory distress syndrome, but there is a growing body of evidence of extrapulmonary manifestations of COVID-19 disease. CASE REPORT This article presents 3 cases of various extrapulmonary symptoms of COVID-19 disease and a literature review of similar clinical cases. Two patients had a medical history of living-donor kidney transplantation, and 1 patient was a kidney donor. We present symptoms, diagnostic processes, laboratory and imaging results, and treatment approach. Patient 1 was 29-year-old woman with new-onset diabetes mellitus due to SARS-CoV-2, which required temporary insulin treatment. Patient 2 was a 34-year-old man with fever, chronic fatigue, back pain, and abdominal pain. Imagining showed acalculous cholecystitis, epiploic appendagitis of the right colic flexure, and inflammation of pericardial fat pad in the left cardiophrenic angle. Coagulopathy due to COVID-19 was the most probable cause of the described processes. Therapeutic doses of low-molecular-weight heparin were administered. Patient 3 was a 68-year-old male kidney donor who had painless, nodular, reddening lesions on both shins, accompanied by itching on both shins and recurrent fever. The diagnosis of erythema nodosum during COVID-19 was made. After treatment with low-molecular-weight heparin, significant decreases of symptoms were observed. CONCLUSIONS We conclude that SARS-CoV-2 infection can have a varied course and can involve other systems and organs. Physicians should be aware of possible extrapulmonary symptoms associated with infection with this virus. Correct diagnosis is a prerequisite for proper treatment and prevention of unexpected complications.

Chest Pain of Atypical Cause in a Young Man.

Granulomatosis with polyangiitis (GPA) is a rare systemic vasculitis that classically affects the upper respiratory tract, lungs, and kidneys. The involvement of other organs occurs but is less frequent. Clinically overt cardiac involvement is rare. We present a rare case of thoracic pain caused by cardiac involvement in GPA, without any other symptoms. The diagnosis was made using an integral approach, with several complementary imaging modalities, including cardiac histology.

The role of peripheral eosinophilia in diagnosing lung disorders: experience from a single pneumonological center.

BACKGROUND: Eosinophilia is rare but one of the important reasons to refer patients to pneumonological centers. Determining etiology of eosinophilia has practical implications for therapeutic intervention and disease prognosis. The study aimed to determine the role of peripheral eosinophilia in the diagnosis of lung disorders. METHODS: In this retrospective study were included 46 patients diagnosed with peripheral eosinophilia with coexisting respiratory symptoms and/or radiologically detected lung lesions. All patients underwent standard diagnostic procedures, including a detailed clinical history review, physical examination, routine laboratory tests with basal cardiological examinations, and serological tests to detect parasites and allergies. Other procedures carried out depended on the symptoms of each patient. The relation between eosinophil counts in the blood and patients' clinical manifestation was investigated to identify the degree of eosinophilia requiring immediate diagnostic procedures and treatment. Statistical analyses were performed using scientific computation libraries in the Python programming language, SciPy, v. 1.3.1. Briefly, the following tests were used: parametric Kruskal-Wallis H test, an independent t-test, ANOVA, the Shapiro- Wilk test, Fisher's and Chi-squared tests, and the Holm-Bonferroni method. RESULTS: Severe eosinophilia (≥5,000 cells/µl) was associated with extrapulmonary involvement and constitutional symptoms. Skin, heart, and pleural diseases were more frequent in these patients than in patients with mild or moderate eosinophilia (p=0.010, p=0.040, and p=0.007, respectively), and only these patients showed signs of kidney disease (p=0.006). Vasculitis was significantly more frequent in the severe eosinophilia group (p=0.048) than in the other two groups. In patients with moderate eosinophilia (1,500-5,000 cells/µl), extrapulmonary symptoms were less common, although signs of cardiac involvement were confirmed in 44% of subjects. In this group, vasculitis was the most commonly observed disease (42% of cases). Mild eosinophilia (<1,500 cells/µl) was mainly associated with airway disease. In this group, vasculitis and interstitial lung diseases were identified, but most were not typically connected with eosinophilia. CONCLUSIONS: Identification of peripheral eosinophilia may essentially determine diagnostic procedures in patients with lung disorders and may be a useful indicator of disease etiology.

