Biomedical subjects

P Fietta

Publications and source records attributed to P Fietta.

22 records · Page 2Linked to original sources

PubMedDate not supplied

Systemic vasculitides: immunogenetics and familial clustering.

The systemic vasculitides (SV) are a heterogeneous group of rare affections characterized by a primary process of inflammation and damage of the blood vessel wall. Their etiopathogenesis is still unknown, but a complex interaction of multiple factors, such as age, sex, ethnic background, immunogenetic mechanisms and environmental influences, is probably involved. A genetic predisposition to SV is suggested by both familial case clusters and immunogenetic studies. The available reports on familial SV via the PubMed (National Library of Medicine) and Biosis indices, as well as personal observations, are summarized here. Furthermore, the evidence for a role of genetic predisposing factors is reported. The literature review suggests that several SV such as giant cell arteritis, Takayasu arteritis, Kawasaki disease, Wegener's granulomatosis and Henoch-Schönlein purpura, are governed by multiple genes encoding host defence molecules and probably triggered by environmental agents. Genetic factors seem to be implicated not only in the susceptibility, but also in the severity and outcome of SV.

Family Health↗

PubMedDate not supplied

Behçet's disease: familial clustering and immunogenetics.

Behçet's disease (BD) is a relapsing, multisystemic inflammatory disorder, characterized by major symptoms consisting of recurrent orogenital ulcerations, eye and skin lesions. Other clinical features may include musculoskeletal, vascular, gastrointestinal, renal, cardiopulmonary or neurological involvement. Vasculitis affecting all types and sizes of blood vessels is the main histopathologic process, in a third of cases complicated by thrombosis. The etiopathogenesis is presently unknown, but BD likely represents the result of a peculiar immune response to hitherto unidentified environmental factors in genetically predisposed subjects. The prevalent distribution in a specific geographical area spanning the Mediterranean basin and Asia, the close association with human leukocyte antigen B*51 in different ethnic groups, and the familial clustering of BD are hallmarks accounting for the strong contribution of a genetic background. The BD familial aggregation is characterized by both genetic anticipation and higher prevalence in childhood patients, likely defining a subset with stronger immunogenetic influences. Polymorphisms in genes encoding for host effector molecules may have a supplementary role in disease susceptibility and/or severity. The contribution of prothrombotic mutations and polymorphisms in the pathogenesis of BD thrombosis is controversial. In this paper, the available reports on BD familial clustering and the evidence for the role of immunogenetic predisposing factors are reviewed.

Behcet Syndrome↗

PubMedDate not supplied

Respiratory system involvement in systemic vasculitides.

The respiratory system may be involved in all systemic vasculitides (SV), although with a variable frequency. Lung disease is a very common and important feature of the antineutrophil cytoplasmic antibodies (ANCA)-associated SV (AASV), such as Wegener's granulomatosis (WG), Churg-Strauss syndrome (CSS), and microscopic polyangiitis (MPA). In WG, almost all patients have either upper airway or lower respiratory tract disease. Solitary or multiple nodules and masses are the most common findings on chest radiograph. Asthma is a cardinal symptom of CSS, often preceded by allergic rhinitis, frequently complicated by nasal polyposis and sinusitis. Pulmonary transient and patchy alveolar infiltrates are the most common radiographic findings. In MPA, diffuse alveolar hemorrhage (DAH) due to alveolar capillaritis is the most frequent manifestation of the respiratory involvement, clinically expressing with hemoptysis, respiratory distress and anemia. However, DAH may be subclinical and has to be suspected when chest radiograph demonstrates new unexplained bilateral alveolar infiltrates, in the face of falling hemoglobin levels. In giant cell arteritis, the most frequent respiratory symptom is cough, usually non-productive, persistent, and responsive to corticosteroids. In Takayasu arteritis, pulmonary involvement is frequently subclinical and detectable by non-invasive techniques. Pulmonary involvement is rare in polyarteritis nodosa, Kawasaki disease, Henoch-Schönlein purpura and cryoglobulinemic vasculitis. In conclusion, the involvement of the respiratory system is a very common and important feature of AASV, whereas is less frequent in other SV. It comprises a wide spectrum of clinical features and radiological findings, and may have a prognostic significance. The assessment of the respiratory system should be included in the work-up of all patients with SV, especially of those with AASV.

Antibodies, Antineutrophil Cytoplasmic↗

PubMedDate not supplied

Hematologic manifestations of primary Sjögren's syndrome.

Sjögren's syndrome (SS) is a chronic autoimmune disorder, primarily characterized by the mononuclear cell infiltration of exocrine glands exiting in parenchymal damage and secretory impairment. The spectrum of the disease extends from an autoimmune exocrinopathy to a systemic process with extraglandular manifestations. SS is defined as primary (pSS) when isolated, or secondary when associated with another autoimmune disease. Patients with pSS may present hematologic abnormalities, such as anemia, hemocytopenias, monoclonal gammopathies and lymphoprolipherative disorders, predominantly non-Hodgkin's lymphoma of B-cell origin. The increased prevalence of B-cell malignancies suggests that SS may be a boundary disease between autoimmunity and lymphoproliferation. In this paper, the hematologic manifestations of pSS are reviewed.

Autoimmunity↗