Elevated levels of d-dimer are associated with inflammation and disease activity rather than risk of venous thromboembolism in patients with granulomatosis with polyangiitis in long term observation.

PURPOSE: Granulomatosis with polyangiitis (GPA) is one of antineutrophil cytoplasmic autoantibody (ANCA) - associated vasculitis. The disease is characterized by necrotizing inflammation of small vessels causing tissue ischemia in a variety of organs. The aim of the present study was an evaluation of inflammation, coagulation and fibrinolysis biomarkers, and their possible associations with various clinical and laboratory parameters in GPA patients. METHODS: A group of 100 consecutive patients with GPA were prospectively followed in the study. In all patients, echocardiography and laboratory tests were performed. RESULTS: The patients were followed-up for a median of 4.0 ± 1.9 years. Circulating d-dimer concentrations were elevated in a majority (56%) of GPA patients, and were significantly higher in GPA patients in the active stage compared to those in remission (median 652 vs. 405 ng/ml, p = 0.0002). In 23 patients (23%) venous thromboembolism (VTE) was diagnosed during observation. However, there were no differences in d-dimer concentrations between patients with and without VTE either in active stage or in remission. Correlation analysis showed that the levels of d-dimer correlated with hs-CRP (r = 0.42, p < 0.0001) and creatinine concentrations (r = 0.58; p < 0.0001), but not with ANCA levels. CONCLUSIONS: In patients with GPA elevated levels of d-dimer are associated with disease activity and inflammation rather than with the risk of venous thromboembolism. The value of d-dimer as a biomarker of venous thromboembolism episodes in patients with small vessel vasculitis is low.

High incidence of venous thromboembolism but not of coronary artery disease in granulomatosis with polyangiitis in first years after diagnosis.

Objectives: Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis, is one of antineutrophil cytoplasmic autoantibody (ANCA) - associated vasculitis. In patients with GPA an increased incidence of venous thromboembolism (VTE), mainly during active disease, has been described. The aim of the present study was to assess the incidence of VTE and its relation with classic risk factors for atherosclerosis, presence of coronary artery disease (CAD), echocardiographic parameters and laboratory findings in GPA patients. Methods: The group of consecutive patients with GPA were followed in the study. In all patients echocardiography and laboratory tests were performed. Results: Ninety six patients with GPA were followed for mean 3 years. In 16 patients (16.6%) VTEs occurred in association with GPA, of which 56% occurred 6 months before or one year after diagnosis of GPA. Classic risk factors for atherosclerosis were present in 77 patients (80.2%) at some moment during follow-up. In patients with VTE there were larger right ventricle diameter (p=0.041) and higher right ventricle systolic pressure (p=0.022) observed. VTEs occurred significantly less frequently in patients treated with cyclophosphamide (p=0.049). In this study group VTE occurred more frequently than CAD: 16 (16.7%) vs. 4 (4.2%); p=0,0049. Patients with VTE were younger than those with CAD (p=0.053) and had higher levels of ANCA-PR 3 (p=0.016). Conclusions: Patients with granulomatosis with polyangiitis in first years after diagnosis have higher risk of venous thromboembolism than coronary artery disease. This finding is probably related to hypercoagulability induced by the disease and its therapy.

[Emphysematous cystitis in a patient with type-1diabetes mellitus - diagnostic difficulties].

A case report of 28 year old female with medical history of bed controlled type 1 diabetes mellitus complicated by autonomic neuropathy in the form of gastroparesis, suffered by emphysematous cystitis caused by Escherichia coli was described. Emphysematous cystitis is a rare urinary tract infection connected with the presence of gas in the bladder lumen or/and within the bladder wall, which occurs mainly in women, in older age, suffering from type 2 diabetes, complicated by microangiopathy, neuropathy, with urinary tract obstruction and weakness of immunity system. Diagnostic difficulties and the delay in correct diagnosis in described case were caused by the dominated complaint of the upper gastrointestinal tract and difficulties in interpretation of imaging methods, such as abdominal X-ray and ultrasound scan. Eventually the use of computed tomography allowed to achieved an accurate diagnosis and choose appropriate treatment. It is possible that this is the first case of emphysematous cystitis described in Poland.

