Article

Intravenous immunoglobulin therapy in patients with multiple mucosal involvement in mucous membrane pemphigoid.

Center for Blistering Diseases, New England Baptist Hospital, Boston, MA 02115, USA.
Clinical Immunology (Impact Factor: 3.77). 02/2002; 102(1):59-67. DOI: 10.1006/clim.2001.5150
Source: PubMed

ABSTRACT Mucous membrane pemphigoid (MMP), also known as cicatricial pemphigoid (CP), is an autoimmune mucocutaneous, blistering disease which can lead to blindness and/or death from sudden asphyxiation, secondary to a scarring process. Conventional therapy for the treatment of MMP consists of high-dose systemic corticosteroids and/or immunosuppressive agents. Some patients do not respond to these treatments and develop multiple serious side effects, which can be potentially fatal. In such patients, alternative treatment modalities are needed. This study presents the use of intravenous immunoglobulin (IVIg) therapy in 15 patients with severe MMP whose disease was nonresponsive to the prolonged use of high-dose systemic corticosteroids and immunosuppressive agents and who developed multiple side effects to them. All 15 patients received an IVIg dose of 1-2 g/kg/cycle. The following objective parameters were used to assess the clinical outcome pre- and post-IVIg therapy: number of side effects, frequencies of recurrences and relapses, duration and total dosage of prednisone therapy, and the quality of life. The differences in these variables between the pre- and post-IVIg data were statistically analyzed using the SAS UNIVARIATE software running the two-sided Wilcoxon signed-rank and sign tests. A statistically significant difference was observed between pre- and post-IVIg therapy data when comparing the aforementioned variables. All 15 patients had an effective clinical response, were able to discontinue previous systemic therapies, and eventually achieved a prolonged clinical remission. IVIg improved the quality of life in all 15 patients and demonstrated a steroid-sparing effect. No serious side effects were observed. IVIg therapy is a safe and effective alternative modality in the treatment of patients with nonresponsive and progressive MMP and can induce a sustained clinical remission.

0 Bookmarks
 · 
48 Views
  • [Show abstract] [Hide abstract]
    ABSTRACT: Elderly individuals are susceptible to autoimmune bullous dermatoses (in particular, pemphigoid, epidermolysis bullosa acquisita and paraneoplastic pemphigus). Bullous dermatoses are associated with high morbidity and mortality. Bullous dermatoses result from autoimmune responses to one or more components of the basement membrane or desmosomes. Pemphigoid results from autoimmunity to hemidesmosomal proteins present in the basement membrane of stratified squamous epithelia. Patients present with tense blisters in flexural areas of the skin. Mild or moderate bullous pemphigoid may be treated with potent topical corticosteroids while extensive disease usually requires systemic corticosteroids or systemic immunosuppressive agents such as azathioprine. Mucosal pemphigoid affects one or more mucous membranes that are lined by stratified squamous epithelia. The two most commonly involved sites are the eye and the oral cavity. Lesions frequently result in scar formation, which may cause blindness. Patients with severe disease or ocular involvement require aggressive therapy with corticosteroids and cyclophosphamide. Epidermolysis bullosa acquisita results from autoimmunity to type VII collagen in the anchoring fibrils of the basement membrane area. Lesions may either arise on an inflammatory base or be non-inflammatory and result primarily from trauma. The inflammatory type of the disease is more responsive to therapy than the non-inflammatory type. Treatment options include corticosteroids, dapsone, cyclosporin, plasmapheresis and immunoglobulin G. Paraneoplastic pemphigus results from autoimmunity to multiple antigens within the desmosomes. The disorder is associated with neoplasms, especially leukaemia and lymphoma. Patients present with severe stomatitis and polymorphous skin eruption. The mucosal and cutaneous involvement may respond to successful treatment of the underlying neoplasm or may require immunosuppressive therapy.
    Drugs & Aging 02/2003; 20(9):663-81. · 2.50 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Mucous membrane pemphigoid (MMP) is a heterogeneous group of autoimmune subepithelial blistering diseases affecting primarily mucous membranes showing marked degree of clinical and immunological variability. We investigated four controversial topics: (i) Does oral pemphigoid (OP) really exist as a separate entity? (ii) Is mucous membrane pemphigoid curable? (iii) What is the best therapeutic option for MMP? (iv) Does exclusive oral IgA dermatitis exist as a distinct entity from MMP? Results from extensive literature searches suggested that (i) it is still unclear whether patients with OP could be considered as a distinct subset of MMP with specific clinical and immunological features; (ii) it is uncertain whether treatment regimens that get MMP under control can be eliminated to allow patients to be in drug-free remission or they should be continuously administered in some capacities; (iii) there is a concerning paucity of good-quality trials on MMP and available recommendations are solely based on generally small patients' cohorts or case series. Some of the 2002 consensus experts' opinions should be possibly updated, particularly regarding the safety of sulfa drugs; (iv) we did not find any strong evidence to support an exclusive oral (and perhaps also mucosal) form of LAD as a separate entity.
    Oral Diseases 01/2014; 20(1):35-54. · 2.38 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Intravenous immunoglobulin (IVIG) is an expensive biologic agent used to treat patients with mucous membrane pemphigoid (MMP) nonresponsive to conventional immunosuppressive therapy (CIST). The high cost of IVIG is of concern to healthcare providers and insurance companies. To compare the cost of IVIG with that of CIST in treating a cohort of 15 patients with severe and extensive MMP. Fifteen patients with biopsy-proven MMP nonresponsive to CIST were subsequently treated with IVIG and demonstrated a positive clinical response. This was a comparative, retrospective study; the mean total duration of the observation period was 8.4 years. A comparison of the cost of IVIG with that of CIST during the study period and the annual cost was performed. The cost of CIST was defined as the actual cost of the drug plus the cost of management of the multiple adverse effects, including hospitalizations, produced by CIST. In the same patient cohort, no significant adverse effects to IVIG were observed and no hospitalizations were required. Hence, the cost of IVIG therapy is simply the actual cost of the IVIG. In this cohort of patients, CIST had significant adverse effects, many of which were hazardous and required prolonged and frequent hospitalizations. The mean total cost using IVIG therapy was significantly less than that of CIST during the entire course of the disease (p < 0.001) and on an annual basis (p < 0.05). IVIG therapy is a safe, clinically beneficial, and less-expensive alternative treatment in patients with progressive MMP that is nonresponsive to CIST.
    Annals of Pharmacotherapy 01/2006; 39(12):2003-8. · 2.57 Impact Factor