Purtscher-like retinopathy in patients with COVID-19.

Purpose: Several ophthalmological complications of coronavirus disease 2019 (COVID-19) involving the anterior and posterior segment have been reported in the literature. We present two patients with Purtscher-like retinopathy presenting with bilateral central vision loss and diffuse peripapillary cotton wool spots extending into the macula bilaterally. Observations: In both cases, the patients were diagnosed with COVID-19 within a week of their presentations and had relatively mild courses of COVID-19. In the first case, the patient's vision was hand motion in the right eye and count fingers in the left eye. A broad workup was negative, revealing no concerns for underlying conditions traditionally associated with Purtscher-like retinopathy. Head and neck imaging was also negative for vascular abnormalities. The patient was given a diagnosis of Purtscher-like retinopathy due to COVID-19 infection and was started on 60 mg of oral prednisone. At 4-week follow up, patient's vision had improved to 20/100 in the right eye and 20/80 in the left eye, although she continued to have minimal central vision. The patient continued to have Purtscher flecken in the posterior pole, but the density of the opacities and retinal hemorrhages had significantly improved. In the second case, the patient's past medical history was significant for Huntington's disease and kidney transplant on dialysis but she had not had any ocular issues prior. One week within diagnosis of COVID-19, she presented with a vision of count fingers in the right eye and 20/400 in the left eye. The patient's baseline dose of 5 mg prednisone was increased to 60 mg for 1 week. She received an Ozurdex implant in both eyes. The patient's vision at 9 week follow up improved to 20/40 pinholing to 20/25 in the right eye and 20/200 pinholing to 20/70 in the left eye and dilated exam showed improving edema and improving density of Purtscher-flecken from initial presentation. Conclusions and importance: Very few cases of COVID-19 associated Purtscher-like retinopathy have been reported in the literature and all have been reported with serious clinical courses of COVID-19. To the best of our knowledge, these are the first cases to be reported after relatively mild courses of COVID-19 and highlight the importance of including COVID-19 in the differential for Purtscher-like retinopathy.

Nuclear cataract as an early predictive factor for recalcitrant juvenile idiopathic arthritis-associated uveitis.

PURPOSE: To analyze factors predictive of having treatment-resistant uveitis in patients with juvenile idiopathic arthritis (JIA)-associated uveitis. METHODS: The medical records of patients diagnosed with JIA-associated uveitis treated at a single tertiary referral center from October 2005 to March 2013 were reviewed retrospectively. The main outcome measures were demographic characteristics, ocular comorbidity, clinical course, treatments, and baseline risk factors associated with poor response to first-line therapies. RESULTS: A total of 96 patients (175 eyes) were included. Of these, 58 patients (108 eyes) required biologic disease-modifying antirheumatic drugs or alkylating agents for their uveitis during follow-up (recalcitrant group), and 38 patients (67 eyes) did not (nonrecalcitrant group). Eyes of the recalcitrant group tended to have a higher incidence of cataract at baseline (49%; P < 0.0001). In the nonrecalcitrant group, the most frequent complications were cataract (20.9%) and secondary glaucoma (20.9%). The mean number of flares in the recalcitrant group was significantly reduced from 3.7/eye/year prior to cataract surgery to 1.6/eye/year after (P < 0.0001). Nuclear cataract was found to be an independent predictor for a severe course of JIA-associated uveitis. Any other type of cataract, posterior synechiae, male sex, or active uveitis at baseline were not found to be independently associated with recalcitrant uveitis. CONCLUSIONS: Nuclear cataract at baseline evaluation is a risk factor for poor response to first-line therapies in JIA-associated uveitis patients.

Current screening and treatments in retinopathy of prematurity in the US.

Retinopathy of prematurity (ROP) is a complex disease characterized by an aberrant developmental retinal angiogenesis in preterm infants and can carry significant visual morbidity, including retinal detachment and blindness. Though large scale, randomized clinical trials have improved our understanding of the pathophysiology and progression of the disease, the management of ROP remains a challenge for ophthalmologists. This review addresses the up-to-date screening approach, diagnosis, and treatment guidelines for ROP in the US.

