Ethnicity and association with ocular, systemic manifestations and prognosis in 194 patients with sarcoid uveitis.

OBJECTIVE: To determine the ophthalmological and extra-ophthalmological clinical characteristics and visual prognosis of patients with sarcoid uveitis in different ethnic groups. METHODS: We retrospectively analysed the data from patients with sarcoid uveitis seen at two departments of Ophthalmology between December 2003 and December 2017. Patients presented biopsy-proven sarcoidosis and/or presumed sarcoid uveitis based on the following criteria: compatible thoracic imaging, associated with elevated angiotensin-conversion enzyme (ACE) and/or lymphocytic alveolitis on bronchoalveolar lavage fluid analysis (> 15% lymphocytes and CD4/CD8 > 3.5). Ophthalmological and general characteristics, as well as visual and global prognoses, were compared in three pre-defined ethnic groups: White Europeans, North Africans and Afro-Caribbeans. RESULTS: A total of 194 patients were included: 145 with biopsy-proven and 49 with presumed sarcoid uveitis. Overall, 68% were White Europeans while 20.6% were North Africans and 11.3% were Afro-Caribbeans. Sixty-nine per cent were women and the median age at presentation was 52.1 years. Median ages at first ocular manifestation of the disease in Afro-Caribbeans and North Africans were respectively 34.3 and 43.1 years, while it was 57.8 years in White Europeans (p < 0.001). Ocular involvement was bilateral in 77.8% (n = 151) of the cases and nearly half of the patients had panuveitis (48.5%). Anterior uveitis was more frequent in Afro-Caribbeans (59.1%; p < 0.0001), while White Europeans presented more frequently with intermediate uveitis. There was a significantly higher frequency of systemic involvement of sarcoidosis in North Africans while White Europeans showed a higher frequency of isolated ocular involvement at onset and during follow-up. Afro-Caribbeans, who had a complete visual recovery in 72.7% of the cases, had a better visual prognosis than other ethnic groups (p = 0.025). CONCLUSION: In this large European series of sarcoid uveitis, we observed ethnicity-related differences regarding uveitis clinical presentation and visual outcome. Although good overall, the visual prognosis seems to be better in Afro-Caribbeans than in other ethnic groups.

Sarcoidosis-related mortality in France: a multiple-cause-of-death analysis.

We evaluated mortality rates and underlying causes of death among French decedents with sarcoidosis from 2002 to 2011.We used data from the French Epidemiological Centre for the Medical Causes of Death to 1) calculate sarcoidosis-related mortality rates, 2) examine differences by age and gender, 3) determine underlying and nonunderlying causes of death, 4) compare with the general population (observed/expected ratios), and 5) analyse regional differences.1662 death certificates mentioning sarcoidosis were recorded. The age-standardised mortality rate was 3.6 per million population and significantly increased over the study period. The mean age at death was 70.4 years (versus 76.2 years for the general population). The most common underlying cause of death was sarcoidosis. Sarcoidosis decedents were more likely to be males when aged <65 years. When sarcoidosis was the underlying cause of death, the main other mentions on death certificates were chronic respiratory and cardiovascular diseases. The overall observed/expected ratio was >1 for infectious disease, tuberculosis and chronic respiratory disease, and <1 for neoplasms. We observed a north-south gradient of age-standardised mortality ratio at the country level.Despite the limitation of possibly capturing the more severe cases of sarcoidosis, this study may help define and prioritise preventive interventions.

Acute anterior uveitis and undiagnosed spondyloarthritis: usefulness of Berlin criteria.

