Biomedical subjects

Stefanos Baltatzis

Publications and source records attributed to Stefanos Baltatzis.

18 recordsLinked to original sources

PubMed2006

Mooren ulcer: an immunopathologic study.

PURPOSE: To determine the pattern and distribution of mononuclear cells, adhesion, and co-stimulatory molecules in the conjunctiva of patients with Mooren ulcer. METHODS: Conjunctival biopsy specimens were obtained from 6 patients with Mooren ulcer and 6 healthy individuals. Immunohistochemistry was performed on frozen sections of the cryopreserved human conjunctivas using monoclonal antibodies directed against CD1alpha, CD3, CD4, CD8, CD20, CD25, CD57, and CD68 cells; the adhesion molecules E-selectin, vascular cell adhesion molecule-1 (VCAM-1), very late activation-4 (VLA-4), ICAM-1, and LFA-1; and the co-stimulatory molecules CD28, B7-1, B7-2, and CTLA-4. RESULTS: Differences in expression on the conjunctival epithelium from patients with Mooren ulcer and normal subjects were noted only for VCAM-1, VLA-4, ICAM-1, and LFA-1. The ratio of CD4+/CD8+ cells in Mooren ulcer specimens was significantly higher (3.5-fold). However, in the substantia propria, Mooren ulcer specimens revealed significantly increased numbers of CD1alpha+, CD3+, CD4+, CD20+, CD28+, B7-1+, B7-2+, and CD68+ cells. The ratios of CD4+/CD8+ cells and B7-2+/antigen-presenting cells in Mooren ulcer specimens were significantly higher (5-fold). All tested adhesion molecules showed significant up-regulation in the patients' conjunctivas. Mooren ulcer vascular endothelial cells prominently expressed E-selectin, VCAM-1, VLA-4, and ICAM-1 compared with normal conjunctiva. CONCLUSION: The simultaneous presence of multiple types of inflammatory cells, adhesion, and co-stimulatory molecules in Mooren ulcer conjunctiva suggests that their interaction may contribute to a sustained immune activation as at least part of the pathogenic mechanism of this disorder.

Antibodies, Monoclonal↗

PubMed2006

Obliterative microangiopathy presenting as chronic conjunctivitis in a patient with relapsing polychondritis.

PURPOSE: To report histopathologic findings and treatment of obliterative microangiopathy in a patient with chronic conjunctivitis due to relapsing polychondritis (RP). METHODS: This interventional case report describes a 50-year-old woman with relapsing polychondritis was referred to the Ocular Immunology and Uveitis Service for bilateral tearing due to refractory chronic conjunctivitis. Ocular examination revealed the presence of bilateral follicular conjunctivitis. Conjunctival biopsy of the inferior palpebral conjunctiva was performed, and the histopathologic findings guided the therapeutic intervention. RESULTS: The pathology of the conjunctival biopsy included granulomatous obliterative microangiopathy with numerous eosinophils, plasma cells, lymphocytes, and epithelioid cells in the substantia propia. The chronic conjunctivitis resolved with systemic methotrexate therapy. CONCLUSIONS: Microangiopathy may cause chronic conjunctivitis in patients with RP and may be a harbinger of evolving nonocular problems as a consequence of incomplete control of this autoimmune disorder. Immunomodulatory therapy should be considered in such cases to prevent possible cardiovascular, renal, respiratory, and neurologic complications of vasculitis. Although methotrexate has been used in treatment of RP-related necrotizing scleritis with poor results, it can be sufficient for the conjunctivitis with microangiopathy associated with RP.

Chronic Disease↗

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↗

PubMed2004

Ocular cicatricial pemphigoid, keratomycosis, and intravenous immunoglobulin therapy.

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.

Adult↗

PubMed2003

Efficacy of etanercept in preventing relapse of uveitis controlled by methotrexate.