Hand, foot and scrotal blisters in a patient with cancer receiving oral chemotherapy.

Tyrosine kinase inhibitors are novel antineoplastic drugs that make use of the molecular abnormalities that have been discovered in certain types of tumours. These agents are associated with important dermatological side effects. This case report discusses an atypical presentation of the hand-foot syndrome in one patient treated with sorafenib. A 78-year-old man, recently started on sorafenib for the treatment of hepatocarcinoma with lung metastasis, presented to the emergency room with painful blistering lesions on the palms, soles and scrotum for 1 week. A punch biopsy was performed and sorafenib was withheld. Direct immunofluorescence study was negative for an autoimmune blistering disorder and histopathology stains did not show any microorganisms. A diagnosis of hand-foot syndrome was made. After 2 weeks, the patient showed marked improvement and the plan was to restart sorafenib at a lower dosage.

Neutrophil collagenase, gelatinase, and myeloperoxidase in tears of patients with stevens-johnson syndrome and ocular cicatricial pemphigoid.

OBJECTIVE: To investigate the levels of matrix metalloproteinases (MMPs), myeloperoxidase (MPO), and tissue inhibitor of metalloproteinase-1 (TIMP-1) in tears of patients with Stevens-Johnson syndrome (SJS) and ocular cicatricial pemphigoid (OCP). DESIGN: Prospective, noninterventional cohort study. PARTICIPANTS: Four SJS patients (7 eyes), 19 OCP patients (37 eyes), and 20 healthy controls who underwent phacoemulsification (40 eyes). METHODS: Tear washes were collected from all patients and were analyzed for levels of MMP-2, MMP-3, MMP-7, MMP-8, MMP-9, MMP-12, MPO, and TIMP-1 using multianalyte bead-based enzyme-linked immunosorbent assays. Total MMP activity was determined using a fluorometric assay. Correlation studies were performed between the various analytes within study groups. MAIN OUTCOME MEASURES: Levels of MMP-2, MMP-3, MMP-7, MMP-8, MMP-9, MMP-12, MPO, and TIMP-1 (in nanograms per microgram of protein) and total MMP activity (in relative fluorescent units per minute per microgram of protein) in tears; MMP-8-to-TIMP-1 ratio; MMP-9-to-TIMP-1 ratio; and the correlations between MMP-8 and MMP-9 and both MMP and MPO. RESULTS: MMP-8, MMP-9, and MPO levels were elevated significantly in SJS and OCP tears (SJS>OCP) when compared with controls. The MMP activity was highest in SJS patients, whereas OCP patients and controls showed lower and similar activities. The TIMP-1 levels were decreased in SJS and OCP patients when compared with those in controls, with levels in OCP patients reaching significance. The MMP-8-to-TIMP-1 and MMP-9-to-TIMP-1 ratios were markedly elevated in SJS and OCP tears (SJS>OCP) when compared with those of controls. Across all study groups, MMP-9 levels correlated strongly with MMP-8 and MPO levels, and MMP-8 correlated with MPO, but it did not reach significance in SJS patients. There was no relationship between MMP-7 and MPO. CONCLUSIONS: Because MMP-8 and MPO are produced by inflammatory cells, particularly neutrophils, the correlation data indicate that they may be the common source of elevated enzymes, including MMP-9, in SJS and OCP tears. Elevated MMP-to-TIMP ratios and MMP activity suggest an imbalance in tear MMP regulation that may explain the predisposition of these patients to demonstrate corneal melting and chronic complications associated with persistent inflammation. Myeloperoxidase in tears may be a sensitive and specific marker for the quantification of ocular inflammation.

Anterior chamber intraocular lens implantation in patients with a history of chronic uveitis: five-year follow-up.