PURPOSE: Inflammatory back pain, the main symptom in spondyloarthritis, is defined by the presence of two or more positive responses among the four items of Berlin Criteria. This study assesses the value of these criteria in detecting previously undiagnosed spondyloarthritis after an episode of acute anterior uveitis. METHODS: Records of patients addressed for etiological diagnosis after an acute anterior uveitis (April 2006 to November 2013) were retrospectively analysed. Patient characteristics, the presence of back pain, Berlin Criteria, the final diagnosis, and the ASAS classification criteria for spondyloarthritis were collected and analysed. RESULTS: The study included 102 patients (59.8% women). The mean age was 44.5 years. Uveitis cases were mainly unilateral (73.5%) and recurrent (58.8%). Twenty-one patients had some type of spondyloarthritis, 20 fulfilling retrospectively the ASAS criteria. Back pain with at least two positive items from Berlin Criteria was 61.9% sensitive and 97.5% specific in diagnosing spondyloarthritis. Considering only one positive item increased the sensitivity (90.5%) but decreased the specificity (91.4%). CONCLUSIONS: Acute anterior uveitis may be the key symptom that reveals a formerly undiagnosed spondyloarthritis. A referral to a rheumatologist should be considered in presence of back pain, even without fulfilment of the classical definition with Berlin Criteria.

[The daily life of patients at the Hôtel-Dieu in Lyon in the nineteenth century]. / La vie quotidienne des patients à l'hôtel-Dieu de Lyon au XIXème siècle.

In 1802 the Hôtel-Dieu in Lyons was incorporated in the so-called Hospices Civils de Lyon. This allowed the expansion and renovation of buildings, as well as the improvement of the conditions of hygiene and comfort of the patients. This hospital was devoted only to the most severely ill or injured adults. 1100 patients were treated by seven doctors, a main surgeon and his deputy, residents and sisters. Broadly speaking the evolution of surgery can be divided into two periods: that of before anesthesia and septic surgery and that of antiseptic and aseptic surgery. We have to mention Gensoul and the resection of the maxillary before anesthesia, Bonnet and Ollier who were devoted to osteo-articular surgery (Ollier's disease), Poncet who built the first aseptic theater, Jaboulay and the resident Carrel who were transplantation's pioneers, Bouveret (paroxysmal tachycardia and Bouveret syndrome), Destot who did the first medical use of X-rays in 1895.

Ocular sarcoidosis: when should labial salivary gland biopsy be performed?

PURPOSE: To assess the usefulness of a labial salivary gland biopsy (LSGB) in subsets of patients with uveitis. METHODS: A retrospective study of 115 consecutive patients with uveitis for whom a LSGB had been done because of suspected ocular sarcoidosis (n = 86) or unexplained uveitis (n = 29). Eighty-six patients had a suspicion of ocular sarcoidosis because of ocular features (n = 67), an elevated angiotensin converting enzyme (ACE) (n = 29), or because of CT findings (n = 32) suggestive of sarcoidosis. The biopsy results were analyzed together with their ophthalmological features and the results of other relevant examinations, such as the serum levels of ACE and a chest radiography or a CT scan. RESULTS: Six of the 115 patients (5.2 %) with uveitis had sarcoid granulomas on the LSGB. At the end of the study, 32 patients had proven sarcoidois while 22 patients were considered as having either indeterminate or presumed sarcoidosis, according to the criteria of Abad et al. A raised ACE (p = 0.016) and a compatible radiology (p = 0.033) were related to a positive LSGB test, but not to the features of uveitis. Granulomas were only found in the LSGB of the patients with an elevated ACE or compatible CT scans. CONCLUSIONS: In this study, the LSGB sensitivity (18.75 %) in the patients with proven sarcoidosis appears to be lower than in other reports. Our results suggest that this investigation is a possible method of tissue diagnosis in patients with raised ACE and/or CT scan pattern compatible with sarcoidosis, and should not be performed in patients with unexplained uveitis or because of their ophthalmological features.

Coexisting relapsing polychondritis and sarcoidosis: an unusual association.

Relapsing polychondritis is an episodic and progressive systemic inflammatory disease characterized by auricular chondritis, polyarthritis, nasal and respiratory tract chondritis. About 30% of the patients have additional autoimmune and or hematological diseases, most frequently systemic vasculitis, rheumatoid arthritis, myelodysplastic syndromes or systemic lupus erythematosus. So far, only one case of coexisting relapsing polychondritis and sarcoidosis in a patient with AIDS has been reported. We describe here a case of sarcoidosis and relapsing polychondritis in an immunocompetent patient. Physicians should be aware of this possible association.

