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Biomedical subjects

C Stephen Foster

Publications and source records attributed to C Stephen Foster.

At least 37 records · Page 2Linked to original sources
PubMed2005

Ahmed valve implantation for uncontrolled pediatric uveitic glaucoma.

PURPOSE: We sought to evaluate the safety and efficacy of Ahmed valve implantation for the management of glaucoma associated with chronic uveitis in pediatric patients. METHODS: A retrospective chart review was conducted of 6 pediatric patients (7 eyes) who underwent Ahmed valve implantation because of refractory uveitic glaucoma. Intraocular pressure (IOP) reduction, preoperative and postoperative visual acuities, the number of hypotensive medications required, and complications associated with the operation were evaluated. RESULTS: Mean follow-up was 36.8 months (range, 6-60). At the last visit, all 7 eyes had IOPs between 9 and 18 mm Hg (average, 12.1). IOP reduction averaged 69.6% (P = 0.0005, paired t-test). The number of hypotensive agents was reduced from an average of 3 to an average of 0.71 medicines per eye (P = 0.001). The only complication was hemorrhagic choroidal detachment postoperatively in two eyes; both resolved within one month. CONCLUSIONS: For children with good immunomodulatory control of their inflammation and appropriate follow-up, Ahmed valve implantation can be an effective and safe procedure for treating pediatric uveitic glaucoma.

Adolescent↗
PubMed2005

Combined pars plana vitrectomy and phacoemulsification to restore visual acuity in patients with chronic uveitis.

PURPOSE: To report the outcomes of combined phacoemulsification and pars plana vitrectomy (PPV) to restore visual acuity in patients with cataract and posterior segment involvement secondary to chronic uveitis. SETTING: Ocular Immunology and Uveitis Service, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts, USA. METHODS: This study comprised 34 patients (20 women, 14 men; 36 eyes) with posterior segment involvement secondary to chronic uveitis who had combined phacoemulsification and PPV from 1998 to 2002. The main outcome measures were visual acuity, intraocular pressure, and cystoid macular edema. RESULTS: The mean patient age was 45 years +/- 16.09 (SD). The mean duration of uveitis before surgery was 56 +/- 44.17 months. In 24 eyes (66.7%), an intraocular lens (IOL) was implanted during surgery; 12 eyes (33.3%) were left aphakic. Five eyes (13.8%) received an intraocular steroid injection intraoperatively. Visual acuity improved in 26 eyes (72.2%), deteriorated in 5 (13.9%), and was unchanged in 5 (13.9%). The main reason for decreased visual acuity was refractory macular edema. During the follow-up, 2 IOLs were explanted secondary to lens intolerance. One IOL was repositioned because of iris capture by the haptics, and 1 dislocated inferiorly, causing monocular diplopia. The mean follow-up was 23.4 +/- 16.7 months. CONCLUSIONS: Results indicate that combined phacoemulsification and PPV is a feasible technique for the removal of cataract and pathologic vitreous in eyes with chronic uveitis. Although the exact role of vitrectomy in patients with uveitis remains to be determined, the combined surgery successfully restored useful vision in most cases.

Adolescent↗
PubMed2005

Initial evaluation of subcutaneous daclizumab treatments for noninfectious uveitis: a multicenter noncomparative interventional case series.

