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The coronavirus disease 2019 (COVID-19) came under the attention of the international medical community when China first notified the World Health Organization of a pneumonia outbreak of then-unknown etiology in Wuhan in December 2019. Since then, COVID-19 caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has appalled the world by spreading at a pandemic speed. Although ophthalmologists do not directly engage in the clinical care of COVID-19 patients, the ophthalmology community has become aware of the close ties between its practice and the pandemic. Not only are ophthalmologists at heightened risk of SARS-CoV-2 exposure due to their physical proximity with patients in routine ophthalmic examinations, but SARS-CoV-2 possesses ocular tropism resulting in ocular complications beyond the respiratory tract after viral exposure. Furthermore, patients could potentially suffer from adverse ocular effects in the therapeutic process. This review summarized the latest literature to cover the ophthalmic manifestations, effects of treatments, and vaccinations on the eye to aid the frontline clinicians in providing effective ophthalmic care to COVID-19 patients as the pandemic continues to evolve.

Background: Psoriasis is primarily a skin disorder with many extracutaneous manifestations. Relationship between eye lesions and psoriasis has been reported in literature. However, much data are not available on ocular manifestations of psoriasis in the Indian population. Objective: The objectives of this study were to assess the frequency of ocular manifestations in psoriasis and to correlate it with factors such as age, gender, duration of disease, type of psoriasis, and severity of psoriasis. Materials and Methods: A total of 100 patients with psoriasis and 100 healthy controls were enrolled in the study. Clinical types of psoriasis, duration of disease, site of involvement, and severity of psoriasis were noted. Slit lamp examination, fluorescein staining of ocular surface, and fundoscopy were done for both groups. Dry eye evaluation was performed by measuring the values of Schirmer 1 and tear film breakup time. Results: Prevalence rate of ocular involvement in psoriasis was found to be 61% as compared to 30% in controls. Most common ocular finding was conjunctival hyperaemia (47%) followed by dry eye (36%) and blepharitis (27%). Scalp psoriasis was found to be significantly associated with blepharitis (P < 0.0001). There was corneal involvement in 9 cases and uveitis in three cases. No correlation was found between the frequency of ocular manifestations and factors such as age, gender, duration, and the severity of psoriasis. Conclusion: Ocular manifestations in psoriasis are common and they affect mostly the anterior segment of the eye. Routine ophthalmological evaluation is important in these patients for early diagnosis, which in turn can prevent sight threatening complications.

Aim: The aim of the study was to report the etiology, pattern, and spectrum of ocular injuries in patients with maxillofacial injuries seen at a tertiary care hospital in northern Nigeria. Patients and Methods:This is a retrospective evaluation of the etiology, spectrum, and treatment of ocular injures seen in patients with midfacial fractures, who presented at Ahmadu Bello University Teaching Hospital, Shika-Zaria, Nigeria, a tertiary care hospital in northern Nigeria. Cases were seen at the oral and maxillofacial clinic for those presenting with midfacial fractures, whereas those presenting with ocular injuries due to midfacial injuries were referred from the ophthalmology department. Data were collected on their demographics, etiology of injuries, body part(s) involved, and treatment.Results:A total of 256 patients had midfacial injuries during the period, of which 219 patients had 357 associated ocular injuries. The age range was between 3 and 76 years. There were more males (83.8%) than females (16.2%), giving a male-to-female ratio of 5.2:1; the 21–30 years' age bracket was most frequently affected (38.4%). The predominant etiology of injuries was road traffic accidents (RTAs) (90.1%), followed by assault (4.9%). Zygomatic complex fractures (38.6%) and orbital wall fractures (24.7%) were the common midfacial injuries. There were 357 ocular injuries, giving a patient: injury ratio of 1:1.6, with subconjunctival hemorrhage (32.5%) and ruptured globe (20.0%) being common. Treatments performed were reduction and immobilization of midfacial fractures plus evisceration (32.0%), followed by reduction and immobilization of fractures alone (23.9%) and reduction and immobilization plus grafting of soft tissues (21.2%).Conclusion:Ocular injuries are quite common in patients with midfacial injuries, with a ratio of 1.6:1. RTA was the most common etiology, with zygomatic complex fractures as the most common midfacial injury. Subconjunctival hemorrhage was the most common ocular injury manifestation, with young adults (21–30 years of age) being most affected. Treatment often involved reduction and immobilization of midface fractures with evisceration and grafting of ocular tissues.