Cardiac Arrhythmias in Patients with Exacerbation of COPD.

Supraventricular and ventricular arrhythmias are common among patients with chronic obstructive pulmonary disease (COPD). Multiple factors can contribute to the development of arrhythmias in patients with exacerbation of the disease, including: respiratory or heart failure, hypertension, coronary disease and also medications. In the present study we seek to determine the prevalence of cardiac arrhythmias and risk factors among patients with exacerbation of COPD. The study was a retrospective evaluation of 2753 24-h Holter recordings of patients hospitalized in 2004-2016. Exacerbation of COPD was diagnosed in 152 patients and the prevalence of arrhythmias in this group of patients was 97%. The commonest arrhythmia was ventricular premature beats (VPB) - 88.8%, followed by supraventricular premature beats (SPB) - 56.5%. Permanent atrial fibrillation accounted for 30.3% and paroxysmal atrial fibrillation (PAF) for 12.5%. Supraventricular tachycardia (SVT) was noted in 34.2% patients and ventricular tachycardia in 25.6%. Respiratory failure increased the risk of SPB, while heart failure increased the risk of VPB. Treatment with theophylline was associated with a higher proportion of PAF and SVT. In conclusion, COPD exacerbation is associated with a high prevalence of cardiac arrhythmias. COPD treatment and comorbidities increase the risk of arrhythmias.

Echocardiographic Assessment in Patients with Granulomatosis with Polyangiitis.

Granulomatosis with polyangiitis (GPA) is one of the most common forms of systemic vasculitis, which usually involves the upper and lower respiratory tract, but it may affect also multiple organs. The aim of the study was an echocardiographic evaluation of cardiac involvement in GPA patients during remission. Eighty eight patients with GPA were evaluated in the study. The control group consisted of 40 age and sex-matched patients without a previous history of cardiovascular disease. We found that there were no differences between GPA and control groups regarding left atrial enlargement and interventricular septal hypertrophy. In one GPA patient, all heart chambers were enlarged. Left ventricle systolic function was decreased (LVEF ≤ 50%) in eight patients with GPA, and left ventricle wall motion abnormalities were observed in 12 patients. Left ventricle relaxation dysfunction, mitral valve and tricuspid valve regurgitation were observed with the same frequency in both GPA and control groups. Aortic regurgitation was the single abnormality that occurred significantly more often in the GPA group than in controls (28% vs. 7.5%; p = 0.03). Pericardial effusion was observed in three GPA patients and in none from the control group. We conclude that the most common echocardiographic manifestation in GPA patients in remission was aortic valve regurgitation. However, cardiac involvement in such patients is rather rare and in the majority of cases clinically insignificant.

[Von Meyenburg complexes. case report].

Von Meyenburg complexes is one of the polycystic liver diseases, characterized by bile duct hamartoma. These cysts come from the biliary tract but the cysts do not communicate with them. Because of asymptomatic course of the lesions usually are diagnosed in the course of diagnostic for another reason. It is not possible to define the entire diagnosis based upon ultrasonography imaging, as cyst could mimic metastasis, micro-abscesses and multiple focal nodular lesions. Because of the small size of the lesion (0.5-15 mm) usually inconclusive is also computed tomography. On the basis of magnetic resonance imaging (MRI) and cholangio-MRI we can determine the diagnosis of the complexes. Liver biopsy is obligatory in case of suspicion of neoplastic process. These complexes do not require treatment, but long-term follow-up is indicated because of the possibility to more frequent cholangiocarcinoma in patient with von Meyenburg complexes. It is probably the first case report of the von Meyenburg complexes described in Poland.