Small vessel vasculitis and vasculitis confined to skin

Cutaneous vasculitis is a heterogeneous group of disorders, which can be confined to the skin or may be part of an associated systemic disease. Various aetiological agents as well as conditions that mimic skin vasculitis, usually present with similar clinical features; mainly palpable purpura. The skin biopsies usually show leukocytoclastic vasculitis. This poses a great diagnositc and therapeutic challenge for the physician. The aetiologies, clinical features, diagnosis and treatment modalities for each form (drugs, infections, malignancies, systemic vasculitides, connective tissue disorders. Sch?nlein-Henoch purpura, cryoglobulinaemia, cutaneous periarteritis nodosa, livedoid vasculitis, erythema elevatum diutinum and urticarial vasculitis) are reviewed.     

Pulmonary vasculitis

Pulmonary vascular inflammation may be seen in a variety of primary lung diseases and in the setting of numerous systemic illnesses. This article reviews those entities in which pulmonary vasculitis represents a central feature of the pathologic process (Wegener's granulomatosis, Churg-Strauss syndrome, and pulmonary capillaritis). In addition, features of pulmonary involvement in other systemic vasculitides (Giant Cell Arteritis, Takayasu's Arteritis, and Beh?et's disease) are described. Finally, general principles for the treatment of vasculitis are reviewed.     

Optic disk vasculitis

A 44-year-old woman developed progressive loss of vision associated initially with a swollen optic disk, and later with optic atrophy and a diffuse retinal vasculopathy, which caused extensive retinal hemorrhagagic. Histopathologic examination showed hemorrhagagic infarction of the retina, as well as infarction of the anterior optic nerve. In the optic nerve, the central retinal vessels showed extensive phlebitis and occlusion of many small arterioles.     

Systemic necrotizing vasculitis

The revival of interest in systemic necrotizing vasculitis was initiated by the discovery of its association with anti-neutrophil cytoplasmic antibodies (ANCA). The close association of certain ANCA subspecificities, for example, proteinase 3 (Pr3) and myeloperxoidase ANCA, with Wegener's granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome has led to their designation as 'ANCA-associated vasculitides'. This article describes the common and divergent clinical and immunological features of the members of this 'new' family of systemic necrotizing vasculitis, which continues to grow with the widespread use of ANCA testing. In addition, the 'standard' treatment for systemic necrotizing vasculitis (daily 'low dose' cyclophosphamide plus glucocorticosteroids or 'Fauci's scheme') is compared with new stage and activity adapted therapeutic regimens.     

Pathogenesis of leukocytoclastic vasculitis

A prospective protocol for the management of the airway was applied to patients in the ICU. Acute complications due to intubation and tracheotomy as well as laryngo-tracheal lesions were studied in 125 consecutive patients during one year from the moment of extubation. Thirty four cases (27%) needed reintubation, and 58 tracheotomies were performed (46%). The average length of intubation was of 10 +/- 7 days. Sixty five patients (53%) had acute complications due to intubation and 30 (52%) had acute complications due to the traccotomy. The high incidence of laryngo-tracheal lesions in the 85 patients who underwent early exploration (76 cases [90%]) was reduced in those who underwent late exploration (11 cases [20%]). Analysis of possible prognostic factors in the development of late lesions allows us to affirm that a reduction in the length of intubation diminishes the presence of late lesions. We recommend the avoidance of oro-tracheal intubation prolonged for more than 10 days by the early carrying out of tracheotomy.     

Infections and vasculitis]

Recent interest in case management by elementary and secondary schools has arisen out of a growing recognition that schools alone are not prepared to address the myriad of health, education, and social service needs of a large number of students, particularly children living in poverty. Boys and girls entering classrooms hungry, sick, homeless, or otherwise distracted are not ready to learn. Improving readiness to learn is a major agenda among educators. Case management is one approach many schools are beginning to use to collaboratively engage parents, community service agencies, the private sector, and communities at large in pursuit of the goal. A promising school-based case management model has been developed and successfully field tested by the University of Washington. The model operationally defines case management, delineates a delivery structure, and includes an evaluation design. Results from a five-year study sponsored by the U. S. Department of Education are encouraging. Although several challenges persist, the future for school case management use is optimistic. Several enhanced variations of the Center for the Study and Teaching of At-Risk Students (C-STARS) case management model are now being demonstrated across the country.     

Necrotizing peripheral nerve vasculitis

Human exposure to nonsteroidal estrogens in the environment has recently been proposed as a risk factor for endocrine disruption and the development of cancers of the breast and reproductive tract. Certain polycyclic aromatic hydrocarbons (PAHs), which closely resemble steroid hormones, are ubiquitous environmental contaminants whose carcinogenicity has been extensively studied. This review examines the available evidence regarding the actions of PAHs on estrogen receptor activity, estrogen metabolism and the hypothalamo-pituitary axis. In most studies, PAHs exhibited either weakly estrogenic or antiestrogenic responses. The possibility is raised that the endocrine toxicology of certain PAHs reflects both genotoxic and non-genotoxic components which may be interrelated, particularly with regard to carcinogenesis.     

Treatment of systemic vasculitis

Progress in the treatment of systemic vasculitis have permitted a decrease of mortality but with an increase in iatrogenic morbidity. Steroids remain the cornerstone of the treatment but precise modalities and other concomitant treatments are depending upon the type of vasculitis. In most cases, systemic vasculitis are primary and the treatment, although important, is symptomatic. However, in some cases such as hepatitis B virus-induced polyarteritis nodosa or hepatitis C virus-induced cryoglobulinemia, the treatment can be etiologic and is directed against the antigen responsible for the systemic vasculitis. In the future, a better understanding of pathological mechanisms, particularly of etiologic factors, and new treatment such as monoclonal antibodies should increase the prognosis of systemic vasculitis.     

Pregnancy in granulomatous vasculitis

OBJECTIVE: To study the fetal and maternal outcome of pregnancy in patients with granulomatous vasculitis. METHODS: Four pregnancies in two patients with Wegener's granulomatosis (WG) and one patient with Churg-Strauss syndrome (CSS) were identified and followed in our specialised clinic for pregnancy and connective tissue diseases. RESULTS: Three pregnancies ended with live babies and one with intrauterine death at 25 weeks of gestation. One WG patient remained in remission throughout pregnancy and the other experienced severe activity at 12 weeks. The CSS patient was in remission during her first pregnancy, but the disease flared severely in the second. CONCLUSIONS: Pregnancy in patients with granulomatous vasculitis requires preconceptual planning, careful clinical management, and vigorous treatment of active disease.