PURPOSE: To assess the feasibility of a study design that may determine whether subcutaneous administration of the interleukin-2 receptor antibody daclizumab can safely reduce the dependence on standard systemic corticosteroids or other immunosuppressive regimens in patients with sight-threatening, noninfectious intermediate uveitis, posterior uveitis, or panuveitis. DESIGN: Prospective, multicenter, nonrandomized, noncomparative, open-label interventional trial. PARTICIPANTS: Fifteen patients, 5 each at 3 clinical centers, with noninfectious intermediate, posterior, or panuveitis, who require a currently stable immunosuppression regimen of systemic corticosteroids and/or other systemic treatments to control noninfectious intraocular inflammation. METHODS: After enrollment and baseline ophthalmic evaluations, 2 induction treatments were given 2 weeks apart using subcutaneous (SC) daclizumab at 2 mg/kg. Subcutaneous daclizumab maintenance treatments were then continued every 2 weeks at 1 mg/kg for 6 months. The initial immunosuppression load was tapered over 8 to 12 weeks in a staggered fashion beginning with the first induction treatment. Safety evaluations were performed at each treatment visit, with a primary efficacy evaluation at 12 weeks and a repeat efficacy evaluation at 26 weeks. MAIN OUTCOME MEASURES: Best-corrected visual acuity (Early Treatment of Diabetic Retinopathy Study [ETDRS] method) with a concurrent taper of concomitant systemic immunosuppression medication load (tabulated by use of a weighted scoring system) was assessed; target for success was defined as a 50% or greater reduction in concomitant immunosuppression load by 12 weeks while maintaining visual acuity within 5 ETDRS letters of baseline. Ocular inflammation was assessed at each visit with standardized grading scales. RESULTS: Ten of 15 patients (67%) receiving SC daclizumab treatments every other week successfully achieved the primary efficacy end point of reducing their concomitant immunosuppression load by at least 50% while maintaining their baseline visual acuity at 12 and 26 weeks. Subcutaneous daclizumab injections were well tolerated with no serious adverse events observed during the 6 months of treatments, although 3 patients experienced possibly related, nonserious adverse events. CONCLUSIONS: Subcutaneous daclizumab induction treatments at 2 mg/kg followed by 1 mg/kg maintenance treatments every other week seems safe and, in most cases, may reduce the concomitant immunosuppressive load required to treat noninfectious uveitis for 12 to 26 weeks.

Adolescent↗
PubMed2005

Long-term follow-up of patients with birdshot retinochoroidopathy treated with corticosteroid-sparing systemic immunomodulatory therapy.

PURPOSE: To evaluate the outcomes of patients with birdshot retinochoroidopathy (BSRC) treated with corticosteroid-sparing systemic immunomodulatory therapy (IMT). DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS: Thirty-five patients with BSRC evaluated at the Ocular Immunology and Uveitis Service of the Massachusetts Eye and Ear Infirmary from 1980 through 2003. METHODS: Data on age, gender, follow-up time, delay to diagnosis or referral, treatment before and during follow-up, complications of BSRC or treatment, Snellen visual acuities (VAs), and electroretinograms (ERGs) were recorded from patient charts. MAIN OUTCOME MEASURES: Disease progression as determined by Snellen VAs and serial ERGs, ocular complications of BSRC or corticosteroids, and complications of systemic IMT. RESULTS: Twenty-eight patients with a mean follow-up of 81.2 months were included. None of the patients had sufficient control of their inflammation before referral. All patients were treated with corticosteroid-sparing systemic IMT at some point during their follow-up: 92.9% were treated with cyclosporine, 67.9% with mycophenolate mofetil, 17.9% with azathioprine, 10.7% with oral methotrexate, and 7.1% with daclizumab. Ocular complications of BSRC and/or corticosteroids were cataract (53.6%), cystoid macular edema (35.7%), glaucoma (21.4%), epiretinal membrane (10.7%), and retinal detachment (3.6%). Average Snellen VAs at the time of initial visit were 0.64 (right eye) and 0.59 (left eye). Average final Snellen VAs were 0.74 (right eye) and 0.71 (left eye). (Logarithm of the minimum angle of resolution equivalents were -0.23, right eye initial; -0.19, right eye final; -0.38, left eye initial; and -0.31, left eye final.) In the right eye, 78.6% of patients and, in the left eye, 89.3% of patients had either the same or improved VA at the end of the follow-up. The 30-hertz flicker implicit time was prolonged in 58.3% of initial ERGs and in 62.5% of final ERGs. The bright scotopic amplitude was abnormal in 45.5% of initial and final ERGs. CONCLUSIONS: Long-term preservation of visual function is attainable with systemic corticosteroid-sparing IMT for patients with BSRC. Prompt treatment with systemic IMT may offer the best hope of maintaining retinal function in what is often thought of as a chronically progressive disease resistant to treatment.

Adult↗
PubMed2005

Discoid lupus erythematosus masquerading as chronic blepharoconjunctivitis.

