Long-Term Follow-Up after Intravenous Immunoglobulin Therapy in Patients with Severe Ocular Mucous Membrane Pemphigoid Unresponsive to Conventional Therapy
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...
Saved in:
| Main Authors: | , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2018-01-01
|
| Series: | Journal of Ophthalmology |
| Online Access: | http://dx.doi.org/10.1155/2018/8372146 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1832568229725208576 |
|---|---|
| author | Stefania Leuci Massimo Amato Elena Calabria Raffaele Piscopo Fausto Tranfa Gianrico Spagnuolo Michele Davide Mignogna |
| author_facet | Stefania Leuci Massimo Amato Elena Calabria Raffaele Piscopo Fausto Tranfa Gianrico Spagnuolo Michele Davide Mignogna |
| author_sort | Stefania Leuci |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-9360362b83c449debba47a1618f22871 |
| institution | Kabale University |
| issn | 2090-004X 2090-0058 |
| language | English |
| publishDate | 2018-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Journal of Ophthalmology |
| spelling | doaj-art-9360362b83c449debba47a1618f228712025-02-03T00:59:27ZengWileyJournal of Ophthalmology2090-004X2090-00582018-01-01201810.1155/2018/83721468372146Long-Term Follow-Up after Intravenous Immunoglobulin Therapy in Patients with Severe Ocular Mucous Membrane Pemphigoid Unresponsive to Conventional TherapyStefania Leuci0Massimo Amato1Elena Calabria2Raffaele Piscopo3Fausto Tranfa4Gianrico Spagnuolo5Michele Davide Mignogna6Oral Medicine Complex Unit, Department of Neuroscience, Reproductive and Odontostomatological Sciences, University “Federico II” of Naples, Naples, ItalyDentistry Unit, Department of Medicine, Surgery and Dentistry, University of Salerno, Fisciano, ItalyOral Medicine Complex Unit, Department of Neuroscience, Reproductive and Odontostomatological Sciences, University “Federico II” of Naples, Naples, ItalyEye Center, Humanitas Research Hospital, Rozzano Milano, ItalyOrbital Unit, Department of Neuroscience, Reproductive and Odontostomatological Sciences, University “Federico II” of Naples, ItalyOral Medicine Complex Unit, Department of Neuroscience, Reproductive and Odontostomatological Sciences, University “Federico II” of Naples, Naples, ItalyOral Medicine Complex Unit, Department of Neuroscience, Reproductive and Odontostomatological Sciences, University “Federico II” of Naples, Naples, ItalyMucous 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.http://dx.doi.org/10.1155/2018/8372146 |
| spellingShingle | Stefania Leuci Massimo Amato Elena Calabria Raffaele Piscopo Fausto Tranfa Gianrico Spagnuolo Michele Davide Mignogna Long-Term Follow-Up after Intravenous Immunoglobulin Therapy in Patients with Severe Ocular Mucous Membrane Pemphigoid Unresponsive to Conventional Therapy Journal of Ophthalmology |
| title | Long-Term Follow-Up after Intravenous Immunoglobulin Therapy in Patients with Severe Ocular Mucous Membrane Pemphigoid Unresponsive to Conventional Therapy |
| title_full | Long-Term Follow-Up after Intravenous Immunoglobulin Therapy in Patients with Severe Ocular Mucous Membrane Pemphigoid Unresponsive to Conventional Therapy |
| title_fullStr | Long-Term Follow-Up after Intravenous Immunoglobulin Therapy in Patients with Severe Ocular Mucous Membrane Pemphigoid Unresponsive to Conventional Therapy |
| title_full_unstemmed | Long-Term Follow-Up after Intravenous Immunoglobulin Therapy in Patients with Severe Ocular Mucous Membrane Pemphigoid Unresponsive to Conventional Therapy |
| title_short | Long-Term Follow-Up after Intravenous Immunoglobulin Therapy in Patients with Severe Ocular Mucous Membrane Pemphigoid Unresponsive to Conventional Therapy |
| title_sort | long term follow up after intravenous immunoglobulin therapy in patients with severe ocular mucous membrane pemphigoid unresponsive to conventional therapy |
| url | http://dx.doi.org/10.1155/2018/8372146 |
| work_keys_str_mv | AT stefanialeuci longtermfollowupafterintravenousimmunoglobulintherapyinpatientswithsevereocularmucousmembranepemphigoidunresponsivetoconventionaltherapy AT massimoamato longtermfollowupafterintravenousimmunoglobulintherapyinpatientswithsevereocularmucousmembranepemphigoidunresponsivetoconventionaltherapy AT elenacalabria longtermfollowupafterintravenousimmunoglobulintherapyinpatientswithsevereocularmucousmembranepemphigoidunresponsivetoconventionaltherapy AT raffaelepiscopo longtermfollowupafterintravenousimmunoglobulintherapyinpatientswithsevereocularmucousmembranepemphigoidunresponsivetoconventionaltherapy AT faustotranfa longtermfollowupafterintravenousimmunoglobulintherapyinpatientswithsevereocularmucousmembranepemphigoidunresponsivetoconventionaltherapy AT gianricospagnuolo longtermfollowupafterintravenousimmunoglobulintherapyinpatientswithsevereocularmucousmembranepemphigoidunresponsivetoconventionaltherapy AT micheledavidemignogna longtermfollowupafterintravenousimmunoglobulintherapyinpatientswithsevereocularmucousmembranepemphigoidunresponsivetoconventionaltherapy |