Predicting kidney outcomes among Latin American patients with lupus nephritis: The prognostic value of interstitial fibrosis and tubular atrophy and tubulointerstitial inflammation.

OBJECTIVE: To assess the effect of tubulointerstitial inflammation (TII) and interstitial fibrosis and tubular atrophy (IFTA) on kidney survival in lupus nephritis (LN). METHODS: Two hundred eighty five patients with biopsy-proven LN were retrospectively studied. Kidney survival was defined as the time from initial biopsy to end-stage kidney disease (ESKD), dialysis, or transplant. Kidney survival analysis was performed by the Kaplan-Meier method and the statistical difference between survival curves compared by the log-rank test. Cumulative incidence functions with competing risk of death for kidney survival were also graphed. Multivariable Cox proportional hazards regression and competing-risk analyses were performed to identify independent predictors of ESKD. RESULTS: Fifty-seven patients (20%) progressed to ESKD during a median time of 4.2 (2.0-55.2) months after biopsy. TII was present in 206 (72.3%) biopsies, while IFTA in 99 (34.7%) biopsies. Patients with moderate-to-severe IFTA had worse kidney survival than those with none or mild IFTA in both the Kaplan-Meier (p = 0.018) and the competing-risk analyses (p = 0.017). Patients with class IV ± V LN had worse kidney survival than those with non-class IV LN by the Kaplan-Meier method (p = 0.050), but not in the competing-risk analysis (p = 0.154). Worse kidney survival was also found among those with fibrous crescents than those without, in both the Kaplan-Meier (p = 0.010) and the competing-risk (p = 0.011) analyses. By multivariable Cox regression analysis, older age (HR 1.04, 95% CI 1.01-1.07) and class IV ± V LN (HR 5.06, 95% CI 1.82-14.09) were associated with higher risk of ESKD after adjusting for sex, ethnicity, TII, and IFTA. By competing-risk analyses, class IV ± V LN (SHR 3.32, 95% CI 1.25-8.83) and no response to immunosuppressive therapy (SHR 4.55, 95% CI 1.54-13.41) were associated with a higher risk of ESKD, while eGFR >90 mL/min/1.73 m2 (SHR 0.98 for each ml/min/1.73 m2, 95% 0.97-0.99) with a lower risk. CONCLUSIONS: Patients with moderate-to-severe IFTA had worse kidney survival than those with none or mild IFTA. Worse kidney survival was also found among those with class IV LN and fibrous crescents versus those without IV LN and fibrous crescents, respectively.

Predictors of severe hemolytic anemia and its impact on major outcomes in systemic lupus erythematosus: Data from a multiethnic Latin American cohort.

OBJECTIVE: To determine the predictors of the occurrence of severe autoimmune hemolytic anemia (AIHA) and its impact on damage accrual and mortality in SLE patients. METHODS: Factors associated with time to severe AIHA (hemoglobin level ≤7 g/dL) occurring from the onset of SLE symptoms were examined by Cox proportional hazards regressions. The association of severe AIHA with mortality was examined by logistic regression analyses while its impact on damage was by negative binomial regression. RESULTS: Of 1,349 patients, 49 (3.6%) developed severe AIHA over a mean (SD) follow-up time of 5.4 (3.8) years. The median time from the first clinical manifestation to severe AIHA was 111 days (IQR 43-450). By multivariable analysis, male sex (HR 2.26, 95% CI 1.02-4.75, p = 0.044), and higher disease activity at diagnosis (HR 1.04, 95% CI 1.01-1.08, p = 0.025) were associated with a shorter time to severe AIHA occurrence. Of the SLEDAI descriptors, only hematologic (leukopenia and/or thrombocytopenia) showed a certain trend toward significance in the multivariable analysis (HR 2.36, 95% CI 0.91-6.13, p = 0.0772). Severe AIHA contributed neither to damage nor to mortality. CONCLUSIONS: Severe AIHA occurs during the early course of SLE. Male sex and higher disease activity at diagnosis emerged as independent predictors of a shorter time to severe AIHA occurrence. Although not statistically significant, hematological abnormalities at SLE diagnosis could predict the occurrence of severe AIHA in a shorter time. Damage and mortality did not seem to be impacted by the occurrence of severe AIHA.

Systemic lupus erythematosus: The search for the ideal biomarker.