Acute ophthalmoparesis associated with human parvovirus B19 infection.

PURPOSE: Parvovirus B19 infection (PVB19) has been linked with a broad spectrum of clinical syndromes. In addition to erythema infectiosum and asymptomatic infection, other less common manifestations include transient aplastic crisis in patients with hemoglobinopathies, pure red cell aplasia and pancytopenia in immunocompromised persons, nonimmune hydrops fetalis, chronic arthritis, myocarditis, and hepatitis. METHODS: Only 19% of patients had peripheral nervous system damage, mainly including brachial plexitis and carpal tunnel syndrome. Two cases of cranial nerves palsies have been described in children in the literature, including one case of peripheral facial nerve palsy and one case of velopalatine hemiparalysis. We report the first case of acute ophthalmoparesis associated with PVB19 infection. RESULTS: We present a 40-year-old man with PVB19 with acute sixth cranial nerve palsy, diagnosed on the basis of serology and polymerase chain reaction carried out both on serum and cerebrospinal fluid. CONCLUSIONS: Clinicians should be aware of this possible clinical presentation.

Lyme-associated uveitis: Clinical spectrum and review of literature.

BACKGROUND: Clinical presentation of Lyme-associated uveitis is poorly described. We reported here a case series of seven patients with uveitis related to Lyme disease and a review of the literature. METHODS: A retrospective study in our university hospital between 1 May 2003 and 31 July 2016 on 1006 uveitis patients and review of Pubmed library. RESULTS: Seven patients (71.4% male, mean age = 53 (38-70)) were diagnosed with a Lyme-associated uveitis. All anatomical types of uveitis were found (four intermediate, three anterior, and three posterior uveitis); most were unilateral (n = 6; 85.7%), one granulomatous and two with synechiae. Peripheral retinal vasculitis was present in four patients. They all had a risk of exposure or extra-ophthalmological symptoms. Antibiotic and steroid treatment was rapidly effective in all patients. Four patients presented recurrences of uveitis, of whom two received a second antibiotic treatment, which is quite common in literature. Persistent or recurrence of symptoms can be explained by three hypotheses: (1) reinfection, (2) relapse of original infection, and (3) autoimmune reaction. CONCLUSION: Lyme-associated uveitis appears varied. Hyalitis and involvement of the posterior segment and retinal vasculitis seem to be rather frequent. Its prognosis is mainly good, even if inflammation can be resistant or recurring.

Sarcoidosis and melanoma: a referral center study of 1,199 cases.

BACKGROUND: Case reports and small short case series have described patients with sarcoidosis and melanoma. OBJECTIVES: To examine the relation between sarcoidosis and melanoma. METHODS: Computerized search of all patients seen at our department of dermatology between 1998 and 2007 and review of the literature using a Medline search from 1985 to June 2008. RESULTS: We identified 7 cases in our population of 1,199 melanoma inpatients. Including these cases, 20 cases of sarcoidosis have been described in melanoma patients. Fifteen patients had their sarcoidosis diagnosed after melanoma. In 7 cases, sarcoidosis was related to immunotherapy. Sarcoidosis presented mainly as pulmonary disease without severe organ involvement, with a benign evolution. CONCLUSION: Our referral center study shows a prevalence of sarcoidosis of 0.58% among melanoma inpatients. Clinicians should be aware of the possibility that sarcoidosis may initially manifest itself in melanoma patients, especially after treatment with immunotherapy, and mimic metastatic disease.

BTNL2 gene polymorphism and sarcoid uveitis.