Background Legionnaire’s disease is one of the major causes of community-acquired pneumonia and is occasionally complicated by neurological symptoms. However, reports of ocular lesions due to Legionnaire’s disease are limited. Case presentation We report the case of a patient with Legionnaire’s disease presenting as bilateral central scotomata due to retinal lesions. The patient consulted due to fever and bilateral central scotomata, as well as other extrapulmonary symptoms. Optical coherence tomography (OCT) showed bilateral accumulations of fluid under the retina, and the patient was diagnosed with bilateral exudative retinal detachment. Later, Legionnaire’s disease was confirmed by pulmonary infiltrates on chest imaging and positive urinary antigen for Legionella pneumophila. After administration of antibiotics, the bilateral central scotomata and bilateral subretinal fluid accumulations completely resolved, as did the other extrapulmonary symptoms and the pulmonary infiltrates. Thus, the bilateral central scotomata due to exudative retinal detachment were thought to be caused by Legionnaire’s disease. Conclusions This case demonstrates that Legionnaire’s disease can present as bilateral central scotomata. We may consider the possibility of extrapulmonary involvement complicating Legionnaire’s disease when we encounter bilateral ocular lesions in patients with fever and pneumonia.

The World Health Organization declared the Coronavirus Disease 2019 (COVID-19) caused by Severe Acute Respiratory Syndrome Coronavirus 2 a “Pandemic” on March 11, 2020. As of June 1, 2020, Severe Acute Respiratory Syndrome Coronavirus 2 has infected >6.2 million people and caused >372,000 deaths, including many health care personnel. It is highly infectious and ophthalmologists are at a higher risk of the infection due to a number of reasons including the proximity between doctors and patients during ocular examinations, microaerosols generated by the noncontact tonometer, tears as a potential source of infection, and some COVID-19 cases present with conjunctivitis. This article describes the ocular manifestations of COVID-19 and the APAO guidelines in mitigating the risks of contracting and/or spreading COVID-19 in ophthalmic practices.

Purpose: To analyze the relation between Meibomian gland dysfunction, dry eye, and sarcoidosis. Materials and Methods: Twenty eyes of 10 sarcoidosis patients (Group 1) and 20 left eyes of 20 age-sex matched healthy volunteers (Group 2) were included. Presence of dry eye was evaluated with Schirmer 1 test, tear film break-up time (T-BUT), Oxford scale scoring, Ocular Surface Disease Index (OSDI) score assessments. A slit-lamp biomicroscope infrared filter (Topcon, SL-D701, IJssel, The Netherlands) was used to evaluate Meibomian glands. The drop-out ratio according to meibography was scored for each eyelid from grade 0 (no loss) through grade 3 (lost area >2/3 of the total Meibomian gland area). Results: Among dry eye tests mean Schirmer 1 and T-BUT values were lower and OSDI score was higher in Group 1 compared to Group 2 and the differences were statistically significant ( p = 0.017, p = 0.039, p = 0.003, respectively). In addition, the upper, lower and total meiboscores were statistically significantly higher in Group 1 ( p = 0.047, p = 0.003, p = 0.005, respectively). Conclusion: A significantly higher presence of dry eye and Meibomian gland drop out ratios was detected in sarcoidosis patients. It is important to monitor sarcoidosis patients for dry eye and Meibomian gland dysfunction and when detected, to treat adequately to prevent ocular surface damage.

Purpose The purpose of this study was to report 2 patients with anterior scleritis manifesting after coronavirus disease 2019 (COVID-19). Methods The patients with confirmed COVID-19 developed anterior scleritis after their systemic symptoms were markedly improved. A thorough systemic workup identified no underlying autoimmune diseases. Ocular characteristics and safety and efficacy of systemic immunosuppressive therapy were evaluated. Results Case 1 was a 67-year-old woman who presented with necrotizing anterior scleritis in both eyes 3 weeks after the onset of COVID-19. One-week treatment with topical betamethasone and oral prednisolone (65 mg daily) did not result in improvement, so she was started on intravenous cyclophosphamide and subcutaneous adalimumab in addition to oral prednisolone. Necrotizing scleritis was gradually improved over 3 months. Case 2 was a 33-year-old man who presented with sectoral anterior scleritis in his right eye 2 weeks after the onset of COVID-19. He was started on topical betamethasone and oral prednisolone (85 mg daily). One week later, all signs and symptoms disappeared, and topical and oral corticosteroids were gradually tapered off over 2 weeks. There was no recurrence of respiratory symptoms or active scleritis in any cases after discontinuation of treatment. Conclusions These cases suggest that COVID-19 can be associated with anterior scleritis, which responds to immunosuppressive and biologic agents. Ophthalmologists should consider anterior scleritis in patients with COVID-19 who present with ocular pain and redness during the convalescent phase of the illness.