Heart of the World's Top Ultramarathon Runner-Not Necessarily Much Different from Normal.

The impact of ultramarathon (UM) runs on the organs of competitors, especially elite individuals, is poorly understood. We tested a 36-year-old UM runner before, 1-2 days after, and 10-11 days after winning a 24-h UM as a part of the Polish Championships (258.228 km). During each testing session, we performed an electrocardiogram (ECG), transthoracic echocardiography (TTE), cardiac magnetic resonance imaging (MRI), cardiac 31P magnetic resonance spectroscopy (31P MRS), and blood tests. Initially, increased cholesterol and low-density lipoprotein cholesterol (LDL-C) levels were identified. The day after the UM, increased levels of white blood cells, neutrophils, fibrinogen, alanine aminotransferase, aspartate aminotransferase, creatine kinase, C-reactive protein, and N-terminal type B natriuretic propeptide were observed. Additionally, decreases in hemoglobin, hematocrit, cholesterol, LDL-C, and hyponatremia were observed. On day 10, all measurements returned to normal levels, and cholesterol and LDL-C returned to their baseline abnormal values. ECG, TTE, MRI, and 31P MRS remained within the normal ranges, demonstrating physiological adaptation to exercise. The transient changes in laboratory test results were typical for the extreme efforts of the athlete and most likely reflected transient but massive striated muscle damage, liver cell damage, activation of inflammatory processes, effects on the coagulation system, exercise-associated hyponatremia, and cytoprotective or growth-regulatory effects. These results indicated that many years of intensive endurance training and numerous UMs (including the last 24-h UM) did not have a permanent adverse effect on this world-class UM runner's body and heart. Transient post-competition anomalies in laboratory test results were typical of those commonly observed after UM efforts.

Cardiac sarcoidosis: worse pulmonary function due to left ventricular ejection fraction?: A case-control study.

Dyspnea and exercise intolerance are usually attributed to pulmonary disease in sarcoidosis patients. However, cardiac involvement may also be responsible for these symptoms. Data regarding the impact of heart involvement on lung function in cardiac sarcoidosis (CS) is limited.The aim of study was to compare the results of pulmonary function tests (PFTs) in patients with and without heart involvement. We performed a retrospective analysis of PFTs in a group of sarcoidosis patients both with and without heart involvement evaluated by cardiovascular magnetic resonance (CMR) study. The study was performed in the period between May 2008 and April 2016.We included data of sarcoidosis patients who underwent testing for possible CS (including CMR study) at a national tertiary referral center for patients with interstitial lung diseases. All patients had histopathologicaly confirmed sarcoidosis and underwent standard evaluation with PFTs measurements including spirometry, plethysmography, lung transfer factor (TL,CO), and 6-minute walking test (6MWT) assessed using the most recent predicted values.We identified 255 sarcoidosis patients (93 women, age 42 ±â€Š10.7 y): 103 with CS and 152 without CS (controls). CS patients had significantly lower left ventricular ejection fraction (LVEF; 56.9 ±â€Š7.0 vs 60.4 ±â€Š5.4, P < .001). Any type of lung dysfunction was seen in 63% of CS patients compared with 31% in the controls (P = .005). Ventilatory disturbances (obstructive or restrictive pattern) and low TL,CO were more frequent in CS group (52% vs 23%, P < .001 and 38% vs 18% P < .01 respectively). CS (OR = 2.13, 95% CI: 1.11-4.07, P = .02), stage of the disease (OR = 3.13, 95% CI: 1.4-7.0, P = .006) and LVEF (coefficient = -0.068 ±â€Š0.027, P = .011) were independent factors associated with low FEV1 but not low TL,CO. There was a significant correlation between LVEF and FEV1 in CS group (r = 0.31, n = 89, P = .003). No significant difference in 6MWD between CS patients and controls was observed.Lung function impairment was more frequent in CS. Lower LVEF was associated with decreased values of FEV1. Relatively poor lung function may be an indication of cardiac sarcoidosis.

