To determine the long-term visual and systemic outcomes of uveitis patients with biopsy-proven sarcoidosis.
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.
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).
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.
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.
Objective
To analyze the factors associated with response to anti–tumor necrosis factor (anti‐TNF) treatment and compare the efficacy and safety of infliximab (IFX) and adalimumab (ADA) in patients ...with refractory noninfectious uveitis.
Methods
This was a multicenter observational study of 160 patients (39% men and 61% women; median age 31 years interquartile range 21–42) with uveitis that had been refractory to other therapies, who were treated with anti‐TNF (IFX 5 mg/kg at weeks 0, 2, 6, and then every 5–6 weeks n = 98 or ADA 40 mg every 2 weeks n = 62). Factors associated with complete response were assessed by multivariate analysis. Efficacy and safety of IFX versus ADA were compared using a propensity score approach with baseline characteristics taken into account. Subdistribution hazard ratios (SHRs) and 95% confidence intervals (95% CIs) were calculated.
Results
The main etiologies of uveitis included Behçet's disease (BD) (36%), juvenile idiopathic arthritis (22%), spondyloarthropathy (10%), and sarcoidosis (6%). The overall response rate at 6 and 12 months was 87% (26% with complete response) and 93% (28% with complete response), respectively. The median time to complete response was 2 months. In multivariate analysis, BD and occurrence of >5 uveitis flares before anti‐TNF initiation were associated with complete response to anti‐TNF (SHR 2.52 95% CI 1.35–4.71, P = 0.004 and SHR 1.97 95% CI 1.02–3.84, P = 0.045, respectively). Side effects were reported in 28% of patients, including serious adverse events in 13%. IFX and ADA did not differ significantly in terms of occurrence of complete response (SHR 0.65 95% CI 0.25–1.71, P = 0.39), serious side effects (SHR 0.22 95% CI 0.04–1.25, P = 0.089), or event‐free survival (SHR 0.55 95% CI 0.28–1.08, P = 0.083).
Conclusion
Anti‐TNF treatment is highly effective in refractory inflammatory uveitis. BD is associated with increased odds of response. IFX and ADA appear to be equivalent in terms of efficacy.
To determine the clinical features of patients with uveitis with biopsy-proven sarcoidosis, document differences in these features according to ethnicity, age and sex, and assess the diagnostic value ...of biochemical and imaging examinations.
Retrospective study of 83 biopsy-proven sarcoid uveitis cases seen at two Departments of Internal Medicine and two Departments of Ophthalmology between April 2004 and March 2014.
Caucasian patients presented with uveitis at a later age than non-Caucasian (58 years vs 41 years; p=0.001) and had more often a chronic form (78.3% vs 43.8%; p=0.01). Women had higher rates of chronic macular oedema than men (48.3% vs 14.3%; p=0.01). There were no statistically significant differences between patients aged ≤50 years and patients aged >50 years. ACE levels were high (>62 U/L) in 61.7% and lysozyme levels high (>16.7 mg/L) in 83.9% of tested patients. Chest X-rays and CTs were suggestive of sarcoidosis in 62.8% and 91.2% of cases, respectively. Among 21 patients with positive tomography and negative X-rays, 13 were Caucasian women >50 years. Endoscopic ultrasound-guided fine-needle aspiration of intrathoracic nodes contributed to the diagnosis in 7 patients with normal labial salivary gland and transbronchial biopsies. Any of the enzyme tests together with any of the imaging tests identified 100% of the patients.
In this largest European series of biopsy-proven sarcoidosis to date, the outstanding diagnostic ability of enzyme test plus imaging test couple suggests that the recourse to invasive procedures should be limited to patients with ocular involvement that would justify systemic treatments.
Distinguishing between macrophage activation syndrome (MAS) and a simple flare of Still's disease (SD) may be challenging. We sought to clarify the clinical features and outcome of MAS in SD and to ...explore predictive factors of MAS development. Demographic and clinical data, treatments, and outcomes were recorded in a cohort of 206 SD patients. SD patients with and without MAS were compared. To explore predictive factors for the development of MAS, patients were compared at the time of SD diagnosis. Twenty (9.7%) patients experienced MAS, which was inaugural in 12 cases. Patients with MAS were more likely to have hepatomegaly (OR, 3.71; 95% CI, 1.14-11.2;
= 0.03) and neurological symptoms (OR, 4.43; 95% CI, 1.08-15.3;
= 0.04) than patients without MAS. Cytopenias, abnormal liver tests, and coagulation disorders were significantly more frequent in patients with MAS; lactate dehydrogenase and serum ferritin levels were significantly higher. An optimized threshold of 3500 μg/L for serum ferritin yielded a sensitivity (Se) of 85% and a negative predictive value (NPV) of 97% for identifying patients with/without MAS. Survival analysis showed that a high ferritin level at the time of SD diagnosis was predictive of MAS development (
< 0.001). Specific factors, including neurological symptoms, cytopenias, elevated LDH, and coagulopathy, may contribute to the early detection of MAS. Extreme hyperferritinemia at the onset of SD is a prognostic factor for the development of MAS.