OBJECTIVE: To evaluate the efficacy of etanercept vs placebo in preventing relapses of uveitis in patients taking methotrexate with control of uveitis and whose methotrexate dosage was being tapered. METHODS: Patients with chronic or recurrent noninfectious uveitis with inflammation controlled by low-dose methotrexate were randomized to either the drug or placebo group in a double-masked manner, given a methotrexate taper schedule, and followed for 24 weeks. The main outcome measures were control of inflammation, visual acuity, and adverse reactions. Data were analyzed both as an attempt-to-treat analysis and an analysis only of those patients who completed the study. RESULTS: A total of 20 patients were randomized to the drug and placebo groups. Relapse of uveitis occurred in 3 of 10 patients in the treatment group and 5 of 10 patients in the control group. Two patients in the treatment group withdrew prematurely from the study due to adverse effects. There was no significant difference between the treatment and placebo groups with regard to the rate of relapse and the final visual acuity. No patient suffered from any irreversible, long-term morbidity or mortality. CONCLUSION: Etanercept has no significant efficacy over placebo in preventing relapses of uveitis in patients being tapered from methotrexate.

Anti-Inflammatory Agents, Non-Steroidal↗

PubMed2003

Mycophenolate mofetil as an immunomodulatory agent in the treatment of chronic ocular inflammatory disorders.

PURPOSE: To evaluate the outcomes of patients with chronic ocular inflammatory disease treated with mycophenolate mofetil as an immunosuppressive and steroid-sparing agent. DESIGN: Retrospective noncomparative interventional case series. PARTICIPANTS: All patients with ocular inflammatory disease treated with mycophenolate mofetil at a single institution between 1998 and 2001. METHODS: Charts of patients seen on the Ocular Immunology and Uveitis Service at the Massachusetts Eye and Ear Infirmary were reviewed. Patients with chronic ocular inflammatory disease were included in the study. MAIN OUTCOME MEASURES: Control of inflammation, steroid-sparing effect, visual acuity, and adverse reactions were measured. RESULTS: A total of 54 patients were evaluated. Control of ocular inflammation with mycophenolate mofetil as monotherapy was achieved in 35 patients (65%) and in 67 eyes (62%), and a steroid-sparing effect was achieved in 29 (54%) patients. Visual acuity was maintained or improved in 51 patients (94%) and in 97 eyes (90%). Side effects requiring discontinuation of medication occurred in 10 patients (18%). There was neither long-term morbidity nor mortality due to mycophenolate mofetil. CONCLUSIONS: Mycophenolate mofetil is effective in the treatment of patients with steroid-dependent or -resistant chronic ocular inflammatory disorders that fail to respond to conventional steroid treatment. It is a safe and effective steroid-sparing immunomodulatory agent and can be considered an important addition to our armamentarium in the care of patients with ocular inflammatory disease.

Adolescent↗

PubMed2003

Long-term progression, prognosis, and treatment of patients with recurrent ocular manifestations of Reiter's syndrome.

PURPOSE: To investigate the spectrum of ocular involvement, to examine the clinical outcome, and to analyze the influence of treatment in patients with chronic ocular manifestations of Reiter's syndrome (RS) referred to a tertiary care ocular immunology service. DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS: Twenty-five patients with RS evaluated at the Ocular Immunology and Uveitis Service of the Massachusetts Eye and Ear Infirmary from 1981 through 2001. METHODS: Charts of patients were reviewed and data on age, gender, follow-up time, ocular symptoms, extraocular involvement, ocular complications, therapy, and visual acuities were recorded. MAIN OUTCOME MEASURES: Visual acuity, ocular complications, disease progression, clinical outcome, and systemic treatment. RESULTS: Twenty-five patients (20 male and 5 female) diagnosed with RS, with a mean age at presentation to our service of 37 years, were studied. The mean follow-up was 48.5 months. Eighty-five percent of patients tested were positive for human leukocyte antigen B27. Sixty-four percent of patients had a positive family history. All patients had oligoarthritis and enthesitis, most commonly affecting the back (56%), Achilles tendon (52%), and sacroiliac joint (24%). Eighty percent had a history of infection, most frequently urethritis (68%). Forty-four percent had a history of mucocutaneous lesions. All patients demonstrated ocular involvement at the time of diagnosis (68% with unilateral and 32% with bilateral disease), 84% had evidence of uveitis, 3% had scleritis, 2% had conjunctivitis, and 1% had pars planitis and iridocyclitis. During follow-up, the ocular complications included conjunctivitis (96%), anterior uveitis (92%), posterior uveitis (64%), keratitis (64%), cataract (56%), intermediate uveitis (40%), scleritis (28%), cystoid macular edema (28%), papillitis (16%), and glaucoma (16%). Systemic treatment for ocular inflammation was initiated in all patients. Ninety-six percent were treated with nonsteroidal anti-inflammatory agents. Eighty-eight percent were treated with corticosteroids, 64% requiring systemic prednisone. Immunosuppressive therapy was initiated in 52% of patients, with all receiving methotrexate. Seven patients required more than one immunosuppressive agent. The mean initial visual acuity was 20/25 in the right eye and 20/30 in the left eye. The mean final visual acuity was 20/25 in the right eye and 20/25 in the left eye. CONCLUSIONS: Reiter's syndrome may be associated with chronic recurrent ocular inflammation. Systemic therapy (including immunosuppressive treatment) typically is required to control the ocular inflammation and to prevent progressive visual loss.