Balloon pulmonary angioplasty - efficient therapy of chronic thromboembolic pulmonary hypertension in the patient with advanced sarcoidosis - a case report.

BACKGROUND: Approximately a quarter of patients with advanced sarcoidosis develop pulmonary hypertension (PH), which affects their prognosis. We report unusual case of confirmed chronic thromboembolic pulmonary hypertension (CTEPH) in a patient with stage IV sarcoidosis successfully treated with balloon pulmonary angioplasty (BPA). CASE PRESENTATION: A 65 years old male with a history of colitis ulcerosa, and pulmonary sarcoidosis diagnosed in 10 years before, on long term oral steroids, with a history of deep vein thrombosis and acute pulmonary embolism chronically anticoagulated was referred to our center due to severe dyspnea. On admission he presented WHO functional class IV, mean pulmonary artery pressure (mPAP) in right heart catheterization (RHC) was elevated to 54 mmHg. Diagnosis of CTEPH was definitely confirmed with typical V/Q scan, and with selective pulmonary angiography (PAG) completes by intravascular imagining (intravascular ultrasound, optical coherent tomography). The patient was deemed inoperable by CTEPH team and two sessions of BPA with multimodal approach resulted in significant clinical and haemodynamical improvement to WHO class II and mPAP decrease to 27 mmHg. CONCLUSIONS: Balloon pulmonary angioplasty, rapidly developing method of treatment of inoperable CTEPH patients, is also extremely useful therapeutic tool in complex PH patients.

A case report of steroid resistant cardiac sarcoidosis successfully managed with methotrexate.

Up to one fourth of sarcoidosis patients may have cardiac involvement, what is potentially a life-threatening condition and requires aggressive treatment. Corticosteroids are generally effective in cardiac sarcoidosis, however may have significant short and long term adverse effects. We present a case of a 42-year-old male, who was diagnosed with pulmonary and cardiac sarcoidosis. He was treated initially with corticosteroids and satisfactory improvement was achieved in the lungs but not in the heart. Methotrexate was added as a second line therapy, being beneficial for the heart as well as steroid sparing agent. Cardiac improvement was documented during serial CMR imaging. (Sarcoidosis Vasc Diffuse Lung Dis 2018; 35: 178-181).

Refined balloon pulmonary angioplasty-A therapeutic option in very elderly patients with chronic thromboembolic pulmonary hypertension.

INTRODUCTION/OBJECTIVES: Balloon pulmonary angioplasty (BPA) is a developing treatment for inoperable chronic thromboembolic pulmonary hypertension (CTEPH). However, to our knowledge there are no published data on BPA in CTEPH subjects aged 75 or over. The aim of the study was to analyze clinical and hemodynamic outcomes of sequential BPA in very elderly patients disqualified from pulmonary endarterectomy (PEA). PATIENTS AND METHODS: We enrolled 10 patients (4 male, 6 female, median age 81 [75-88]) with confirmed CTEPH, mPAP > 30 mmHg, and WHO class > II, disqualified from PEA. Overall, 10 patients underwent 39 BPA sessions (mean 3.9 sessions per patient, range 1-9), and 70 pulmonary arteries were dilated, (mean 6.5 vessels per patient, range 1-14). RESULTS: Pulmonary angioplasty resulted in significant clinical and hemodynamic improvement in every patient: 6 MWT distance increased from a median of 221 m (80-320) to 345 (230-455) and plasma NT-proBNP levels decreased (P < 0.01). Sequential BPA resulted in normalization of mPAP (<25 mmHg) in 6 of 10 patients and mPAP decreased to 25-30 mmHg in three others. In the whole group mPAP decreased from 41 (31-53) mmHg to 23 (17-33) mmHg (P < 0.01). Overall, mean PAP and PVR decreased significantly in all cases, while CO and CI increased (P < 0.01). No severe complications occurred during BPA and over a median follow-up of 553 days (range 81-784), and all patients are still alive and in good general health. CONCLUSION: This study demonstrated the safety and efficacy of refined BPA in CTEPH patients aged 75 or over, disqualified from PEA. Refined BPA may emerge as an alternative therapeutic strategy in very elderly CTEPH patients who are suitable for surgery, but this requires further validation in a large prospective study.