PURPOSE: To compare the incidence of long-term complications after cataract surgery with primary anterior chamber intraocular lens (AC IOL) implantation in uveitic patients and patients without a history of intraocular inflammation (control group). SETTING: Single-center private practice. DESIGN: Retrospective clinical study. METHODS: The study comprised patients who between November 2005 and August 2010 had cataract extraction followed by AC IOL implantation because conventional placement was not possible. Outcome measures were the incidence of intraoperative and postoperative complications, preoperative corrected distance visual acuity (CDVA), and CDVA after 1 year. RESULTS: Of the 39 patients identified through electronic medical records, 17 (17 eyes) had a history of chronic uveitis and 22 (23 eyes) had no intraocular inflammatory disease. There were no significant differences in the incidence of intraoperative and postoperative complications between the 2 groups during follow-up (range 12 to 68 months) (P=.702). Although uveitic eyes had a greater risk for epiretinal membrane formation, the incidence of uveitis flareups attributed to the IOL and deposits on IOL surfaces was comparable to that in the control group (P<.001). The CDVA improved significantly in both groups 1 year after surgery (P<.01 and P<.001, respectively). CONCLUSION: In uveitic eyes with inadequate capsule support, AC IOL implantation restored visual function without a significant increase in long-term postoperative complications compared with eyes that had no history of uveitis.

Infliximab for the treatment of refractory noninfectious Uveitis: a study of 88 patients with long-term follow-up.

OBJECTIVE: To establish the safety and efficacy of infliximab for the treatment of refractory noninfectious uveitis. DESIGN: Retrospective, interventional, noncomparative cohort study. PARTICIPANTS: Eighty-eight patients from a single-center private practice. METHODS: Patients with chronic, recalcitrant uveitis treated with infliximab (Remicade; Janssen Biotech, Inc., Titusville, NJ) were identified through an electronic medical record database. All charts were reviewed for sex, diagnosis, location of inflammation, presence of vasculitis, prior immunomodulatory treatments, duration of infliximab treatment, dose received, secondary side effects, and other medications continued while receiving treatment with infliximab. MAIN OUTCOME MEASURES: The primary outcome measures were the rate of remission, time to remission, relapse rate, failure rate, and patient tolerance. Additional analysis aimed to identity risk factors that would predict a higher success rate of infliximab to treat various types of noninfectious uveitis. RESULTS: Of the 72 patients (81.8%) who achieved clinical remission while being treated with infliximab, 42 (58.3%) required additional immunomodulatory medications. At 7, 18.1, and 44.7 weeks, 25%, 50%, and 75% of patients, respectively, achieved clinical remission off all corticosteroids. Thirty-two patients (36.4%) experienced at least 1 side effect while on infliximab therapy, and 17 patients (19.3%) discontinued treatment secondary to 1 or more intolerable side effects. The most common adverse effects were skin rash (9.1%) and fatigue (8%). Factors associated with a higher chance to achieve clinical remission were nonidiopathic uveitis (P < 0.001), intermediate or panuveitis (P < 0.001), absence of vasculitis (P < 0.001), and a starting dose ≥5 mg/kg (P < 0.011). CONCLUSIONS: Infliximab induces a high rate of complete clinical remission in recalcitrant uveitis and is well tolerated by most patients.

Analysis of a novel protocol of pulsed intravenous cyclophosphamide for recalcitrant or severe ocular inflammatory disease.