Background and aim of the work Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease. Although eyes are seldom affected, this case report presents a 13-year-old Iranian girl with Raccoon eyes and neuropsychiatric manifestations as an initial presentation of SLE. Case presentation The patient was admitted to the pediatric rheumatology inpatient, Mofid Children’s Hospital, Tehran with swelling and ecchymosis around the eyes (Racoon eyes) as well as anorexia, abdominal pain, weight loss, mood disorders and hallucinations. Complete blood count showed normocytic, normochromic anemia, leukopenia, lymphopenia and thrombocytopenia. The C-reactive protein, erythrocyte sedimentation rate, liver and renal functional tests, and urine analysis were normal. The complement levels decreased, antinuclear antibody (ANA) and anti-double stranded DNA (anti-dsDNA) tests were positive. Bilateral pleural effusion and mild pericardial effusion were seen. Bone marrow aspiration showed mild hypocellularity without any evidence of malignancy. The diagnosis of neuropsychiatric SLE (NP-SLE) was held. She was treated by the pulse methyl prednisolone (30 mg/kg/d) and intravenous cyclophosphamide (500 mg/m2), oral prednisolone and hydroxychloroquine. Her appetite improved while hallucination and aggressive behavior decreased. Peri-orbital swelling and ecchymosis decreased. After one year, her appetite became normal; mood disorders, panic, phobic attacks and hallucinations were completely remedied. Swelling and ecchymosis around the eyes were eliminated. Oral prednisolone 10 mgday and hydroxychloroquine (5 mg/kg/day) were continued. Conclusion SLE may present with Raccoon eyes. Rapid detection and treatment of the disease based on clinical symptoms is critical for these patients. Prednisolone and cyclophosphamide are the best choice for treatment of the disease in children.

Objective: The aim of this multi-center study was to assess predictive factors for work-day loss as an indirect cost element in Behçet's syndrome (BS). Methods: In this cross-sectional, multi-center study, 834 BS patients (F/M: 441/393, age mean: 38.4 ± 10.9 years) were included. Data were collected by a questionnaire regarding treatment protocols, disease duration, smoking pattern, frequency of medical visits during the previous year and self-reported work-day loss during the previous year. Results: Work-day loss was observed in 16.2% of patients (M/F: 103/32). The percentages of being a smoker (81.8%), using immunosuppressive (IS) medications (82%), and having disease duration

Background Uveitis is one of the most frequent ophthalmologic manifestations in rheumatology. Uveal inflammation can underlie a systemic inflammatory rheumatic disease (SIRD) in approximately 30% of cases with a significant burden on the quality of life since it represents a cause of blindness in up to 20% of cases in Western countries. Methods In this review, we provide a comprehensive overview of the pathophysiology of uveitis associated with SIRDs. According to our literature survey on the epidemiology of uveitis among SIRDs, spondyloarthritides, Behcet's disease and sarcoidosis get the major impact. Results In Behcet's uveitis, the key players are highly polarized Th1 and Th17 lymphocytes, natural killer T cells and gamma delta T cells. All contribute to a great destructive inflammatory environment with the most serious visual damage resulting from the involvement of the posterior segment of the eye. In contrast, spondyloarthritides-related uveitis derives from a complex interaction between genetic background and extra-ocular inflammatory mediators originating from enthesitis, arthritis, psoriatic lesions and microbiome pro-inflammatory alterations. In such conditions, the immune infiltration of CD4+ T cells, Th17 and natural killer cells along with pro-inflammatory cytokines, TNF-alpha among all, leads to intraocular inflammation. Lastly, granuloma formation represents the primary hallmark lesion in sarcoid uveitis. This suggests a profound link between the innate system that mainly recruits activated macrophages and adaptive system involving by Th1, Th17 and Th17.1 cells. Conclusions Awareness among rheumatologists of a potential severe ocular involvement generates new insights into targeted therapeutic approaches and personalized treatments for each patient.

Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) can lead to severe ophthalmologic sequelae. The main risk factor is the severity of the initial ocular involvement. There are no recommendations for ocular management during acute phase.We conducted a national audit of current practice in the 11 sites of the French reference center for toxic bullous dermatoses and a review of the literature to establish therapeutic consensus guidelines. We sent a questionnaire on ocular management practices in SJS/ TEN during acute phase to ophthalmologists and dermatologists. The survey focused on ophthalmologist opinion, pseudomembrane removal, topical ocular treatment (i.e. corticosteroids, antibiotics, antiseptics, artificial tear eye drops, vitamin A ointment application), amniotic membrane transplantation, symblepharon ring use, and systemic corticosteroid therapy for ophthalmologic indication. Nine of 11 centers responded. All requested prompt ophthalmologist consultation. The majority performed pseudomembrane removal, used artificial tears, and vitamin A ointment (8/9, 90%). Combined antibiotic-corticosteroid or corticosteroid eye drops were used in 6 centers (67%), antibiotics alone and antiseptics in 3 centers (33%). Symblepharon ring was used in 5 centers (55%) if necessary. Amniotic membrane transplantation was never performed systematically and only according to the clinical course. Systemic corticosteroid therapy was occasionally used (3/9, 33%) and discussed on a case-by-case basis.The literature about ocular management practice in SJS/ TEN during acute phase is relatively poor. The role of specific treatments such as local or systemic corticosteroid therapy is not consensual. The use of preservatives, often present in eye drops and deleterious to the ocular surface, is to be restricted. Early amniotic membrane transplantation seems to be promising.