Objective: Dermatomyositis associated with anti-MDA5 autoantibodies (DM-MDA5+) is a rare autoimmune disease usually characterized by skin involvement, often-severe lung involvement, and general ...features. Several reports of infections have been described, sometimes early after the introduction of immunosuppressive therapy. We studied the infection risk in a DM-MDA5+ population. Methods: A retrospective cohort study comparing the number and type of infections during the follow-up of 19 patients with DM-MDA5+ and 37 patients with another type of inflammatory myopathy was analyzed. Patients in both groups were matched on initial immunosuppressive therapy. We described and compared significant infectious complications (SIC) in each group. Results: Patients DM-MDA5+ had more SIC: 27 events in the DM-MDA5+ group versus 6 in the controls (HR 7.08, 95% CI 2.50−20.04, p < 0.0001). The number of SIC per patient was higher in DM-MDA5+ (1.4 ± 1.57 vs. 0.16 ± 0.44, p < 0.001). These were mainly lung (n = 13, 48%) and skin infections (n = 6, 22%), more often infections of an undetermined infectious agent (n = 11, 41%) or of bacterial origin (n = 9, 33%). A few cases of opportunistic infections were reported. The median duration of follow-up without SIC event in the DM-MDA5+ cohort was 3.5 months. Conclusion: Patients with DM-MDA5+ have an increased infection risk compared to others inflammatory myopathies irrespective of immunosuppressive therapy exposure. These results highlight the importance of monitoring for infection during patient follow-up.
Sarcoidosis is one of the leading causes of uveitis. To date, no studies have assessed the factors specifically related with recovery in ocular sarcoidosis. In this study, we aimed to determine ...factors associated with ocular and extraocular recovery in patients with sarcoid uveitis.
A retrospective study of sarcoid uveitis, with a three-year minimum follow-up in Lyon University Hospital between December 2003 and December 2019. Patients presented biopsy-proven sarcoidosis or presumed sarcoid. Recovery was defined by a disease-free status, spontaneously or despite being off all treatments for three years or more.
143 patients were included: 110 with biopsy-proven and 33 with presumed sarcoid uveitis. Seventy-one percent were women, the median age at presentation was 53 years, and 71% were Caucasian. Chronic uveitis was the main clinical presentation (75%), mostly panuveitis (48%) with bilateral involvement (82%). After a median follow-up of 83.5 months, recovery was reported in 26% of patients. In multivariable analysis, Caucasian ethnicity (
= 0.007) and anterior uveitis (
= 0.008) were significantly associated with recovery, while increased intraocular pressure was negatively associated (
= 0.039).
In this large European cohort, one quarter of patients recovered. Caucasian ethnicity and anterior uveitis are associated with ocular and extraocular recovery.
Recently, concerns have been raised about an increased risk of cardiac sarcoidosis in patients with sarcoid uveitis. While cardiac sarcoidosis has a high mortality burden, there is still a lack of ...precise data on this association. The objective of this study is to describe the frequency and type of cardiac complications associated with sarcoidosis of a large cohort of patients with sarcoid uveitis. We analyzed the cardiac outcomes of a monocentric retrospective cohort of consecutive adults with a diagnosis of sarcoid uveitis between January 2004 and March 2020 in a tertiary French university hospital. A total of 294 patients with a final diagnosis of sarcoid uveitis were included. At final follow-up, seven (2.4%) patients of the cohort had cardiac sarcoidosis. Cardiac sarcoidosis was more frequent among patients with previously reported systemic sarcoidosis (p = 0.008). The prevalence of cardiac sarcoidosis among patients with sarcoid uveitis is low, but patients with previously diagnosed sarcoidosis or those who develop systemic sarcoidosis during follow-up appear to be at increased risk.
Still's disease (SD) is a heterogeneous autoinflammatory disorder for which several phenotypes have been described. We conducted a retrospective study to re-evaluate the dichotomous view of the ...disease, to compare the juvenile and adult forms, and to look for prognostic factors. We collected data from ten French centers, seeking patients with a diagnosis of adult-onset SD (AOSD) or systemic juvenile idiopathic arthritis (sJIA). We identified 238 patients, 152 (64%) of whom had AOSD while 86 (36%) had sJIA. The median age at SD onset was 26.6 years. In patients with identifiable patterns, the course of SD was systemic in 159 patients (74%), chronic in 55 (26%). Sore throat and myalgia were more frequent in patients with AOSD. Abnormal liver tests, serum ferritin and C-reactive protein levels were higher in AOSD group. Fever and skin rash were predictive of complete remission or recovery and high lactate dehydrogenase level was a poor prognosis factor. Symptoms such as splenomegaly, skin rash, high polymorphonuclear neutrophils count and macrophage activation syndrome were predictive of a systemic phenotype. Overall, there were no major differences between sJIA and AOSD. Our results are consistent with the "biphasic" model of an autoinflammatory disease that can progress to chronic arthritis if not treated early.
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