BACKGROUND: Uveitis is a frequent and early feature of sarcoidosis. As BTNL2 (butyrophilin-like 2) gene polymorphism was found linked with the susceptibility to sarcoidosis, we investigated whether a specific genotype of BTNL2 gene G16071A (or rs2076530) single-nucleotide polymorphism (SNP) would be associated with the risk of sarcoid uveitis in all patient subgroups. METHODS: The study compared the genotype frequencies of SNP G16071A of 135 patients with sarcoid uveitis (Sa+Uv+) with those of 196 patients with sarcoidosis without uveitis (Sa+Uv-), 81 patients with uveitis without sarcoidosis (Sa-Uv+), and 271 controls with no sarcoidosis nor uveitis (Sa-Uv-). Three hypothetical subgroups of patients with sarcoid uveitis (Sa+Uv+ cases) were considered: (1) subgroup I: patients aged <45 years of both sexes and all ethnic origins; (2) subgroup II: Caucasian women aged >45 years; and (3) subgroup III: all other patients. RESULTS: A statistically significant difference in genotype frequencies was found between the groups Sa+Uv- and Sa-Uv- (p=3.2×10-6) and between the groups Sa+Uv+ and Sa+Uv- (p=7.1×10-3). There was no difference between the three subgroups of Sa+Uv+ patients. There was a statistically significant difference in genotype frequencies between Sa+Uv- and Sa+Uv+ subgroup II (p=0.005) but no difference between Sa+Uv- and Sa+Uv+ subgroup I. CONCLUSION: No association was found between G16071A and the susceptibility to sarcoid uveitis. BTNL2 gene G16071A SNP seems to be a predisposing factor for sarcoidosis except in Caucasian postmenopausal women with sarcoid uveitis in whom the GG genotype prevails. These and future results will help in understanding differences between particular subgroups of patients with sarcoid uveitis.

Uveitis of Unknown Etiology: Clinical and Outcome features. A Retrospective Analysis of 355 Patients.

Purpose: Despite the huge advance in diagnostic technics, about one-third of uveitis is still considered of unknown etiology. In this study, we aimed to report their clinical features and to describe how a diagnosis has been finally reached for some patients.Methods: We retrospectively reviewed all patients with uveitis referred to our tertiary center between 2002 and 2016. The unknown etiology was admitted after a new ophthalmologic examination and a full work-up in internal medicine in our tertiary center.Results: Among 957 patients with uveitis, 355 had uveitis of unknown etiology. The clinical and epidemiological characteristics of this subgroup were no different from those with a known etiology. Out of 104 patients who were followed-up for more than 1 year, a diagnosis was finally achieved in 20 patients. The diagnosis was determined either because of the occurrence of a new clinical symptom (n = 10), a new/repeated non-ophthalmologic investigation (n = 7), or a new/repeated ophthalmic exam (n = 3).Conclusion: A prolonged follow-up, with repeated exams, may allow the determination of an etiology in about one-fifth of uveitis initially considered as idiopathic.

18F-fluorodeoxyglucose positron emission tomography is useful for the diagnosis of intraocular sarcoidosis in patients with a normal CT scan.

AIM: To assess the usefulness of 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET/CT) and the predictive factors for the diagnosis of sarcoidosis in patients with uveitis who have normal thoracic tomography. METHODS: We retrospectively reviewed 67 consecutive patients with uveitis of unknown aetiology or a suspected sarcoidosis. All patients with normal thoracic tomography underwent an 18F-FDG PET/CT, which was blindly reinterpreted. We then assessed the proportion of positive 18F-FDG PET/CT and the impact on the final aetiology, using Abad's criteria for the diagnosis of intraocular sarcoidosis. RESULTS: 19 of the 67 patients (28.4%) had mediastinal hypermetabolic foci on their 18F-FDG PET/CT consistent with sarcoidosis. It identified a biopsy site in two cases, which were consistent with sarcoidosis. At the end of the study, six patients (10%) had a proven sarcoidosis, six patients (9%) were considered as having a presumed sarcoidosis and 18 patients (26.9%) as having indeterminate sarcoidosis. 18F-FDG PET/CT enabled the diagnosis of presumed sarcoidosis in these six patients. An older age at diagnosis (p=0.004) and the presence of synechiae (p=0.02) were significantly related to an abnormal 18F-FDG PET/CT, with a trend for an elevated ACE (p=0.0993). We established a nomogram to estimate the probability of having positive findings on the 18F-FDG PET/CT according to different predictive factors. CONCLUSION: 18F-FDG PET/CT enabled the diagnosis of intraocular sarcoidosis even in patients with a normal CT scan. Older age at diagnosis, presence of synechiae and elevated ACE are associated with positive findings on 18F-FDG PET/CT consistent with sarcoidosis.