International audience; Muscular weakness and hypotonia may be associated with multisystem involvement giving rise to complex phenotypes, many of which are uncharacterized. We report a patient presenting with congenital hypotonia and severe ocular and brain abnormalities, evoking a Muscle Eye Brain disease (MEB). She had global muscular weakness, hypotonia and amyotrophy, joint hyperlaxity, kyphoscoliosis, respiratory insufficiency, dysmorphic features and severe intellectual disability. Brain MRI showed cortical atrophy and hypoplasia of the corpus callosum. Normal CK levels, non-progressive course and absence of dystrophic features or alpha-dystroglycan abnormalities on the muscle biopsy were not typical of MEB. CGH array identified a large de novo duplication in chromosome 11, including regions partially duplicated in three other patients with common clinical features. This report adds to the differential diagnosis of complex phenotypes characterized by muscular, ocular and CNS involvement and highlights the potential contribution of still unrecognized chromosomal abnormalities to these phenotypes.

Nevus sebaceous (NS) is a hamartomatous disorder of the skin and adjacent tissues characterized by epidermal, follicular, sebaceous, and apocrine gland abnormalities. It occurs in approximately 1 in 1,000 live births. A hyperactivation of Ras pathways has been recently assumed to be the cause of this phenotype. NS lesions may be isolated or coupled with extracutaneous manifestations, mostly of the central nervous, ocular, and skeletal systems, or kidneys; in this case, the term “NS syndrome” is used. Cutaneous distribution of NS usually follows the linear patterns known as “lines of Blaschko,” with lines that do not follow the segmental trajectory of the peripheral sensory nerves but instead reflect the streams or trends of growth of embryonic tissues. Histological characteristics of the lesions appear to be actually more decisive than the clinical evaluation to distinguish between the types of epidermal nevus: the typical NS has both papillated epidermal hyperplasia and a predominance of subjacent abnormal follicular–sebaceous glands.Seizures, mental retardation, and/or cognitive developmental delay are the most common neurologic abnormalities associated with NS and usually are present within the first months of life; eye and skeletal involvement may be present as well.The definitive treatment of NS is full-thickness excision. However, the necessity and timing of excision to prevent possible future malignancy are not clear; lasers and photodynamic therapy are alternatives currently being explored for the treatment of NS, with varying degrees of success.

Ocular involvement is a common manifestation of inflammatory rheumatic diseases, often requiring a multidisciplinary collaboration between rheumatologists and ophthalmologists. The aim of this study was to standardize "red flags" for referral for rheumatologists and ophthalmologists using a Delphi consensus for the management of rheumatic diseases with ocular involvement. The scientific board comprised 11 Italian hospital-based rheumatologists (N = 6) and ophthalmologists (N = 5). A systematic review identified potential red flags for referral. The panel developed 19 statements consisting of (a) referral from ophthalmologist to rheumatologist (b) referral from rheumatologist to ophthalmologist and (c) overarching principles relating to multidisciplinary roles/goals and management. Voting was performed anonymously using an online Delphi method. Each participant expressed a level of agreement on each statement using a 5-point scale (1="strongly disagree"; 5="strongly agree"). Total cumulative agreement was defined as the sum of the percentage of response to items 4 ("agree") and 5 ("absolutely agree"), consensus defined as ≥ 80% cumulative agreement for each statement. Positive consensus among 11 participants was reached for 15/19 (78.9%) statements. Statements not reaching consensus were discussed in a face-to-face meeting prior to the second vote (10 participants). Positive consensus was reached for all 19 statements, with final total cumulative agreement of 90-100%. This is the first Delphi consensus undertaken to standardize red flags for referral to rheumatologists and ophthalmologists for patients with rheumatic diseases and ocular involvement.

Postmortem pathological examinations, animal studies, and anecdotal reports suggest that coronavirus disease 2019 (COVID-19) could potentially affect intraocular tissue. However, published evidence is scarce and conflicting. In our study, we screened 100 eyes of 50 patients hospitalized for COVID-19. Relevant medical and ophthalmological history was assessed as well as symptoms, laboratory results, specific treatments, clinical course, and outcome. Ophthalmic exams including assessment of best corrected visual acuity (BCVA), intraocular pressure (IOP), color perception, ocular motility, ophthalmoscopy as well as optical coherence tomography (OCT) of the macula and the optic disc was performed at hospital admission and 29 to 192 days later. Of the 50 patients included, 14 (28%) were female. Median age was 64.5 (range 29–90) years. COVID-19 severity was mild in 15 (30%), severe in 30 (60%), and critical in five cases (10%). At baseline, median BCVA was 0.1 (0–1.8) Logarithm of the Minimum Angle of Resolution (LogMAR) and median IOP was 16 (8–22) mmHg. At follow-up, no relevant changes in BCVA and IOP were documented. No signs of active intraocular inflammation or optic nerve affection were found and OCT findings were widely stable during the observation period. Our findings suggest that COVID-19 does not regularly affect intraocular tissue.