PURPOSE: To recognize discoid lupus erythematosus (DLE) as a treatable cause of chronic blepharoconjunctivitis. DESIGN: Retrospective observational case series. PARTICIPANTS: Records of 5 patients with biopsy-proven DLE were reviewed. METHODS: Clinical and pathology records were examined. MAIN OUTCOME MEASURES: Patients' clinical and histopathological characteristics and response to treatment were assessed. RESULTS: Clinical features included meibomian gland dysfunction, blepharitis, chalazia, trichiasis, madarosis, conjunctivitis, chronic eyelid edema, and eyelid plaques. Histopathology showed hyperkeratotic epithelium, degeneration of the basal cell layer, and a perivascular lymphocytic infiltrate. There was delayed diagnosis in all cases, ranging from 4 months to 25 years. All of the patients responded to systemic hydroxychloroquine therapy. CONCLUSIONS: Heightened awareness of eyelid DLE may lead to earlier detection and specific therapy for this chronic disorder.

Adult↗
PubMed2005

Analysis of pediatric uveitis cases at a tertiary referral center.

OBJECTIVE: To analyze demographics, anatomic data, diagnoses, systemic associations, and visual outcomes of pediatric patients in a large tertiary eye center. DESIGN: Retrospective cohort study. METHODS: The records of 1242 patients with uveitis referred to the Ocular Immunology and Uveitis Service of the Massachusetts Eye and Ear Infirmary (MEEI) from 1985 to 2003 were reviewed retrospectively. Two hundred sixty-nine patients 16 years and younger were identified. RESULTS: Among 269 children with uveitis, 53.5% were girls, 82% were Caucasian, and 82% were born in the United States. Mean age was 8 years (standard deviation, 3.8; range, 1-16). Anterior uveitis represented 56.9% of cases; intermediate, 20.8%; panuveitis, 16%; and posterior, 6.3%. Nongranulomatous (77.6%) and noninfectious (85.7%) were the most frequent types of inflammation. The process was bilateral in 74.4% of patients. Mean follow-up was 22 months, with mean age of 8 years at diagnosis. Mean duration of uveitis at the time of presentation at the MEEI was 2 years. The range of time between the diagnosis of uveitis and referral was 1 day to 5.6 years. The length of time between diagnosis of uveitis and the referral to the tertiary center strongly correlated with the complication rate and degree of visual impairment in our study. The longer the time before the patients were seen by the uveitis expert, the worse the visual outcomes. No systemic associations were found in 58% of patients, juvenile idiopathic arthritis was responsible for 33% of cases, 8% of patients had other systemic associations, and 1% had tubulointerstitial nephritis uveitis syndrome. CONCLUSIONS: Uveitis remains a serious cause of morbidity and visual loss in children. Timely referral to uveitis specialists in the tertiary referral centers may lead to improved visual outcomes in children with chronic uveitis.

Acute Disease↗
PubMed2005

Stevens-Johnson syndrome and toxic epidermal necrolysis: a review of the literature.

OBJECTIVE: To perform a comprehensive review of Stevens-Johnson syndrome and toxic epidermal necrolysis. DATA SOURCES: A MEDLINE search was performed for the years 1975 to 2003 using the keywords Stevens-Johnson syndrome and toxic epidermal necrolysis to identify relevant articles published in English in peer-reviewed journals. STUDY SELECTION: All clinical studies that reported on 4 or more patients, review articles, and experimental studies that concerned disease mechanisms were selected and further analyzed. Clinical reports that included fewer than 4 patients were selected only if they were believed to carry a significant message about disease mechanism or therapy. RESULTS: Stevens-Johnson syndrome and toxic epidermal necrolysis seem to be variants of the same disease with differing severities. A widely accepted consensus regarding diagnostic criteria and therapy does not exist at present. Despite the recent experimental studies, the pathogenic mechanisms of these diseases remain unknown. Although progress in survival through early hospitalization in specialized burn units has been made, the prevalence of life-long disability from the ocular morbidity of Stevens-Johnson syndrome and toxic epidermal necrolysis has remained unchanged for the past 35 years. Further progress depends on modification of the acute phase of the disease rather than continuation of supportive care. The available published evidence indicates that a principal problem in the pathogenesis is immunologic and that immunomodulatory intervention with short-term, high-dose intravenous steroids or intravenous immunoglobulin holds the most promise for effective change in survival and long-term morbidity. CONCLUSIONS: The results of this review call for a widely accepted consensus on diagnostic criteria for Stevens-Johnson and toxic epidermal necrolysis and multicenter collaboration in experimental studies and clinical trials that investigate disease mechanisms and novel therapeutic interventions, respectively.

Adolescent↗
PubMed2005

Masquerade scleritis.