Autoimmune hemolytic anemia and common variable immunodeficiency: a case-control study of 18 patients.

To describe the main characteristics and treatment of autoimmune hemolytic anemia (AHA) in patients with common variable immunodeficiency (CVID), we analyzed data from 18 patients, 4 from an earlier study and 14 from the French DEF-I cohort on adult patients with primary hypogammaglobulinemia. To be included, patients had to have CVID and a previous history of AHA with a hemoglobin level < or =90 g/L at onset. To determine whether AHA is associated with a particular clinical phenotype of CVID, we conducted a case-control study from the DEF-I cohort. The estimated frequency of AHA in CVID patients from the DEF-I cohort was 5.5% (14/252). Median age at AHA diagnosis was 26 years (range, 1-57 yr), and 27.5 years (range, 5-61 yr) at CVID diagnosis. CVID was diagnosed before the onset of AHA in only 2 patients (11%). CVID was diagnosed more than 6 months after AHA in 10 cases (55.5%), and the 2 conditions were diagnosed concomitantly in 6 cases. The 14 patients included in the DEF-I cohort were compared with 238 control patients with CVID but without AHA. Corticosteroids were used as initial treatment for all patients in the current study. An initial response was obtained in 15 of 18 (83%) patients. Overall, 9 of these (60%) achieved a lasting response with steroids alone (7 patients) or in combination with intravenous immunoglobulin (2 patients). Seven patients underwent splenectomy, and 5 additional splenectomies were performed for associated autoimmune thrombocytopenic purpura. After splenectomy, a lasting response was obtained in 3 of the 7 patients with AHA. However, 5 of the 12 splenectomized patients experienced life-threatening infection. Severe infection occurred in 2 of 4 patients receiving immunosuppressive drugs. At the end of follow-up, 13 of 18 (72%) patients were in treatment-free remission (13 complete responses), and 4 of 18 (22%) were in remission while on prednisone < or =20 mg/d. One patient had died, of cancer.

A Comparative Study of Characteristics and Outcomes of Patients with Proved and Suggested Sarcoid Uveitis Occurring after Ophthalmic Procedure.

PURPOSE: To describe patients with new onset sarcoid uveitis occurring after an ophthalmic procedure and compare them with patients with sarcoid uveitis without ocular procedure. METHODS: Retrospective analysis of case records from patients with postophthalmic procedure sarcoid uveitis seen at our institution between April 2004 and October 2016. Patients with a previous history of uveitis were not included. Each patient was randomly matched with four controls from our incident cohort of new onset sarcoid uveitis without ophthalmic procedure. RESULTS: We identified 11 patients (8.5%) from our incident cohort of sarcoid uveitis (n=130), who were all women, with a postophthalmic procedure uveitis (mostly after cataract surgery (36%)). These patients were older (69.7 vs 52.7 years) and presented more synechiae than controls. After a mean follow-up of 30 (3-60) months, there was no significant difference between the postprocedure and the control group with regard to demography, clinical presentation, disease course, treatment, and outcome. CONCLUSIONS: Sarcoid uveitis has similar characteristics in patients with new onset sarcoid uveitis after or without ophthalmic procedure. As a consequence, ophthalmic intervention should be seen as a potential trigger of latent sarcoidosis.

Diagnosis of Lyme-associated uveitis: value of serological testing in a tertiary centre.