Inverted papilloma is an uncommon histopathological finding of maxillary sinus tumor. We reported here a rare case of inverted papilloma of maxillary sinus presented initially with ipsilateral proptosis. A middle-aged lady with history of having allergic rhinitis presented with progressive left eye proptosis. Assessment revealed presence of orbital mass arising from paranasal sinus, in which the histopathological examination was initially inconclusive, and later it turned up to be inverted papilloma, partly with malignant transformation. High index of suspicion is necessary especially in those patients with recurrent non-specific sinonasal symptoms, which may need repeated biopsy and histopathological examination. Ocular manifestation signifies a more advance disease.

Ocular manifestations are a feature of West Nile virus infection. They mostly occur in association with severe neuroinvasive disease. Linear chorioretinitis is suggestive of the diagnosis and may raise diagnostic suspicion when associated with evocative systemic signs, and in an epidemic context. Various other less specific inflammatory ocular manifestations have been reported, including anterior uveitis, occlusive retinal vasculitis, optic neuritis, and diplopia. The pathophysiology of ocular disease remains unclear, but it reflects the neuroinvasiveness of the disease. Although ocular involvement most often resolves without visual sequelae, some patients may have permanent loss of vision, adding to the need for the development of a specific treatment and/or vaccines.

Brucellosis is a systemic zoonotic infection transmitted to humans by contact with the body fluids of infected animals or consumption of infected animal products. Although nearly eradicated in many developed countries, the disease is still endemic in Middle and South America, Central Asia, the Arabian Peninsula, Middle East, and Mediterranean regions. Any organ or system of the body may be involved. Skeletal system, spleen, liver, and bone marrow are the most frequently affected sites. Ocular involvement is reported relatively rarely, in 3.4%-26% of brucellosis cases, usually as a complication of chronic phases of the disease. Although ocular complications have been known since the early 20th century, decades after recognition of the disease, there is no convincing data about its true incidence, as most citations in the literature refer to case reports. Ocular brucellosis has severe complications and may result in total loss of vision in a quarter of patients despite appropriate treatment. A literature search conducted in PubMed on 10 September 2018 using the keywords "ocular-brucellosis" and "uveitis-brucellosis" and limiting to only human studies in the English literature published after 1950 yielded 27 and 29 articles, respectively. Of these, 14 were case reports or case series and included a total of 131 cases. We also found five case reports by searching for "oküler-brusella" and "göz-brusella", two in the Turkish Medline database (www.turkmedline.net) and four in Google search, with a total of six cases. Herein, we describe a case of ocular involvement of chronic brucellosis presenting with chorioretinitis that led to severe visual loss, and present a systematic review of previous cases reports and series in the literature. We believe that in an endemic area such as our country, routine ophthalmologic evaluation of all patients diagnosed with brucellosis and immediate initiation of therapy are necessary to prevent disease progression and severe sequelae.

PURPOSE To evaluate contrast sensitivity in patients with Behcet's disease (BD) without ocular involvement. METHODS The study group was composed of 47 BD patients (20 to 50 years of age) who did not have ocular involvement. The control group was composed of 47 normal volunteers who were similar to the study group in terms of age and gender. No participants in this study had any ocular or systemic pathologies except for BD. The contrast sensitivity measurements were performed using the Functional Acuity Contrast Test under photopic conditions, and the results were compared between the two groups. RESULTS The mean age of the BD patients and control subjects was 34.5 ± 9.7 and 33.2 ± 7.6 years, respectively. The mean disease duration of the BD patients was 5.5 ± 6.4 years. There was a statistically significant decrease at five spatial frequencies (A, 1.5; B, 3; C, 6; D, 2; and E, 18 cycles per degree) in patients with BD compared with control subjects (p < 0.001, p = 0.004, p = 0.002, p < 0.001, and p = 0.001, respectively). CONCLUSIONS The contrast sensitivity of BD patients without ocular involvement was lower than that of the control group. Further studies seem mandatory to confirm our results.

Findings of several reports suggest that rituximab, a chimeric monoclonal anti-CD20 antibody causing B-lymphocyte depletion, might represent a treatment option for people with granulomatosis with polyangiitis (GPA) (former Wegener's granulomatosis). This study presents the results of rituximab treatment in two patients with treatment-refractory GPA. First patient received rituximab for a granulomatous posterior orbital mass lesion, and eye symptoms were resolved after three courses of treatment. The second patient had eye and paranasal sinus involvement and benefited from two courses of rituximab treatment, with significant clinical improvement. Rituximab may represent an effective novel treatment for remission induction in GPA.