PURPOSE: To report two cases in which malignancy masqueraded as scleritis, delaying the diagnosis. METHODS: Two patients initially diagnosed and treated for unilateral scleritis were referred for management of persistent inflammation. Additional evaluation uncovered underlying malignant processes. RESULTS: The first patient presented with scleritis initially responsive to systemic corticosteroids, with relapse one month later. Upon referral, peripheral fundus examination revealed elevated lesions. Additional studies confirmed the diagnosis of choroidal melanoma. The patient was treated with proton-beam irradiation. The second patient developed necrotizing scleritis unresponsive to systemic steroids, methotrexate, and cyclophosphamide. A scleral biopsy disclosed an undifferentiated high-grade carcinoma, likely metastatic. Exenteration was performed. CONCLUSIONS: Scleritis can present a diagnostic challenge. It is often the sole initial manifestation of an occult systemic problem. Treatment-resistant scleritis should raise the suspicion of an infectious or malignant masquerade.

Adenocarcinoma↗
PubMed2005

Eosinophil activation in Wegener's granulomatosis: a harbinger of disease progression?

PURPOSE: To investigate the relation between eosinophil activation in tissue from patients with an active, limited form of Wegener's granulomatosis (WG) affecting the eye and subsequent systemic disease activity. METHODS: Analysis of ocular specimens obtained from 10 patients was performed. Sections were probed with antibodies to assess the presence of major basic protein (MBP) and eosinophil cationic protein (ECP). RESULTS: Four of the 10 specimens demonstrated the presence of MBP and ECP. WG progressed to the complete form in two of these patients, who received no or inadequate treatment. The other two, treated with cyclophosphamide for one year, did not progress to the complete form of WG during observation after therapy. CONCLUSIONS: Activated eosinophils in sclera or conjunctiva of patients with ocular limited WG may predict progression to complete WG.

Adult↗
PubMed2005

Somatostatin for Uveitic Cystoid Macular Edema (CME).

PURPOSE: To report the favorable response of bilateral recalcitrant uveitic cystoid macular edema (CME) to treatment with a somatostatin analog. METHODS: Medical ophthalmic history and the results of ophthalmic examinations were recorded. Fluorescein angiography (FA) studies were reviewed. RESULTS: A 52-year-old white female with intermediate uveitis developed bilateral recalcitrant CME. Treatment with subcutaneous injections of the somatostatin analog octreotide resulted in partial resolution of the CME and improvement of visual acuity. CONCLUSIONS: Somatostatin may play a role in the treatment of CME secondary to uveitis.

Antineoplastic Agents, Hormonal↗
PubMed2005

Mucous membrane pemphigoid: an update.

PURPOSE OF REVIEW: To review articles on mucous membrane pemphigoid, published between June 2004-May 2005. RECENT FINDINGS: Decreased glycosylation of mucin was found in patients with ocular cicatricial pemphigoid. A unique antigen in oral mucous membrane pemphigoid has not yet been identified. Increased vascular cell adhesion molecule and intercellular adhesion molecule 1 expression was found in skin of patients affected by mucous membrane pemphigoid. Autoreactive T cells to an epitope of bullous pemphigoid antigen 180 kilodaltons were identified in the blood of some patients with mucous membrane pemphigoid. Circulating IgA against an antigen in mucous membrane pemphigoid was found in about 20% of patients, without prognostic significance. Enhanced sensitivity for direct immunofluorescence was reported if skin biopsy specimens were stored for 24 hours in saline. An enzyme-linked immunosorbent assay for detection of circulating autoantibodies against laminin-5 was developed. Sensitivity was higher than indirect immunofluorescence on salt-split skin and immunoblotting. Patients with younger onset (<60 years) of ocular cicatricial pemphigoid were found to have disease evolution similar to that of an older group (>70 years) but were visually impaired earlier in life. Intravenous immunoglobulin as treatment of ocular cicatricial pemphigoid was found to be superior to conventional immunosuppressants, with fewer side effects and better long-term outcome for halting disease activity. Intraoperative adjunction of mitomycin C during fornix reconstruction with amniotic membrane resulted in achieving a deeper fornix in 83% of patients with various cicatrizing conjunctivitis. Transplantation of cultured epithelial cells of oral mucosa in corneal limbal stem cell deficiency was successful in improving visual acuity and reestablishing corneal transparency in mid- to advanced ocular cicatricial pemphigoid. SUMMARY: Further advances have been achieved in the field of mucous membrane pemphigoid.

