Ocular cicatricial pemphigoid, keratomycosis, and intravenous immunoglobulin therapy.

Cornea. 2004 Nov;23(8):819-22.

• Iaccheri B,
• Roque M,
• Fiore T,
• Papadaki T,
• Mathew B,
• Baltatzis S,
• Emara B,
• Tokarewicz AC,
• Foster CS.

Ocular Immunology and Uveitis Service, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA 02114, USA.

PURPOSE: To report the case of a patient developing fungal keratitis in the context of uncontrolled ocular cicatricial pemphigoid (OCP), which, despite intravenous immunoglobulin (IVIg) and other immunomodulatory therapy, progressed to end-stage pemphigoid, with corneal opacification, ankyloblepharon, and xerosis. Keratoprosthesis (KPro) restored functional vision for the patient. METHODS: A 39-year-old man presented with uncontrolled CP and corneal ulcer in the left eye. Conjunctival biopsy diagnosed OCP; corneal scraping and biopsy diagnosed the cause of the corneal ulcer. OCP was treated with systemic steroids, immunosuppressive drugs, and IVIg. Visual rehabilitation was accomplished with Ahmed valve and a type II Dohlman KPro. RESULTS: Immunohistology of the biopsied conjunctiva showed IgG at the epithelial basement membrane zone, confirming the clinical diagnosis of OCP. Microbiologic studies of the corneal biopsy specimen were negative for Acanthamoeba and herpes but positive for Aspergillus niger. The patient's keratomycosis resolved with topical antifungal therapy. Treatment with Dapsone, intravenous-pulse steroid, oral cyclophosphamide, and intravenous immunoglobulin (IVIg) failed to control the OCP, with resultant complete conjunctivization of the cornea. Keratoprosthesis improved the patient's visual acuity from hand movements to 20/20. CONCLUSIONS: Patients with uncontrolled OCP are at increased risk of corneal infection. The difficulty in diagnosing keratomycosis and the relatively rare occurrence of OCP explain the uniqueness of our reported case. OCP may progress to "end-stage" disease despite therapy. Keratoprosthesis can restore vision in selected otherwise seemingly hopeless cases.

PMID: 15502484 [PubMed - indexed for MEDLINE]

Visual outcome in herpes simplex virus and varicella zoster virus uveitis: a clinical evaluation and comparison.

Ophthalmology. 2002 Aug;109(8):1532-7.

• Miserocchi E,
• Waheed NK,
• Dios E,
• Christen W,
• Merayo J,
• Roque M,
• Foster CS.

Immunology and Uveitis Service, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston 02114, USA.

PURPOSE: To compare clinical characteristics and outcomes in patients with uveitis caused by herpes simplex virus (HSV) and varicella zoster virus (VZV). DESIGN: Retrospective comparative study. PARTICIPANTS: Forty patients with HSV uveitis and 24 patients with VZV uveitis. METHODS: A retrospective study of 40 patients with HSV and 24 patients with VZV uveitis was performed. The patients were followed between May 1987 and September 1999 (median follow-up time, 46 months). The diagnosis of HSV uveitis was made clinically and serologically, and the diagnosis of VZV uveitis was made clinically. MAIN OUTCOME MEASURES: Clinical presentation of the disease, ocular complications, visual acuity, surgical and medical treatments needed. RESULTS: Both populations were comparable for gender and age at disease onset. The course of the disease tended to be remitting and recurrent in HSV patients and chronic in VZV patients (P = 0.046). The most frequent ocular complication in both groups was secondary glaucoma (54% HSV, 38% VZV). Twenty-five percent of VZV patients developed posterior pole complications (cystoid macular edema, epiretinal membrane, papillitis, retinal fibrosis, and detachment) compared with 8% of HSV patients (P = 0.069). Treatment modalities selected were generally similar in the two groups, although periocular and systemic steroids were required more frequently in HSV patients (60% versus 25%; P = 0.01). Surgical procedures were required with similar frequency in both populations. The percentage of eyes that were legally blind at end of follow-up was also comparable (HSV, 20%; VZV, 21%). The visual outcome was similar in the studied populations. CONCLUSIONS: This study represents the only direct comparison of HSV and VZV uveitis patients reported in the literature. HSV patients were more likely to be treated with periocular and systemic steroids, and VZV patients were more likely to develop posterior pole complications (a finding of borderline significance). Other parameters evaluated in this study were not statistically different in the two patient groups.

PMID: 12153807 [PubMed - indexed for MEDLINE]

Efficacy and safety of chlorambucil in intractable noninfectious uveitis: the Massachusetts Eye and Ear Infirmary experience.

Ophthalmology. 2002 Jan;109(1):137-42.

• Miserocchi E,
• Baltatzis S,
• Ekong A,
• Roque M,
• Foster CS.

Immunology and Uveitis Service, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, 243 Charles Street, Boston, MA 02114, USA.