Optic neuritis (ON) may be associated to a range of autoimmune or infectious diseases. We report herein a case of ON induced by Rickettsia conorii. A 53-year-old woman presented with a recent decrease in visual acuity and headache. ON was diagnosed on the basis of ophthalmologic examination and flash visual evoked potentials. Etiological investigation made in our department eliminated first autoimmune disorders (vasculitis and connective tissue diseases). Rickettsial optic neuritis was confirmed by detection of specific antibodies in serum and the negativity of other serologic tests. An association between corticosteroids and cyclines was prescribed with improvement of visual acuity.

PubMedID: 1488888 Abstract HLA B5 plays an important role in the aetiology of Behçet's disease. The frequency of HLA B5 differs between racial groups and geographical regions. Ocular involvement, which could lead to serious complications, is a very important feature of Behçet's disease. Various racial groups having different genetic properties live in the Cukurova region, located in southern Turkey. To investigate the role of HLA B5 in Behçet's disease in Cukurova region, HLA B5 antigens were investigated by lymphocytotoxicity assay in 65 patients and 200 healthy controls. A significantly higher frequency was found in the patient group. Neither ocular involvement, nor the type of uveitis showed any correlation with HLA B5, but it was found that patients presenting with frequent ocular attacks had significantly higher HLA B5 positivity than the patients with rare attacks. This observation suggests that HLA B5 would be a negative factor in visual prognosis in Behçet's disease. 1992 Institution Acta Ophthalmologica Scandinavica

A case report of a 31 year-old woman from Paraíba State (North-Eastern Brazil) that presented severe involvement of ocular globes, ears and meninges. Diagnosis was established after enucleation of her left eye, when adult worms were seen in the midst of a granulomatous inflammatory process. Her response to the initial treatment with levamisole and cambendazole was good, but there was a relapse after the fifth month of treatment even with maintenance doses of both medications. She later received ivermectin and albendazol and responded well. Paciente do sexo feminino, com 31 anos, procedente da Paraíba, apresentava envolvimento severo de ambos os globos oculares, com perda da visão à esquerda, comprometimento da audição e das meninges. Após enucleação do olho esquerdo fragmento de helminto identificado como Lagochilascaris minor foi observado em processo inflamatório granulomatoso. Inicialmente a paciente foi tratada com levamisol e cambendazol, com bom resultado. Verificou-se, todavia, piora do quadro após cinco meses, com eliminação de larvas do ascarídeo em lesão presente na órbita esquerda, embora a medicação fosse mantida com administração periódica. Houve boa resposta terapêutica, com regressão do quadro, após substituição dos anti-helmínticos anteriores pela associação ivermectina e albendazol.

Purpose: To investigate the value of color Doppler imaging (CDI) of orbital vasculature in the assessment of ocular involvement in patients with Behçt's disease (BD) without clinical ophthalmologic abnormalities. Methods: CDI of the orbital vessels were performed on 26 eyes of 13 patients who were diagnosed as having BD with ocular involvement (group 1), 65 eyes of 33 patients who had BD without ocular involvement (group 2) and 40 eyes of 20 healthy volunteers (group 3). Peak systolic (PSV) and end-diastolic (EDV) blood flow velocities and resistivity index (RI) measurements were obtained for the ophthalmic artery (OA) and central retinal artery (CRA). The mean velocity of the central retinal vein (CRV) was also measured. Results: For the OA, PSV and EDVs were significantly lower and RIs were significantly higher in group 2 than in control subjects. In group 1, only the EDVs of OA were significantly lower than in healthy subjects. For the CRA, PSV and EDVs were significantly lower, and RIs were significantly higher in both BD groups than those in group 3. When group 1 and group 2 were compared, the differences between PSV, EDV and RI measurements for the CRA and OA were statistically insignificant. There was no significant difference in blood flow velocity of the CRV between the three groups. Conclusion: Major hemodynamic changes were observed in the ophthalmic vasculature of Behçt's patients with or without ocular involvement by CDI. CDI may detect ocular blood flow alterations before initial clinical manifestations. © Springer Science+Business Media, Inc. 2007.