Adolescent↗

PubMed2003

Human mast cell subtypes in conjunctiva of patients with atopic keratoconjunctivitis, ocular cicatricial pemphigoid and Stevens-Johnson syndrome.

PURPOSE: Investigate mast cell (MC(S)) subtypes in atopic keratoconjunctivitis (AKC), ocular cicatrical pemphigoid (OCP), and Stevens-Johnson Syndrome (SJS). METHODS: MC(S) subtypes were determined by immunohistochemistry of conjunctiva (obtained from 34 patients--9 AKC, 9 OCP, 9 SJS and 7 normal) using monoclonal antibodies directed against chymase (MC(C)) and tryptase (MC(T)). Double staining was used to distinguish MC(S) as positive for both chymase and tryptase (MC(TC)). RESULTS: The number of MC(S) was significantly increased in AKC, OCP and SJS patients, compared to normal subjects. MC( C) were especially high in AKC, and moderately high in OCP. MC(T ) and MC(TC) were similar in each disease group. CONCLUSIONS: While the AKC findings were not surprising, the result in OCP and SJS suggests that MC(S) play an underappreciated role in the inflammatory process of these disorders. Disparate proportions of MC(S) subtypes in these diseases may imply differential functions of MC(S) in these disorders.

Adult↗

PubMed2002

Sectorial keratitis and uveitis: differential diagnosis.

PURPOSE: The purpose of this study was to examine the importance of considering the differential diagnosis for patients with sectorial keratitis and uveitis by case summary and literature review. METHODS: A retrospective review of patients with sectorial keratitis and uveitis seen at the Ocular Immunology and Uveitis Service of the Massachusetts Eye and Ear Infirmary and a summary of the diagnoses of cases with similar ocular findings that have been reported in the literature. RESULTS: Data on six patients with sectorial keratitis and uveitis were reviewed. Four patients were female and two were male, ages 21-50 years. All were eventually diagnosed with herpes simplex viral stromal sectorial keratitis. The corneal infiltrates were most common in the superior corneal quadrants, located in the posterior corneal layers. Anterior non-granulomatous uveitis was present in all cases. One case had bilateral ocular involvement. Five of the six patients responded to topical steroids and antiviral treatment. Immunomodulation with methotrexate, cyclosporine, and systemic prednisone was required in one patient. Glaucoma was a complication in six of the seven eyes. Five patients developed corneal scarring, localized in the anterior and mid-stroma. CONCLUSION: The differential diagnosis of sectorial keratitis and uveitis is limited. The entities included in the differential are diverse; some of them threaten not only vision but also life. It is important for the ophthalmologist to be familiar with these entities, in order to pursue pertinent diagnostic investigations and arrive at an accurate diagnosis and institute appropriate management.

Acyclovir↗

PubMed2002

Ocular characteristics and disease associations in scleritis-associated peripheral keratopathy.

OBJECTIVES: To evaluate ocular characteristics and systemic disease associations in patients with scleritis-associated peripheral keratopathy and its different patterns, and to assess any ocular or systemic prognostic significance of the presence of the types of peripheral keratopathy in patients with scleritis. DESIGN: Review of 125 patients with scleritis alone and 47 patients with scleritis-associated peripheral keratopathy; review of patients with scleritis and the different patterns of peripheral keratopathy: peripheral corneal thinning, stromal keratitis, and peripheral ulcerative keratitis (PUK); review of ocular and systemic outcomes comparisons between patients with scleritis with and without peripheral keratopathy. RESULTS: Patients with peripheral keratopathy had more necrotizing scleritis (57%, P<.001), decrease in vision (81%, P<.001), anterior uveitis (62%, P<.002), impending corneal perforation (62%, P<.001), and potentially lethal specific-disease association (87%, P<.001) than did patients with scleritis alone. Patients with PUK had the worst ocular and systemic outcomes. Of the 24 patients with PUK, 16 (67%) had necrotizing scleritis (P =.02), virtually all had a potentially lethal systemic disease (P =.02), and all had impending corneal perforation (P<.001). CONCLUSION: The detection of peripheral keratopathy, and especially PUK, in a patient with scleritis indicates a poor ocular and systemic prognosis.

