SERPINA1 Gene Variants in Granulomatosis with Polyangiitis.

Alpha-1 antitrypsin (A1AT) deficiency is one of the most common genetic disorders in Caucasian population. There is a link between granulomatosis with polyangiitis (GPA) and most frequent variants of SERPINA1 gene encoding severe alpha-1 antitripsin deficiency. However, the potential effect of Pi*Z, Pi*S as well as other SERPINA1 variants on clinical course of vasculitis are not well understood.

Peripheral Arterial Tonometry in Pulmonary Vasculitis.

Vascular wall inflammation in primary vasculitides results in diminished vessel dilation and finally impaired blood flow, causing multiple organs dysfunction and ultimate damage. In granulomatosis with polyangiitis (GPA), the inflammatory process concerns small and medium sized vessels and its pulmonary location is often predominant. The pivotal role in the development of that pathology plays vascular endothelium.

Clinical Manifestations of Huge Diaphragmatic Hernias.

Translocation of abdominal organs into the thoracic cavity may cause dyspnea, heart disorders, and gastric symptoms. Diaphragmatic hernias can cause diagnostic difficulties, since both clinical and radiological symptoms might imitate different disorders. In these cases computed tomography of the chest is the method of choice.

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.

Lung Lesions During Fever of Unknown Origin.

Fever of unknown origin (FUO) remains one of the most difficult diagnostic challenges. The causes of FUO can be various diseases located in different organs. The aim of the study was to determine the prevalence and nature of pulmonary lesions during FUO.