The aim of this manuscript is to describe radiological findings of extra-pulmonary sarcoidosis. Sarcoidosis is an immune-mediated systemic disease of unknown origin, characterized by non-caseating epitheliod granulomas. Ninety percent of patients show granulomas located in the lungs or in the related lymph nodes. However, lesions can affect any organ. Typical imaging features of liver and spleen sarcoidosis include visceromegaly, with multiple nodules hypodense on CT images and hypointense on T2-weighted MRI acquisitions. Main clinical and radiological manifestations of renal sarcoidosis are nephrolithiasis, nephrocalcinosis, and acute interstitial nephritis. Brain sarcoidosis shows multiple or solitary parenchymal nodules on MRI that enhance with a ring-like appearance after gadolinium. In spinal cord localization, MRI demonstrates enlargement and hyperintensity of spinal cord, with hypointense lesions on T2-weighted images. Skeletal involvement is mostly located in small bone, showing many lytic lesions; less frequently, bone lesions have a sclerotic appearance. Ocular involvement includes uveitis, conjunctivitis, optical nerve disease, chorioretinis. Erythema nodosum and lupus pernio represent the most common cutaneous manifestations encountered. Sarcoidosis in various organs can be very insidious for radiologists, showing different imaging features, often non-specific. Awareness of these imaging features helps radiologists to obtain the correct diagnosis. • Systemic sarcoidosis can exhibit abdominal, neural, skeletal, ocular, and cutaneous manifestations. • T2 signal intensity of hepatosplenic nodules may reflect the disease activity. • Heerfordt’s syndrome includes facial nerve palsy, fever, parotid swelling, and uveitis. • In the vertebrae, osteolytic and/or diffuse sclerotic lesions can be found. • Erythema nodosum and lupus pernio represent the most common cutaneous manifestations.

Clinical case A 43-year-old woman in remission from T-cell acute lymphoblastic leukaemia was referred to our hospital with suspected leukaemic retinitis. The funduscopic examination of her left eye revealed multifocal yellow-white peripheral retinitis and retinal hemorrhage. The patient was treated for cytomegalovirus retinitis after an extended hematological investigation showed no abnormalities. Initial improvement was followed by papillitis in the left eye and motility restriction in the right eye. Magnetic resonance and lumbar puncture confirmed leukaemia relapse. Specific treatment was initiated with complete resolution. Discussion Ocular involvement may precede hematological leukaemia relapse. Physicians should be alerted when ocular symptoms appear in these cases.

Adamantiades-Behçet’s disease is a multisystemic vasculitis with multiorgan involvement. Ocular disorders occur often in this syndrome typically in the form of a relapsing-remitting panuveitis and vasculitis and can lead to blindness as one of its most disabling complications if left untreated. There are known risk factors related with the worst visual prognosis, which require early and intensive treatment in order to obtain a rapid suppression of inflammation and to prevent future relapses. The management strategy to avoid vision loss and blindness currently involves the use of local and systemic drugs including steroids and immunosuppressive and biologic agents. This review aims to demonstrate how the introduction and the use of biologic agents improves the visual outcome of patients with Adamantiades-Behçet’s disease.

Background: KL-6 is a human glycoprotein secreted by type II alveolar cells in the lung, and its serum levels increase in pneumonia of various causes. We previously reported that serum KL-6 levels in uveitis patients with sarcoidosis were significantly higher than those of other uveitis patients and healthy controls. Additionally, the combined measurement of serum KL-6 and angiotensin converting enzyme (ACE) was useful for screening uveitic patients to diagnose sarcoidosis. The purpose of the present study was to investigate the clinical usefulness of quantifying serum KL-6 levels for following-up the patients with sarcoidosis. Methods: Sera were obtained from 36 uveitic patients diagnosed with sarcoidosis and the same number of healthy volunteers. To examine the influence of systemic medication, we also collected blood samples from four more sarcoidosis patients, who were systemically treated with corticosteroid or angiotensin converting enzyme (ACE) inhibitor, an anti-hypertensive drug. The serum concentration of KL-6 was measured by a human KL-6 electrochemiluminescence immunoassay (ECLIA). Results: The mean KL-6 concentrations of sarcoidosis patients and healthy controls were 449.3 +/- 66.3 (mean +/- SE) and 192.1 +/- 11.3, respectively. The average levels of serum KL-6 were significantly elevated in sarcoidosis patients compared with healthy control subjects (P < 0.001), and there were significant correlations between serum KL-6 and ACE levels in the patients with sarcoidosis (r=0.70 and P < 0.0001). Moreover, serum KL-6 concentrations were less affected by systemic corticosteroid, and unaffected by ACE inhibitory drugs in contrast to ACE levels. Conclusions: Measurement of serum KL-6 in the uveitic patients may be useful to follow-up the diagnosed sarcoidosis, as well as for diagnosing sarcoidosis, because the serum KL-6 level was well correlated with the ACE level, and less affected by systemic medication than ACE levels.