Autoantibodies↗
PubMed2005

Valsalva retinopathy: diagnostic challenges in a patient with pars-planitis.

PURPOSE: To describe a case of Valsalva retinopathy in a patient with pars-planitis and to discuss its management. METHODS: We present a case of a 19-year-old male with a six-month history of bilateral intermediate uveitis, referred for evaluation of recent-onset spontaneous bilateral vitreous hemorrhages. Clinical evaluation and fluorescein angiography were negative for the presence of neovascularization. On closer questioning the patient revealed that he had his most recent symptoms after heavy weight lifting. RESULTS: The patient was instructed to avoid heavy weight lifting. Over the next two months, vitreal hemorrhages had resolved. However, despite the treatment with systemic prednisone, the patient's intermediate uveitis was still active, and cystoid macular edema had developed. Therefore, we proceeded to systemic immunomodulatory therapy, which controlled the inflammation and preserved vision in each eye. CONCLUSIONS: Although a very rare cause, Valsalva maneuver should be included in the differential diagnosis of bilateral vitreous hemorrhages. Ophthalmologists should advise patients with known eye problems that can predispose to vitreal hemorrhages to refrain from it.

Adult↗
PubMed2004

A nonrandomized comparison of the clinical outcome of ocular involvement in patients with mucous membrane (cicatricial) pemphigoid between conventional immunosuppressive and intravenous immunoglobulin therapies.

The purpose of this study was to compare the clinical outcomes of intravenous immunoglobulin (IVIg) therapy to conventional immunosuppressive therapy in patients with mucous membrane pemphigoid (MMP), also known as cicatricial pemphigoid (CP), whose disease progressed to involve the eye. Before ocular involvement, all the patients in this study were diagnosed and treated with immunosuppressive agents, for biopsy-proven MMP, affecting the skin and/or mucous membranes, other than the conjunctiva. Eight patients in group A were treated with IVIg after the diagnosis of ocular cicatricial pemphigoid (OCP) was established. The efficacy and safety of IVIg therapy were compared to a clinically similar group of eight patients treated with conventional immunosuppressive therapy (group B). The inclusion criteria for both groups were: (1). presence of MMP at extraocular sites confirmed by biopsy before entry into the study; (2). entry into the study occurred when ocular involvement was noted and confirmed by biopsy; (3). presence of conventional immunosuppressive therapy at the time of ocular involvement; (4). a minimum of 18 months of follow-up after diagnosis of ocular involvement. The mean length of the therapy, after the onset of ocular involvement, was 24 months (range 16-30) in group A and 45 months (range 21-90) in group B. The median time between initiation of therapy and clinical remission in group A and group B was 4 and 8.5 months, respectively. This difference was statistically significant (P < 0.01). No recurrence of ocular inflammation was recorded in any of the patients in group A. On the contrary, at least one recurrence (median 1) was recorded in five patients in group B (range 0-4). This difference was statistically significant (P < 0.05). All eight patients in group A and group B presented to the ophthalmologist in stage 2 of OCP at the time of the initial visit. At the last follow-up visit, no progression to advanced stages of OCP was recorded in all eight patients in group A. On the contrary, only four patients in group B remained in stage 2 of OCP at the last follow-up exam. The conjunctival scaring progressed from stage 2 to stage 3 in the remaining four patients of group B. At the last follow-up visit, both eyes of each patient in group A were free of inflammation. Some level of conjunctival inflammation at the last follow-up visit was noted in five patients in group B (range 0-1.5, P < 0.05). Both groups of patients were studied during the same time period. The results of this study suggest that ocular involvement in patients with MMP may be considered an indication for initiating IVIg therapy, since it was more effective in arresting progression of OCP, when compared to conventional immunosuppressive therapy. These data indicate that IVIg produced a faster control of the acute inflammation and that no recurrences were observed during the follow-up. This clinical difference could be because of the reduced production of pathogenic antibody, and/or restoration of the immunoregulation, which may have been disturbed.

Aged↗
PubMed2004

Inhibition of MMP-dependent chemotaxis and amelioration of experimental autoimmune uveitis with a selective metalloproteinase-2 and -9 inhibitor.

