Search

We present a case of a 57-year-old woman suffering from granulomatosis with polyangiitis (GPA), who in the seventh months of immunosuppressive treatment (cyclophosphamide) progressed with new pulmonary changes and perforations of the hard palate and bronchi. Rituximab was introduced resulting in B-cell depletion and disappearance of anti-PR3 antibody. Palatal holes have substantially diminished and all bronchial perforations disappeared, covered by fibrous tissue. In the fourth month after rituximab administration, large scarring obstruction of the right main bronchus with upper and middle lobes atelectasis emerged. Because of increasing dyspnoea, stenotic bronchus was re-opened by bronchoscopy. Intervention was complicated by bilateral pneumothorax and later, on the seventh day, by fatal pulmonary bleeding. To our knowledge, this is the first report of GPA refractory to cyclophosphamide complicated by palatal and bronchial perforations.

The aim of the study was to evaluate the presence and spectrum of cardiac abnormalities identified by cardiac magnetic resonance (CMR) in subjects in clinical remission of Churg-Strauss syndrome (CSS) and granulomatosis with polyangiitis (Wegener's) (WG) with normal ECG and transthoracic echocardiography (TTE). Eleven (7 females, 4 males, mean age 42.4 ± 9.6 years) CSS and 10 (4 females, 6 males, mean age 45.3 ± 10.9 years) WG patients in clinical remission with normal ECG and TTE underwent CMR. Segmental peak-systolic myocardial strain (εps) was measured using feature tracking cine-sequence based technique. Left ventricular (LV) ejection fraction, end-diastolic volume and myocardial mass indexes were 66.2 ± 5.8 %, 66.1 ± 6.6 ml/m(2), and 61.0 ± 8.9 g/m(2), respectively. No patient showed regional wall motion abnormalities and signs of myocarditis. Nine CSS and 8 WG patients demonstrated decreased segmental longitudinal, circumferential or radial εps and myocardial late gadolinium enhancement (LGE) (6 subendocardial, 10 midwall, 8 subepicardial) areas. In CSS and WG subjects with LVLGE lesions the mean LVLGE extent was 2.0 ± 1.6 % and 2.3 ± 1.5 % (p = 0.65), respectively. Segmental εps was decreased longitudinally (-11.8 ± 5.6 %) for subendocardial LGE, radially (13.7 ± 8.7 %) for subepicardial LGE, and circumferentially (-16.6 ± 4.2 %), longitudinally (-13.2 ± 5.5 %) and radially (18.8 ± 8.1 %) for midwall LGE, if compared to longitudinal (-22.7 ± 5.1 %), circumferential (-23.6 ± 5.6 %) and radial (34.2 ± 15.7 %) εps in controls (11 females, 10 males, mean age 43.9 ± 10.5 years) (all p < 0.01). Despite clinical remission, normal ECG and TTE, most CSS and WG patients demonstrate decreased segmental εps and non-ischemic LGE lesions without signs of myocarditis.

Background: Two-dimensional speckle-tracking echocardiography (STE) is a novel technique providing accurate assessment of myocardial function. However, its value in granulomatosis with polyangiitis (Wegener's) (WG) has not been studied., Objective: To assess the presence and frequency of systolic left ventricular (LV) dysfunction using STE and to determine incremental value of STE over standard echocardiography to detect myocardial abnormalities in WG., Methods: Twenty-two WG patients (11 males, 11 females, mean age 46.8 ± 12.3 years) and 22 sex- and age-matched healthy subjects underwent standard and STE. Global longitudinal, circumferential, and rotational deformation parameters were calculated., Results: All patients had LV ejection fraction (EF) >50%. LVEF was 65.0 ± 7.5% and LV end-diastolic volume index 44.8 ± 11.8 mL/m(2) . Regional LV wall motion abnormalities were found in 7 (32%), while abnormal global STE determined systolic dysfunction in 16 (73%) subjects (P = 0.008). Global longitudinal, circumferential and radial peak-systolic deformational parameters (strain or strain rate) were decreased in 11 (50%), 9 (41%), and 3 (14%) patients (P = 0.02), respectively. Comparing patients with abnormal and normal STE derived global systolic function, the former had higher cumulative disease extent index (10.6 ± 3.0 vs 7.5 ± 1.8; P = 0.03) and vasculitis damage index (7.9 ± 1.9 vs 6.0 ± 1.7; P = 0.04)., Conclusions: Despite normal LVEF the global systolic LV abnormalities detected by STE are common in WG. They correspond to the extent and severity of WG and are more frequent than regional wall motion abnormalities in standard echocardiography., (© 2012, Wiley Periodicals, Inc.)

Objectives: The aim of the study was to assess cardiac involvement in patients with Wegener's granulomatosis (WG), who failed to achieve remission following >6 months induction therapy for life or organ threatening disease., Methods: Eleven WG patients (eight males, mean age 47 ± 13 years), who failed to achieve remission despite >6 months induction therapy, underwent transthoracic echocardiography (TTE) and cardiac magnetic resonance (CMR)., Results: Cardiac involvement was present in 9 (82%) patients. Regional wall motion abnormalities were found in two individuals, but none had left ventricular (LV) ejection fraction <50%. Nine patients had late gadolinium enhancement (LGE) lesions involving LV myocardium and right ventricle free wall was involved in four patients. LGE lesions were found in subepicardial, midwall and subendocardial LV myocardial layers. CMR revealed myocarditis in six patients. Patients with myocarditis had a higher number of LV segments with LGE (5.2 ± 3.4 vs 1.0 ± 1.2, p = 0.03) and more frequent diastolic dysfunction by TTE (5 vs 0, p = 0.02) than those without. Pericardial effusion was observed in five patients, while localized pericardial thickening in six patients., Conclusions: In WG resistant to >6 months induction therapy cardiac involvement is frequent and is characterized by foci of LGE lesions and signs of myocardial inflammatory process.