Background: Resistin, a recently identified adipocytokine, has been found to play an important role in inflammation and the processes of inflammation-related diseases. Serum resistin levels in patients with Behcet’s disease (BD) have not yet been investigated. We aimed to evaluate the relation between resistin and interleukin-6 (IL-6) in Behcet patients with or without ocular involvement and in normal controls. Methods: Twenty-two patients with BD and 19 healthy control subjects were included in this study. While 14 patients had posterior segment involvement of the eye, the other 8 did not have ocular disease. Serum resistin and interleukin-6 (IL-6), levels were measured in all samples. Data from all groups were tested for statistical significance. Results: The mean resistin and IL-6 concentrations were significantly higher in patients with BD than the control subjects ( p = 0.011 and p = 0.0001, respectively).There was a significant difference in resistin and IL-6 levels between the patients with non-ocular BD and controls ( p = 0.013 and p = 0.0001, respectively), as well as resistin and IL-6 levels between the ocular BD group and the control group ( p = 0.05 and p = 0.0001, respectively). However, there was no significant difference between patients with ocular versus non-ocular BD. Interpretation: Resistin levels were found to be raised in Behcet patients with or without ocular involvement compared with the control subjects.

Sarcoidosis is a multisystem inflammatory disease of unknown etiology affecting multiple organs. Earlier reports suggested that sarcoidosis was a disease of the developed world. However, recent reports suggest that the disease is found in the developing countries as well. Clinical, radiological, and histopathological similarities with tuberculosis pose a great challenge in countries endemic for tuberculosis. Mantoux test, high resolution computed tomography, and transbronchial lymph node and lung biopsies are diagnostic modalities, which play an important role in the diagnosis of sarcoid. In this review, we look at the epidemiology of sarcoid in tuberculosis-endemic regions, the sarcoidosis-tuberculosis link, clinical profile, diagnostic modalities, dilemma in the diagnosis, and the treatment of this disease.

PubMedID: 12648516 Leukemias are systemic hematopoietic neoplasias and not infrequently cause ocular findings. Serous retinal detachment (SRD) is one of these manifestations and even may be the first sign of the underlying leukemia. Here we reported a case with chronic lymphocytic leukemia (CLL) presenting with SRD and discussed the clinical importance and therapeutic options. © 2003 Elsevier Science Ltd. All rights reserved.

Background Stevens-Johnson syndrome (SJS) associated with Mycoplasma pneumoniae (M. pneumoniae) infection is mainly observed in children. In adults, drugs are a major cause of SJS, but some adult patients with SJS are infected with M. pneumoniae. We analyzed patients with SJS associated with M. pneumoniae infection to elucidate the differences between drug-induced SJS and M. pneumoniae-associated SJS and also to study differences between M. pneumoniae-associated SJS in children and adults. Methods This is a retrospective review of Japanese patients who have been reported as M. pneumoniae- associated SJS in medical Journals published from 1981 to 2009, compared with data of Japanese patients with drug-induced SJS reported from 2000 to 2009. Results Thirty-eight cases of M. pneumoniae-associated SJS and 78 cases of drug-induced SJS were analyzed in this study. Ocular lesions were observed more frequently in M. pneumoniae-associated SJS than in drug-induced SJS (p 0.01), and adult patients showed a higher ratio of sequelae in their eyes than did patients under 20 years of age (p 0.01). Sixty-six percent of adult patients with M. pneumoniae-associated SJS developed fever/respiratory symptoms and mucocutaneous lesions on the same day. In contrast, most of the patients under 20 years of age developed fever/respiratory symptoms before mucocutaneous involvement. This means that these adult patients were infected and immunized previously and developed allergic reactions to M. pneumoniae soon after the later infection. Conclusions In order to prevent ocular sequelae in adult patients when M. pneumoniae infection is suspected, more intensive treatment may be needed in adult patients than in younger patients.

We describe 2 adult patients with a subepidermal bullous dermatosis with exclusively linear IgA depositions along the epidermal basement membrane zone that were deposited in the sublamina densa zone as witnessed by direct immunoelectron microscopy. Indirect immunofluorescence microscopy of patients' sera revealed circulating IgA autoantibodies that bound exclusively to the dermal site of salt-split skin substrate. Immunoblot analysis using dermal and keratinocyte extracts were negative. Indirect immunofluorescence microscopy using type VII collagen-deficient skin ("knockout" substrate) showed no IgA binding, whereas linear IgA binding was seen at the epidermal basement membrane zone in normal human skin. The autoantigen in the patients was thus type VII collagen. A diagnosis of IgA-mediated epidermolysis bullosa acquisita (IgA-EBA) was made. We systematically reviewed the literature of this subset of patients with linear IgA dermatosis on the basis of the following criteria: exclusive binding of serum-IgA to the dermal side of salt-split skin or IgA depositions in the sublamina densa zone by indirect or direct immunoelectron microscopy. We learned that IgA-EBA is clinically indistinguishable from the classic "lamina-lucida type" linear IgA dermatosis or from the inflammatory type of IgG-mediated epidermolysis bullosa acquisita (IgG-EBA). Only a minority of the patients with IgA-EBA showed milia or scarring or had therapy-resistant ocular symptoms as in the mechanobullous type of IgG-EBA. Most patients with IgA-EBA responded to dapsone therapy.