Treatment of systemic manifestations

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Especially in the young male, where the prognosis, is the worst, it is important to start treatment as early as possible. Efficacy of this approach has formally been shown for eye disease (Hamuryudan et al., 1997) and in the management of pulmonary artery aneurysms (Hamuryudan et al., 2004).

Azathioprine has been formally shown that to be effective in controlling eye disease and some of the extraocular manifestations including skin-mucosa disease, in a double-blind placebo controlled study. Recommended dose of azathioprine is 2.5mg/kg/day (Yazici et al., 1990).

Cyclosporin-A, a noncytotoxic immunosupress-ant agent is probably still the most rapidly effective agent in controlling eye inflammation. It is found superior to monthly pulses of cyclophosphamide in a single-blind study (Ozyazgan et al., 1992). It induces anti-inflammatory effect at the dosage of 2-5 mg/kg/day. The long-term use of cyclosporin is limited by the development of side effects, particularly hypertension and renal impairment. It has also been reported to have a favorable effect on mucocutaneous disease, thrombophebilitis and systemic symptoms. It was reported in a retrospective survey that the incidence of neurological disease was significantly higher in patients receiving cyclos-porin and cyclosporine-associated side effects could not be differentiated from CNS involvement (Kotake et al., 1999).

We commonly treat severe eye disease with a combination of azathioprin and cyclosporin A mainly using the latter as a remisson inducing agent and the former as the maintenance drug (Yazici et al., 1999).

Interferon alpha is widely used for the uveitis of BS (Kotter et al., 2003). It is also beneficial for the extraocular manifestations of the disease, although less so for oral aphthous ulcers (Kotter et al., 2004).

Infliximab, in uncontrolled studies, has been shown to have a beneficial effect in BS not only in eye disease (Sfikakis, 2002; Ohno et al., 2004; Lindstedt et al., 2005) but on the extraocular manifestations as well (Haugeberg et al., 2004; Sarwar et al., 2005).

Oral and intravenous cyclophosphamide either as single agent or combination with high-dose corticosteroids has been used in patients with systemic vasculitis (pulmonary arterial involvement) and parenchymal CNS involvement (Yazici et al., 2002; Kantarci and Siva, 2003; Hamuryudan et al., 2004).

In brief within the last decades substantial progress has been made in managing BS. Patients with eye disease, pulmonary artery aneurysms and skin-mucosa disease are with little doubt doing better when compared to 20 years ago.

On the other hand, the management of thrombophilia and that of CNS disease are the two main problem areas due to the lack of reported formal clinical trial data.

Key points

• Epidemiology: Prevalence of BS 38-42/ 104 in Turkey. 1/104 in Japan, lower in the West. Distrubution seems to follow the ancient Silk Road.

• Demographics: It is rare before puberty and after the sixth decade. It affects both genders equally but young male patients have more severe disease.

• Etiopathogenesis: BS is a complex multisystem inflammatory disorder of unknown etiology. HLA B51 has been the most consistently reported HLA-associa-tion in the disease. The unifying feature of the inflammation observed in BS is a nonspecific inflammatory hyperreactivity—a phenomenon known as 'path-ergy'—with vasculitis as its main clinical consequence. Thl-mediated immune response seems to be responsible for tissue damage.

• Clinical symptoms: Typically manifests with recurrent oral and genital ulcerations and uveitis, variably accompanied by symptoms affecting skin, the large vessels, gastrointestinal and CNS, or other organs.

• Pathology: BS is a systemic vascular disease, affecting blood vessels of all size. A nonspecific inflammatory hyperreactivity, mainly neutrophilic, with or without vasculitis is its main consequence.

• Prognosis and treatment: In many patients disease abates with the passage of time. Mucocutaneous lesions can often be managed symptomatically. However, if they become severe enough to interfere with quality of life, then systemic therapy is required. Certain manifestations, including ocular, neurological and large vessel involvement are indications for aggressive therapy, especially in the young males in whom the mortality and morbidity are raised especially in early disease. Preliminary experience indicates TNF inhibition might be helpful in disease control.


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Handbook of Systemic Autoimmune Diseases, Volume 5 The Skin in Systemic Autoimmune Diseases

Piercarlo Sarzi-Puttini, Andrea Doria, Giampiero Girolomoni and Annegret Kuhn, editors


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