AIMS: To determine the frequency and clinical presentation of Lyme disease in patients with uveitis and to assess the value of Borrelia burgdorferi serological testing. METHODS: Retrospective study on all patients with uveitis who were referred to our tertiary hospital were serologically tested for Lyme in our laboratory between 2003 and 2016. Screening consisted of determining B. burgdorferi serum IgG and IgM by ELISA method. The patient's serology was considered as positive if the ELISA-positive result in IgM and/or IgG was confirmed by an immunoblot positive in IgM and/or IgG. Lyme-associated uveitis was diagnosed based on serological results as well as response to antibiotics and exclusion of other diagnosis. RESULTS: Of the 430 patients with uveitis (60% women, mean age 49 years) fulfilling inclusion criteria, 63 (14.7%) had an ELISA-positive serology, confirmed by immunoblot for 34 patients (7.9%). The diagnosis of Lyme-associated uveitis was finally retained in seven patients (1.6%). These patients reported either a previous exposure including tick bite or forest walks (n=5), symptoms suggestive of Lyme disease (n=5) and resistance to local and/or systemic steroids (n=7). Among the remaining 27 positive patients, 22 had other established aetiologies and 5 other were unclassified. CONCLUSION: The seroprevalence of B. burgdorferi among our patients with uveitis was 7.9% compared with 6 to 8.5% in the general French population which leads to a low predictive value of serological testing. Its use should be reserved for patients with unexplained uveitis, an exposure history, systemic findings suggestive of Lyme disease and steroids resistance.

Contribution of diagnostic tests for the etiological assessment of uveitis, data from the ULISSE study (Uveitis: Clinical and medicoeconomic evaluation of a standardized strategy of the etiological diagnosis).

PURPOSE: ULISSE is the only study that prospectively assessed the efficiency of a standardized strategy, compared to an open strategy for the etiologic diagnosis of uveitis. Our aim was to evaluate the diagnostic yield of the tests prescribed in the ULISSE study to clarify their relevance. METHODS: ULISSE is a non-inferiority, prospective, multicenter and cluster randomized study. The standardized strategy is a two-steps strategy: in the first step, common standard tests were performed, and in the second step, tests were guided by the clinical and anatomic type of uveitis. We reported the relevance of the diagnostic tests used in the standardized strategy, as well as the profitability of the tests that were prescribed to more than twenty patients in each group. Based on diagnostic criteria, either an ophthalmologist, or an internist, established the profitability of a test by considering whether the test lead to a diagnosis or not. RESULTS: Among the 676 patients included (standardized 303; open 373), a diagnosis was made for 152 (50.4%) in the standardized group and 203 (54.4%) in the open group. The most common entities were HLA-B27 associated uveitis (22%), spondyloarthritis (11%), sarcoidosis (18%), tuberculosis (10.7%) and herpes virus infections (8.5%). Among the first step's systematic tests, tuberculin skin test was the most contributive investigation (17.1%), followed by chest X-ray (8.4%), C reactive protein and ESR (6.6% and 5.1%), complete blood count (2.2%) and VDRL (2.0%). The second step's most often contributive tests were: HLA B27 (56.3%), chest-CT (30.3%) and angiotensin converting enzyme (ACE) (16.5%). HLA B27 and ACE were significantly more contributive in the standardized group than in the open group. Immunological tests were never contributive. Among the free investigations, or among the investigations guided by clinical or paraclinical findings, the most often contributive tests were: Quantiferon® (24%), electrophoresis of serum protein (7.8%) and sacroiliac imagery (46.4%). Intracellular serologies (1.7%), serum calcium (2.1%) and hepatic tests (3.3%) were exceptionally contributive. Among the third intention tests, labial salivary gland biopsies were contributive in 17.9% of cases, but the profitability of other invasive investigations (anterior chamber tap, vitrectomy, bronchoscopy and lumbar puncture) or specialized imagery (18F-FDG PET, Brain MRI) could not be determined since these test were rarely performed. CONCLUSION: Only a few diagnostic tests are useful for the etiological assessment of uveitis. They are often cheap, simple, more often guided by the clinical findings, and lead to an etiological diagnosis in most patients. On the other hand, some tests are never or exceptionally contributive, such as immunological tests or intracellular serologies. Further studies are required to evaluate the profitability of third intention imagery and invasive investigations.

Lofgren syndrome and peritoneal involvement by sarcoidosis: case report.