Adolescent↗

PubMed2002

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

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.

Adult↗

PubMed2002

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

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-Behçet'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.

Adolescent↗

PubMed2002

Multifocal choroiditis and panuveitis: immunomodulatory therapy.

PURPOSE: To report our analysis of the efficacy of immunomodulatory therapy on the course of 19 patients with multifocal choroiditis and panuveitis (MCP). DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS: Nineteen patients with multifocal choroiditis with panuveitis evaluated on the Ocular Immunology and Uveitis Service of the Massachusetts Eye and Ear Infirmary from 1978 to 2000. METHODS: Fifteen patients were treated with systemic immunomodulatory therapy; 4 patients (who refused therapy) were treated with systemic steroids. All patients were analyzed for control of inflammation, visual acuity outcome, and tolerance of immunomodulatory therapy. MAIN OUTCOME MEASURES: Control of inflammation and visual acuity. RESULTS: Nineteen patients with bilateral MCP with a mean follow-up of 72.7 months were studied. Fifteen were treated with immunomodulatory agents, whereas 4 patients received only systemic steroids; these 4 developed serious systemic steroid-related complications, and 12 others had cataract and/or glaucoma related to chronic topical, regional, or systemic steroid use before immunomodulatory therapy. Two patients who refused immunomodulatory therapy lost considerable vision in three of their four eyes. Of the 15 patients treated with immunomodulatory drugs, 7 patients lost considerable vision in one eye on steroid therapy but maintained good vision in the other eye once immunomodulatory therapy was instituted. No patient lost vision in any eye once he or she was treated with immunomodulatory treatment. CONCLUSIONS: Immunomodulatory therapy controls inflammation and preserves vision in patients with multifocal choroiditis and panuveitis.

Adjuvants, Immunologic↗

PubMed2002

Limbal stem cell transplantation in chronic inflammatory eye disease.

OBJECTIVE: The goal of this study was to describe the outcome of limbal stem cell transplantation (LSCT) in patients with severe ocular surface disease caused by underlying chronic inflammatory eye disease. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Nine patients with limbal stem cell deficiency caused by an underlying ocular inflammatory disease who underwent LSCT. METHODS: The authors reviewed the records of 11 eyes of 9 patients with immunologically mediated ocular surface disease that underwent LSCT. MAIN OUTCOME MEASURES: The main outcome measures were reepithelialization of the corneal surface, restoration of corneal surface, and improvement in visual acuity. RESULTS: A total of 11 eyes underwent either autologous (n = 1) or HLA-matched living related donor (n = 10) LSCT for ocular surface disease secondary to inflammatory disease. Reepithelialization of the corneal surface in the immediate postoperative period occurred in 10 eyes (91%) within an average of 10 days (range, 3-21 days). Long-term restoration of the corneal surface was achieved in six (55%) eyes. Visual acuity improved in six eyes (55%). Reasons for poor outcomes included microbial infection, limbal stem cell graft rejection, and corneal ulceration. No donor eyes had complications. CONCLUSIONS: Patients with underlying immunologically mediated diseases, such as Stevens-Johnson syndrome, toxic epidermal necrolysis, or ocular cicatricial pemphigoid, who undergo LSCT have lower success rates than do those patients with noninflammatory ocular surface diseases.

Adult↗

PubMed2002

Acute comitant esotropia after cataract surgery.

A 52-year-old woman developed comitant esotropia after cataract surgery. The ocular history was significant for accommodative esotropia; preoperatively, she manifested peripheral fusion with central suppression. Immediately postoperatively, the patient was diplopic with comitant esotropia and was treated with extraocular muscle surgery. The appearance of horizontal strabismus after cataract surgery in patients with a history of accommodative esotropia, especially those with subnormal binocularity, is a possible complication that should be discussed with the patient.

Accommodation, Ocular↗

PubMed2002

Relapsing polychondritis: a clinical review.

