scholarly journals Long-Term Follow-Up after Intravenous Immunoglobulin Therapy in Patients with Severe Ocular Mucous Membrane Pemphigoid Unresponsive to Conventional Therapy

2018 ◽  
Vol 2018 ◽  
pp. 1-7
Author(s):  
Stefania Leuci ◽  
Massimo Amato ◽  
Elena Calabria ◽  
Raffaele Piscopo ◽  
Fausto Tranfa ◽  
...  

Mucous membrane pemphigoid (MMP) is a heterogeneous group of rare, systemic, autoimmune subepidermal inflammatory disease that affects mucous membranes and the eye. In its most severe forms, this disease needs systemic therapy, usually based on steroids and immunosuppressant agents. In unresponsive cases or in the presence of contraindications or severe side effects due to conventional systemic corticosteroid and/or immunosuppressant therapy, a therapy shift to high-dose intravenous immunoglobulins (IVIg) has been recommended in other reports. This new therapy has proven to be effective in stopping ocular pemphigoid, but the data regarding the long-term effect on the disease activity or reactivation are extremely scarce, so the novel scientific aim of this study was to evaluate the clinical outcomes after a 9-year follow-up in 12 eyes (6 patients) affected by MMP with ocular involvement, successfully treated with IVIg therapy, as previously described in our report published in 2008. The evaluation of ocular and extraocular disease progression was performed at the end of IVIg therapy and at the end of the follow-up period. After 9 years, all the eyes enrolled showed a long-lasting remission of ocular and oral symptoms with a significant steroid-sparing effect. In conclusion, the IVIg has to be considered as a safe and successful alternative therapy in patients with severe ocular mucous membrane pemphigoid; furthermore, this kind of therapy seems to be effective in maintaining the clinical remission by the time.

2014 ◽  
Vol 1 (1) ◽  
pp. 13
Author(s):  
Kazu Ode ◽  
Yoshinori Taniguchi ◽  
Yoshitaka Kumon ◽  
Yoshio Terada

We report a case of successful high-dose intravenous immunoglobulin (IVIG) use in a patient with refractory polyarteritis nodosa (PAN). Treatments with prednisolone (PSL) and various types of immunosuppressants including methotrexate (MTX) and intravenous cyclophosphamide (IVCY) were unsuccessful, and then, high-dose IVIG therapy was added. High-dose IVIG therapy improved all symptoms including high fever, arthralgia, mononeuritis multiplex and indurated erythema due to PAN. Moreover, serum inflammatory markers were also normalized. High-dose IVIG is maintaining complete remission for PAN without flare-up for additional 4 years. Therefore, high-dose IVIG therapy might be considered as a first-line therapy in patients with PAN or alternative therapy in refractory PAN.


2009 ◽  
Vol 4 (1) ◽  
pp. 72
Author(s):  
Vera Bril ◽  

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a significant source of disability, and early diagnosis and immunomodulatory therapy administration are critical to minimise disease progression and axonal degeneration. Intravenous immunoglobulin (IVIg) therapy is considered to be a first-line treatment for CIDP. Comparative short- and long-term data of IVIg versus corticosteroids in CIDP patients are limited. Of the five published placebo-controlled studies in CIDP, four reported only on short-term improvements in disability (≤6 weeks). However, the IGIV CIDP Efficacy (ICE) study, the largest randomised, placebo-controlled CIDP study published to date (n=117), reported significant improvements in disability, functional impairment and quality of life with IVIg (Gamunex®) 1g/kg maintenance therapy every three weeks for up to 48 weeks. Furthermore, long-term IVIg administration was safe and well tolerated, particularly given the short duration of the infusions. Data suggest that a long-term scheduled maintenance regimen of IVIg in appropriate patients may provide substantial benefit and reduce the risk of CIDP relapse.


2018 ◽  
Vol 64 (2) ◽  
pp. 253-255
Author(s):  
Oleg Kit ◽  
Yevgeniy Kolesnikov ◽  
Roman Myagkov ◽  
Leonid Kharin ◽  
Yevgeniya Nepomnyashchaya

Most of melanomas of the gallbladder are metastatic lesions of cutaneous melanoma. Primary melanomas of the gallbladder are described as single, polypoid, intraluminal masses emanating from the mucous membrane. The most important characteristic is the absence of melanoma damage to the skin. If it is not possible to localize primary melanoma a multidisciplinary approach to diagnostic search comes to the fore. Predicting for primary melanoma of the gallbladder is a difficult task due to the small number of cases and the absence of long-term follow-up for this category of patients.


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