OBJECTIVE: An unusual case of a patient with Löfgren syndrome peritoneal involvement by sarcoidosis. PATIENT AND METHODS: A 36-year-old woman presented with Löfgren syndrome and an increase in liver enzyme levels. An abdominal CT scan showed multiple nodules on the peritoneum mimicking peritoneal carcinomatosis. Laparoscopy was conducted with biopsy of the peritoneal nodules. RESULTS: Biopsy specimens from the peritoneum, liver, and bronchi showed noncaseating granulomas, and the search for tuberculosis was negative. Clinical and biological features resolved within 6 months, without therapy with steroids, while a thoracic CT scan as well as an abdominal CT scan showed no change. CONCLUSION: To our knowledge, this is the first reported case of peritoneal sarcoidosis associated with Löfgren syndrome. A longer follow-up will, however, be required to assess the chronicity of the disease.

Successful treatment with rituximab in a patient with mental nerve neuropathy in primary Sjögren's syndrome.

We present the case of a 79-year-old female patient with chronic obstructive pulmonary disease on home oxygen therapy who was diagnosed as having Sjögren's syndrome with arthralgias and painful sensory neuropathy. She subsequently developed bilateral numb chin syndrome. Because of refractory and disabling symptoms under high corticosteroids and severe chronic obstructive pulmonary disease, we decided to treat her with rituximab rather than immunosuppressants. This resulted in significant subjective improvement in sicca syndrome while arthalgias, distal and chin paresthesias resolved completely. To our knowledge this is the first description of such a case.

Efficacy of Methylprednisolone Pulse Followed by Oral Prednisone in Birdshot Chorioretinopathy.

PURPOSE: To report the outcomes of initial methylprednisolone pulse then oral prednisone in the treatment of birdshot chorioretinopathy (BSCR). METHODS: We retrospectively reviewed medical records of 21 BSCR patients, treated with initial methylprednisolone, prednisone, or immunosuppressive therapy. Main outcome measures were changes in visual acuity, intraocular inflammation, concomitant immunosuppressive drug or intravitreal steroid, and adverse events. RESULTS: In total, 14 patients were initially treated with methylprednisolone; four patients with prednisone; and three with immunosuppressive drug. Intraocular inflammation was controlled in 10 of 14 patients (71%) at 1 year, with a mean dose of 6.2 mg/day prednisone. Patients treated initially by methylprednisolone had improved visual acuity at 1 year, but it was not significantly different from patients treated initially by prednisone. CONCLUSIONS: Corticosteroid therapy, including initial methylprednisolone pulse then prednisone, was effective in stabilizing vision and decreasing inflammation in most patients with BSCR.

Long-term visual and systemic prognoses of 83 cases of biopsy-proven sarcoid uveitis.

AIMS: To determine the long-term visual and systemic outcomes of uveitis patients with biopsy-proven sarcoidosis. METHODS: A retrospective study of biopsy-proven sarcoid uveitis, with a 3-year minimum follow-up, seen at Lyon University Hospital, between April 2004 and January 2016. RESULTS: A total of 83 patients were included, with a median age at onset of 52 (37-62) years and an unbalanced gender ratio (women 77.1%). Thirty-one patients had original systemic sarcoidosis in addition to ocular localisation, whereas 52 initially presented with isolated sarcoid uveitis. Among the latter, 7.7% (n=4) developed an extraocular disease after a median follow-up duration of 60 (44-110) months. The systemic spread in these patients included cutaneous sarcoids (n=2), arthritis (n=1) and multiple mononeuritis (n=1). Complete visual recovery was obtained for 60.2% of all patients and 89.2% had retrieved best-corrected visual acuity (BCVA) >20/50 in both eyes. A unilateral loss of BCVA of worse than 20/200 was documented in two patients in the isolated sarcoid uveitis group. No patient suffered from bilateral severe visual impairment or blindness. Factors linked to a poor visual prognosis, defined by BCVA ≤20/50 in at least one eye, were chronic macular oedema (p=0.009) and persistent ocular inflammation (p=0.0005). CONCLUSIONS: In this large European series of biopsy-proven sarcoidosis to date, with a prolonged follow-up, sarcoid uveitis was suggestive of a favourable systemic and visual outcome. Clinically isolated uveitis that revealed sarcoidosis remained a strictly ocular condition in most cases.