OBJECTIVE: This study comprehensively reviews the literature related to relapsing polychondritis (RP). METHODS: A detailed search via MEDLINE (PubMed) was performed using relapsing polychondritis as the key term. Relevant articles were analyzed with a focus on history, epidemiology, etiology, pathogenesis, clinical manifestations, diagnosis, treatment, and prognosis of RP. RESULTS: RP is a rare episodic and progressive inflammatory disease of presumed autoimmune etiology first described in 1923. RP affects cartilage in multiple organs, such as the ear, nose, larynx, trachea, bronchi, and joints. In addition, it can affect proteoglycan-rich tissues, such as the eyes, aorta, heart, and skin. The diagnosis of RP is based on the presence of clinical criteria. A standardized therapeutic protocol for RP has not been established. Nonsteroidal anti-inflammatory drugs, dapsone and/or colchicine, may control disease activity in some patients. In other patients, immunosuppressive drugs and prednisone have been effective. RP is a potentially lethal disease; pulmonary infection, systemic vasculitis, airway collapse, and renal failure are the most common causes of death. Earlier studies indicate survival rates between 70% at 4 years and 55% at 10 years. In a recent study, a survival rate of 94% at 8 years may be due to improved medical and surgical management. CONCLUSIONS: RP is a rare, multisystemic, and potentially fatal disease. The pathogenesis and optimal therapeutic approach to patients with RP is poorly understood.

Cartilage↗

PubMedDate not supplied

Ocular manifestations and concepts of systemic vasculitides.

Vasculitic disorders are relatively rare. Their etiology and pathophysiology remain enigmatic, leading to confusing nomenclature and multiple classification schemes. Untreated vasculitis can be fatal. Early diagnosis is the key to successful treatment and better prognosis. However, early diagnosis can be difficult; vasculitic conditions usually present with non-specific symptoms for a long period before clinically overt manifestations occur. Ophthalmologists should be familiar with the ocular manifestations of the vasculitic disorders because they may not only be sight-threatening, but more importantly could be the presenting manifestations of active, potentially lethal systemic disease. This review summarizes clinical and ocular manifestations of systemic vasculitic disorders. Furthermore, it discusses general concepts in diagnosis and treatment of these diseases in an effort to provide a practical framework for the ophthalmologist evaluating patients with vasculitis.

Eye Diseases↗

PubMedDate not supplied

Outcomes of treatment with immunomodulatory therapy in patients with corticosteroid-resistant juvenile idiopathic arthritis-associated chronic iridocyclitis.

PURPOSE: To describe the clinical outcome of patients with juvenile idiopathic arthritis (IA)-associated chronic iridocyclitis unresponsive to conventional therapy, in whom inflammation was eventually controlled using immunomodulatory therapy (IMT), and to determine if patients treated early with IMT have better visual acuity outcomes than those treated with corticosteroid alone. METHODS: Patients with JIA-associated chronic iridocyclitis receiving immunomodulatory therapy at the Ocular Immunology and Uveitis Service of the Massachusetts Eye and Ear Infirmary between 1981 and 2001 were studied. INCLUSION CRITERIA: JIA with chronic corticosteroid-dependent uveitis; IMT for a minimum of one year; minimum follow-up of three years; inflammation eventually controlled for a minimum of six consecutive months by IMT. RESULTS: At last visit, 51% of eyes (23 eyes) had acuity of 20/20 to 20/40, 16% (7 eyes) had 20/50 to 20/100, and 33% (15 eyes) had 20/200 or less. In patients whose chronic inflammation was eventually controlled within three years from onset of the uveitis, final bilateral vision was maintained within 20/20 to 20/30, except for a patient whose one eye had poor vision at initial consultation. When acuities at the end of the follow-up period of patients treated early with IMT were compared with acuities at the initial consultation of patients treated late with IMT, with duration of uveitis matched for each patient, visual acuities of those treated early were statistically significantly better than those treated late with IMT (p < 0.005 right and left eyes pooled; p = 0.0075 best eyes; p = 0.0375 worst eyes). CONCLUSIONS: We noted improvement or maintenance of visual acuity (86%) during the course of follow-up of patients with treatment-resistant JIA-associated uveitis treated with effective IMT. However, only IMT given early in the disease course was noted to be associated with bilateral visual acuity of 20/30 or better.

Adolescent↗