PURPOSE: To report our experience with the use of chlorambucil for otherwise treatment-resistant uveitis and to assess its safety and efficacy. DESIGN: Noncomparative interventional case series. PARTICIPANTS: Twenty-eight patients with intractable noninfectious uveitis. METHODS: We reviewed the records of 28 patients (56 eyes) with chronic noninfectious uveitis who were treated with chlorambucil from 1987 to 2000. Diagnoses included Adamantiades-Behcet's disease (ABD) (7 patients), juvenile rheumatoid arthritis (JRA)-associated uveitis (10 patients), pars planitis (2 patients), sympathetic ophthalmia (1 patient), idiopathic uveitis (6 patients), Crohn's disease (1 patient), and HLA-B27-associated uveitis (1 patient). All patients were refractory to other immunomodulatory therapy and systemic steroids. The median duration of treatment with chlorambucil was 12 months (range, 4-50 months), whereas the median daily dosage was 8 mg (range, 4-22 mg). Patients were followed for a median follow-up period of 46 months (range, 4-166 months) after chlorambucil treatment was begun and continued to be followed for relapse after cessation of therapy. MAIN OUTCOME MEASURES: Visual outcome, response to treatment, treatment-related side effects, drug dosage, previous and final treatment, discontinuation of systemic corticosteroids. RESULTS: Chlorambucil was discontinued in seven patients because of side effects: two females had temporary amenorrhea develop, two patients had unacceptable gastrointestinal intolerance, one patient had infection, and 2 patients had progressive leukopenia. Nineteen patients (68%) showed positive clinical response to the treatment, four (14%) initially responded then relapsed after discontinuation of the drug, three patients with ABD had improvement of ocular disease but worsening of systemic symptoms, and two had persistent inflammation. Visual acuity was improved in 24 eyes (43%), stable in 22 (39%), and worsened in 10 eyes (18%). Systemic prednisone was successfully discontinued in 19 of the 28 patients (68%), and 14 patients were free of inflammation at the end of follow-up without any systemic medication. CONCLUSIONS: Chlorambucil can be a safe and effective alternative for preserving vision in patients with otherwise treatment resistant uveitis.

PMID: 11772593 [PubMed - indexed for MEDLINE]

The effect of treatment and its related side effects in patients with severe ocular cicatricial pemphigoid.

Ophthalmology. 2002 Jan;109(1):111-8.

• Miserocchi E,
• Baltatzis S,
• Roque MR,
• Ahmed AR,
• Foster CS.

Immunology and Uveitis Service, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, 243 Charles St., Boston, MA 02114, USA.

PURPOSE: To determine the clinical outcome of patients with ocular-cicatricial pemphigoid (OCP) and the influence of systemic treatment on clinical progression. DESIGN: Noncomparative interventional case series. PARTICIPANTS: Sixty-one patients with biopsy-proven OCP. METHODS: Patients with documented disease progression treated with chemotherapy and/or corticosteroids were followed between 1985 and 2000. The parameters evaluated were ocular stage at presentation, visual acuity, ocular complications, disease progression, control of ocular inflammation, and presence of extraocular involvement. Systemic treatment and related side effects were analyzed. MAIN OUTCOME MEASURES: Visual acuity, ocular complications, extraocular involvement, disease progression, clinical outcome, systemic treatment, and related side effects. RESULTS: Sixty-one patients (32 female; 29 male) with a mean age of 67 years were studied. Extraocular involvement was present in 50% of patients. Sixty percent of eyes were initially seen with stage III (advanced cicatrizing) disease at first evaluation. Seven percent of involved eyes at first visit and 21% at the end of follow-up were legally blind. The most common ocular complications encountered were dry eye, corneal abnormalities, and glaucoma. Dapsone was the most commonly used drug (51 patients), followed by methotrexate (24 patients), azathioprine (23 patients), and cyclophosphamide (15 patients); prednisone, always given as adjunctive treatment, was used in 17 patients. Control of ocular inflammation (total or partial) was achieved in 90% of patients, but 46% of them needed continuation of systemic treatment to avoid disease recurrences, and 10% progressed despite different drugs used. Two agents were required in 32% of cases to control disease activity. The most common treatment-related side effects were hematologic complications (n = 34) followed by gastrointestinal (n = 17), cardiovascular (n = 15), and urinary complications (n = 11). Dapsone was responsible for the greatest number of side effects (n = 43); methotrexate caused the least trouble (n = 6). Corticosteroid-related complications (n = 34) were mostly cardiovascular and endocrinologic. CONCLUSIONS: Ocular-cicatricial pemphigoid is an autoimmune disease that, untreated, progresses to conjunctival scarring and blindness; systemic immunosuppression is required to control it. Long-term systemic treatment and more than one drug are frequently necessary to avoid recurrences, exposing elderly patients to a higher risk of drug toxicity. The most frequently encountered treatment-related side effects were anemia, leukopenia, liver toxicity, and hypertension.

PMID: 11772589 [PubMed - indexed for MEDLINE]

Eye involvement in autoimmune blistering diseases.

Clin Dermatol. 2001 Nov-Dec;19(6):742-9. Review. No abstract available.

• Ekong AS,
• Foster CS,
• Roque MR.

Ocular Immunology and Uveitis Service, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts 02114, USA. Tonyekong@aol.com

PMID: 11705684 [PubMed - indexed for MEDLINE]