Cardiac sarcoidosis - silent destroyer.

We report a case of histologically proven pulmonary sarcoidosis and cardiac involvement in a 53-year old woman with progression leading to the heart failure documented in cardiovascular magnetic resonsnce studies.

Prevalence of cardiac sarcoidosis in white population: a case-control study: Proposal for a novel risk index based on commonly available tests.

Cardiac sarcoidosis (CS) is a life-threatening and underdiagnosed manifestation of the disease, which requires a complicated and expensive diagnostic pathway. There is a need for simple tool for practitioners to determine the risk of CS without access to specialized equipment.The aim of study was to determine the prevalence of CS in a group of patients diagnosed with or followed up because of sarcoidosis. A secondary objective was the search for factors associated with heart involvement.We performed a prospective case-control study (screening analysis) in consecutive sarcoidosis patients collected from October 2012 to September 2015. Cardiac magnetic resonance (CMR) imaging was performed to confirm or exclude cardiac involvement in all patients. The study was conducted in a hospital-based referral center for patients with sarcoidosis and other interstitial lung diseases.Analysis was performed in a group of 201 patients (all white) with biopsy-proven sarcoidosis, mean age 41.4 ±â€Š10.2, 121 of them (60.2%) males. Four patients with previously recognized cardiac diseases, which make CMR imaging for CS inconclusive, were not included.Cardiac involvement was detected by CMR in 49 patients (24.4%). Factors associated with an increased risk of CS (univariate analyses) included male sex (odds ratio [OR]: 2.5; 1.21-5.16, P = 0.01), cardiac-related symptoms (OR: 3.53; 1.81-6.89, P = 0.0002), extrathoracic sarcoidosis (OR: 3.48; 1.77-6.84, P = 0.0003), elevated serum NT-proBNP (OR: 3.82; 1.55-9.42, P = 0.004), any electrocardiography abnormality (OR: 5.38; 2.48-11.67, P = 0.0001), and contemporary radiological progression sarcoidosis in the lungs (OR: 2.98; 1.52-5.84, P = 0.001). Abnormalities in echocardiography and Holter ECG were also risk factors, but not significant in multivariate analyses. A CS Risk Index was developed using a multivariate model to predict CS, achieving an accuracy of 82%, sensitivity of 50%, specificity of 94%, and likelihood ratio 8.1.CS was detected in one fourth of patients. A CS Risk Index based on the results of easily accessible tests is cost-effective and may help to identify patients who should be urgently referred for further diagnostic procedures.

"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.

Cardiac Sarcoidosis: Is it More Common in Men?

INTRODUCTION: Sarcoidosis is a systemic granulomatous disease which predominantly affects the lungs, although granulomas can also involve all other organs, including the heart. Cardiac sarcoidosis (CS) may occur at any stage of the disease and may be the cause of sudden cardiac death, even in a previously asymptomatic patient. The aim of this study was to evaluate the incidence of CS in a large group of patients diagnosed or followed up due to sarcoidosis. METHODS: We performed a retrospective analysis of patients at our institution discharged with the final diagnosis "sarcoidosis" (ICD-10: D86) from January 2008 to October 2012. Only those with biopsy (from respiratory tract or lymph nodes) confirmed diagnosis of sarcoidosis were included. We then selected the subset of patients with cardiac involvement due to sarcoidosis confirmed by positive magnetic resonance imaging. RESULTS: The study covered 1375 consecutive sarcoidosis patients (51 % men), who were hospitalized during 5 years. Multiorgan disease was detected in 160 cases (11.7 %), and cardiac involvement was found in 64 patients (4.7 % of all), 70.3 % of whom were men. Twelve of those with CS were in stage I, 48 in stage II, and four in stage III. The odds ratio for having cardiac involvement in men compared to women was 2.3 (95 % CI 1.36-4.0, p = 0.002). CONCLUSIONS: Cardiac involvement in sarcoidosis was diagnosed in the similar percentage as in previously published data but was significantly more frequently in men.