The chemotaxis of inflammatory cells depends on proteolytic disruption of extracellular matrix components. The metalloproteinases (MMP)-2 and -9 enable T-lymphocytes to pass through basement membranes. Selective inhibition of only MMP-2 and -9 almost completely abolished the ability of lymphocytes to digest collagen. The chemotaxis of lymphocytes was reduced 40%. In our in-vivo model of experimental autoimmune uveitis (EAU), 46% of the animals in the treated group remained disease-free, whereas all animals in the control group developed EAU. The mean activity of the disease was also statistically significantly reduced. The data suggest that selective MMP-2 and -9 inhibition might be a treatment option.

Amino Acid Motifs↗
PubMed2004

Pulse IV cyclophosphamide in ocular inflammatory disease: efficacy and short-term safety.

PURPOSE: To assess the efficacy and short-term safety of appropriately monitored pulse IV cyclophosphamide therapy in the treatment of patients with severe or treatment-resistant autoimmune ocular inflammatory disease. DESIGN: Retrospective noncomparative interventional case series. PARTICIPANTS: Thirty-eight patients with severe or recalcitrant ocular inflammation of diverse etiologies. METHODS: Charts of patients seen on the Ocular Immunology & Uveitis Service at the Massachusetts Eye & Ear Infirmary were reviewed. Thirty-eight consecutive patients treated with pulse IV cyclophosphamide between January 1995 and March 2002 were analyzed. MAIN OUTCOME MEASURES: The control of inflammation, steroid-sparing effect, visual acuity, and adverse reactions. RESULTS: A positive response to treatment occurred in 68% of patients during the study period, with 55% achieving complete quiescence. A steroid-sparing effect was achieved in all patients previously on systemic steroid, allowing successful discontinuation of the drug in 41%. Visual acuity was maintained in 66% and improved in 21% of involved eyes. The most common side effects observed were fatigue (63%), nausea (32%), and headache (22%). None required a permanent discontinuation of therapy. CONCLUSIONS: Pulse IV cyclophosphamide is an effective therapeutic modality in patients with severe or treatment-resistant ocular inflammatory disease.

Adolescent↗
PubMed2004

Intravenous immunoglobulin therapy in patients with ocular-cicatricial pemphigoid: a long-term follow-up.

OBJECTIVE: To report the clinical outcome and long-term follow-up of 10 patients with progressive ocular-cicatricial pemphigoid (OCP), nonresponsive to conventional therapy and treated with IV immunoglobulin (IVIg) therapy, reported earlier as a preliminary study. DESIGN: Noncomparative, prospective, interventional case series according to a defined protocol for IVIg therapy. PARTICIPANTS: Ten patients, with a diagnosis of OCP present bilaterally confirmed by both biopsy and immunofluorescence studies and who had failed conventional therapy and had objectively demonstrated a positive response to IVIg therapy in a preliminary study, published in 1999. MAIN OUTCOME MEASURES: Comparison of objective clinical outcome parameters before and after IVIg therapy, including visual acuity (VA) and prevention of progression of subepithelial conjunctival fibrosis and blindness. RESULTS: All 10 patients initially demonstrated signs of clinical improvement with IVIg therapy. The total number of IVIg cycles ranged from 20 to 42 (mean, 32), and the total duration of IVIg therapy ranged from 25 to 43 months (mean, 35). Eight patients who completed the protocol had an improvement in their VA and did not have further progression of subepithelial conjunctival fibrosis. These 8 patients have been maintained in a sustained remission for a total follow-up period ranging from 24 to 48 months (mean, 35) after the discontinuation of IVIg therapy. Two patients did not complete the protocol. Both had initially demonstrated a positive clinical response. One patient had worsening of the OCP and, after IVIg therapy, was abruptly and involuntarily withdrawn. In the second patient, deterioration occurred after ocular surgery. Intravenous immunoglobulin therapy was not provided postoperatively. These 2 patients who did not complete the protocol lost vision. CONCLUSIONS: Intravenous immunoglobulin therapy is an effective treatment in OCP in patients nonresponsive to conventional therapy. In 8 patients who completed the protocol, progression of the disease was not observed. A gradual withdrawal of IVIg therapy, as described in the protocol, may be beneficial in maintaining a sustained clinical remission. Abrupt cessation or discontinuation can result in a severe recurrence that may possibly progress to blindness.

Aged↗