PURPOSE: To analyze the success rate of pulsed intravenous (IV) cyclophosphamide (CyP) for noninfectious ocular inflammatory disease and to identify risk factors for failure of therapy. DESIGN: Retrospective, interventional, noncomparative cohort study. PARTICIPANTS: One hundred ten eyes of 65 patients. METHODS: Through a computer search of the Massachusetts Eye Research and Surgery Institution's database, we identified patients who were treated with IV CyP between May 2005 and April 2012. We obtained demographic and clinical information through review of the electronic health record of each patient. MAIN OUTCOMES MEASURES: Clinical response, corticosteroid-sparing effect, recurrence rate, calculated "risk factors" for failure, visual acuity, and adverse reactions. RESULTS: Pulsed IV CyP achieved complete remission of inflammation (for ≥ 2 visits) in 54 patients (84.4%). Sustained remission of inflammation occurred in 70% of patients within 3 months, 86.6% of patients within 6 months, and 91.7% within 9 months. The mean time to achieving quiescence was 3.5 months. The success rate in reducing corticosteroid to prednisone ≤ 10 mg/d within 6 months, while maintaining control of ocular inflammation, was 89.7% (95% confidence interval [CI], 81.1-93.5%). The mean duration of clinical remission for those patients who had a positive response to CyP was 32.67 months (95% CI, 25.91-39.43). Relapse of vasculitis was observed in 1 patient (1.5%) after completing the course of therapy. Early initiation of therapy during the course of the disease was correlated with a lesser rate of recurrence (P = 0.028). The most common adverse effects were nausea (29%) and transient lymphopenia (26%). The mean best-corrected visual acuity (BCVA) improved from 0.59 ± 0.66 at baseline to 0.30 ± 0.54 at 6 months of follow-up (P<0.001). The mean follow-up period was 31.61 ± 20.47 months. CONCLUSIONS: Pulsed IV CyP employing our protocol results in an extremely high rate of sustained complete remission in patients with recalcitrant and fulminant, vision-threatening ocular inflammatory disorders, with an excellent safety profile in the hands of physicians trained and skilled in the art of this therapy. It also allows tapering and discontinuing corticosteroids in most patients. Early initiation of therapy may decrease the risk of relapses. FINANCIAL DISCLOSURE(S): The authors have no proprietary or commercial interest in any materials discussed in this article.

Glaucoma and uveitis.

Despite its relative rarity, uveitis is the third leading cause of preventable blindness worldwide. Glaucoma associated with uveitis is one of the most serious complications of intraocular inflammation. We review in detail the epidemiology and pathogenesis of uveitic glaucoma and the safety and efficacy of the current medical and surgical treatment modalities.

Profile of local interleukin expression in a cohort of ocular cicatricial pemphigoid patients.

PURPOSE: We investigated the expression of IL-1, IL-6, IL-12, IL-13, and IL-17 in the conjunctiva of patients with ocular cicatricial pemphigoid (OCP), also labeled as ocular mucous membrane pemphigoid (MMP). METHODS: A retrospective case-control study was done on 5 biopsy-proven OCP subjects and 6 healthy volunteers. Conjunctival specimens were obtained, and the local expression of IL-1, IL-6, IL-12, IL-13, and IL-17 was studied by immunohistochemistry. Clinical and therapeutic features were collected during follow-up. RESULTS: No remarkable IL-1, IL-6, IL-12, IL-13, or IL-17 expression was observed in normal conjunctival specimens. All OCP samples had remarkable amounts of IL-12 and IL-17 expression especially in the epithelium and stroma; there also was stromal overexpression of IL-6. The mean follow-up after the biopsy was 13 months (range 9-15 months). CONCLUSIONS: Our results demonstrated, for the first time to our knowledge, a local overexpression of IL-6, IL-12, and IL-17 in conjunctiva of OCP compared to controls.

Long-term safety and visual outcomes of anterior chamber intraocular lens implantation in patients with a history of chronic uveitis.

PURPOSE: To compare the visual outcomes and prevalence of long-term complications in patients with quiescent uveitis after phacoemulsification with traditional in-the-bag intraocular lens (IOL) implantation versus primary or secondary anterior chamber (AC) IOL implantation due to inadequate capsule support. SETTING: Single-center private practice. DESIGN: Retrospective observational cohort study. METHODS: Patients with quiescent uveitis who had cataract surgery with subsequent AC IOL implantation were identified through an electronic medical record database. They were age matched with patients with uveitis who had conventional placement of a posterior chamber (PC) IOL. The rate of postoperative complications and corrected distance visual acuity (CDVA) were reviewed preoperatively and 1, 3, and 6 months and 1, 2, 3, and 4 years postoperatively. RESULTS: The incidence of postoperative complications was not statistically different for any criterion except posterior capsule opacification development in the PC IOL cohort (relative risk, 0.071). The mean follow-up was 36.11 months ± 17.3 (SD) in the AC IOL group and 53.72 ± 20.4 months in the PC IOL group. The mean preoperative CDVA was 1.49 ± 1.07 logMAR in the AC IOL group and 0.5 ± 0.26 logMAR in the PC IOL group. Both groups had a significant improvement in CDVA visual acuity 3 years postoperatively (P=.001 and P=.010 respectively). CONCLUSIONS: In uveitic eyes with inadequate capsule support, AC IOL implantation was safe and effective in providing satisfactory improved CDVA without a significant increase in long-term complications compared with eyes that had PC IOL placement.

