Al-Johani, K.; (2010) Outcomes of therapy of immunologically-mediated diseases of the oral mucosa. Doctoral thesis , UCL (University College London).

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Abstract

Immune-mediated diseases (IMDs) can give rise to long standing painful oral mucosal disease which adversely affect oral function and perhaps lessens quality of life. The present series of studies, retrospectively determine the clinical presentation and long-term efficacy and safety of treatment of large groups of patients with oral lichen planus, mucous membrane pemphigoid, pemphigus vulgaris and orofacial granulomatosis. These diseases are some of the challenging disorders to be managed by oral medicine specialists. It was found that patients with oral lichen planus (OLP) rarely have extra-oral manifestations of LP. The symptoms of OLP can generally be controlled with topical corticosteroids and/or tacrolimus. While tacrolimus is not notably better than topical corticosteroids for the management of OLP, it does not seem to increase any risk of malignant transformation. Adverse side effects are uncommon with topical corticosteroids, while 21% of patients with OLP may have adverse side effects with tacrolimus, particularly unpleasant taste. In the present cohort of 49 patients with orofacial granulomatosis (OFG) the onset of disease was characterised by facial swelling in 50% and the long-term behaviour of OFG was characterised by the development of further clinical manifestations with most patients developing orofacial swelling and/or intra-oral ulceration. The response of OFG to therapy was typically remitting and although a lessening of soft tissue swelling oral ulceration could generally be achieved with topical and/or systemic therapy. Complete remission of facial swelling occurred in 50% of patients within 3 years of therapy but may be achieved quicker when intra-lesional corticosteroids are used. Spontaneous remission was rare. Significant adverse side effects to therapy were rare. In a cohort of 62 patients, mucous membrane pemphigoid typically manifested as recurrent oral mucosal ulceration and/or desquamative gingivitis and 32.3% patients had some extra-oral involvement. Treatment generally lessened painful symptoms however gingival lesions rarely resolved. Adverse side effects affected 50% of patients; however in the majority of affected individuals these were minor. In a cohort of 40 patients with pemphigus vulgaris the mouth was often the initial site of involvement but other mucocutaneous sites could be affected. Management necessitated topical and systemic therapy. Adverse side effects occurred in 50% patients and were mainly associated with systemic immunosuppressive agents (e.g. azathioprine). The results of this present study indicate that the long-term treatment of IMDs of the oral mucosal are challenging to both the patients and clinicians. While many patients do experience an improvement in their disease status, many do not. The precise impact of IMDs upon the quality of life of affected individuals remains unclear.

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