Serous macular detachment associated with dome-shaped macula and tilted disc.

INTRODUCTION: An entirely new type of staphyloma has been recently described as dome-shaped macula (DSM). It is characterized by an abnormal convex macular contour within the concavity of a posterior staphyloma. We found DSM associated with serous macular detachment (SMD) and tilted disc in two consecutive cases. CASE REPORTS: Case 1: A 37-year-old female presented to our department because of sudden onset blurred vision in her right eye (OD). The best-corrected visual acuity (BCVA) was 0.5 in both eyes. Funduscopy evidenced bilateral tilted disc associated with posterior staphyloma. Optical coherence tomography (OCT) demonstrated a DSM with SMD in her OD. After 15 months of follow-up, BCVA of her OD remained stable with chronic SMD. Case 2: A 32-year-old female presented to our department because of blurred vision in her OD. The BCVA was 0.4 in the OD and 1.0 in the left eye (OS). Bilateral tilted disc and posterior staphyloma were evidenced in the funduscopy. OCT demonstrated a bilateral DSM with SMD in her OD. After 45 months of follow-up, two further episodes of transient SMD were observed in her OD and seven in her OS. The final BCVA was 0.63 in the OD and 0.8 in the OS. DISCUSSION: SMD associated with tilted disc constitutes a potential cause of subretinal fluid accumulation in myopic patients. OCT is essential for the detection of both SMD and DSM.

Road to remission: a comprehensive review of therapy in uveitis.

INTRODUCTION: Although uveitis remains the third leading cause of preventable blindness in the US, the care and management of patients with uveitis and ocular inflammatory disease sit poised to make evolutionary if not revolutionary changes in the years ahead. This review serves to highlight important advances in the pharmacologic options available for the treatment of uveitis and ocular inflammation. AREAS COVERED: Advances in steroid therapy (both topical and extended delivery), updates in the clinical safety of systemic immune modulation, and the emerging therapies for uveitis and ocular inflammatory disease are some of the areas covered in this review. EXPERT OPINION: Corticosteroids have been the mainstay in the care of patients with ocular inflammatory disorders for many years. Indeed, some physicians still use only steroids for treating inflamed eyes. However, the mission is remission of all corticosteroids in order to prevent the complications associated with long-term corticosteroid use. The goal is to achieve quiescence through aggressive use of corticosteroids to extinguish the fire and then move along to achieve steroid-free remission through immunosuppressant agents.

Celecoxib for recurrent sclerouveitis after syphilitic panuveitis. A case report.

PURPOSE: To report a case of recurrent ocular inflammation after optimal therapy of bilateral syphilitic panuveitis responding to oral celecoxib. METHODS: A case report was conducted. RESULTS: A 76-year-old man presented with painful blurry vision in both eyes. Ocular examination disclosed bilateral panuveitis. Serological testing confirmed blood and cerebrospinal fluid syphilitic involvement. After 2 weeks of intravenous penicillin therapy, recurrent episodic sclerouveitis was observed. CONCLUSION: Ocular inflammation after healing of infectious uveitis is a rare ophthalmic sequela. In an immunocompetent patient, either re-infection or immune uveitis should be evoked. Non-steroidal therapeutic options, as celecoxib, could be a good option of treatment in such immune cases.