During the last decades, there has been an increased interest in the discovery and validation of biomarkers that reliably reflect specific aspects of lupus. Although many biomarkers have been developed, few of them have been validated and used in clinical practice, but with unsatisfactory performances. Thus, there is still a need to rigorously validate many of these novel promising biomarkers in large-scale longitudinal studies and also identify better biomarkers not only for lupus diagnosis but also for monitoring and predicting upcoming flares and response to treatment. Besides serological biomarkers, urinary and cerebrospinal fluid biomarkers have emerged for assessing both renal and central nervous system involvement in systemic lupus erythematosus, respectively. Also, novel omics techniques help us to understand the molecular basis of the disease and also allow the identification of novel biomarkers which may be potentially useful for guiding new therapeutic targets.

Kidney transplantation outcomes in lupus nephritis: A 37-year single-center experience from Latin America.

OBJECTIVE: We assessed patient and graft outcomes and prognostic factors in kidney transplantation in patients with end-stage kidney disease (ESKD) secondary to lupus nephritis (LN) undergoing kidney transplantation from August 1977 to December 2014 in a Latin American single center. METHODS: The primary endpoint was patient survival, and the secondary endpoints were death-censored graft survival for the first renal transplant and the rate of recurrent LN (RLN). Kaplan-Meier method was used for survival analysis. Factors predicting patient and death-censored graft survivals were examined by Cox proportional-hazards regression analyses. RESULTS: 185 patients were retrospectively evaluated. Patient survival rates were 88% at one year, 82% at three years, 78% at five years, and 67% at ten years. Death-censored graft survival for the first renal transplant was 93% at one year, 89% at three years, 87% at five years, and 80% at ten years. RLN was diagnosed in 2 patients (1.08%), but no graft was lost because of RLN. Thirty-nine (21.1%) patients died, and 65 (35.1%) patients experienced graft loss during the follow-up. By multivariable analyses, older recipient age and 1-month posttransplantation eGFR <45 ml/min/1.73m2 were associated with lower patient survival and an increased risk of graft loss, while induction immunosuppressive therapy exerted a protective effect on patients' survival. In the subgroup of patients in whom disease activity was measured at the time of transplantation, a higher SLEDAI score was also associated with lower patient survival and an increased risk of graft loss. CONCLUSION: In a mostly Mestizo population, kidney transplantation is an excellent therapeutic alternative in LN patients with ESKD. Older recipient age, an eGFR <45 ml/min/1.73m2 at one month posttransplantation, and disease activity at the time of transplantation are predictive of a lower patient and death-censored graft survival, while induction immunosuppressive therapy has a protective effect on patient survival. RLN is rare and does not influence the risk of graft loss.

Trans-scleral diode laser cyclophotocoagulation for refractory glaucoma after high-risk penetrating keratoplasty.

To analyze the intraocular pressure reduction, number of anti-glaucoma medications needed, and post-operative complications of trans-scleral diode laser cyclophotocoagulation (DCPC) in patients with high-risk penetrating keratoplasty (PKP) and secondary refractory glaucoma. Prospective interventional, longitudinal, non-comparative series of cases, including 16 eyes of 15 patient's post-PKP on maximal anti-glaucoma medical therapy with intraocular pressures above 22 mmHg. All patients received 18 shots, 360° peri-limbal (avoiding the long posterior ciliary nerves and arteries at 3 and 9 o'clock positions) of trans-scleral DCPC (2000 mW, time: 2.0 s/shot). There was a 55.5 % reduction (total of 14.0 mmHg) of the mean pre-operative IOP (31.5 mmHg) after the first diode laser application (p = 0.0020). Re-treatment was required in 31.2 % of eyes over a mean period of 10.7 months. In these five eyes, the mean pre-operative IOP was 40.4 mmHg, which decreased to 15.0 mmHg post-therapy, and a mean IOP reduction of 25.4 mmHg (p = 0.0218). There was a 51.0 % reduction in the mean number of medications used after the first, and a 57.1 % reduction after a second laser application. The incidence of failure (IOP ≥ 22 mmHg or need of additional medical therapy) from initial intervention to loss of follow-up was 1.3 % per person-month. DCPC effectively reduces the intraocular pressure and the number of anti-glaucoma medications with few complications in patients after high-risk PKP and secondary glaucoma. Only, one-third of the eyes needed a second intervention to control the intraocular pressure. Post-DCPC complications were limited to phthisis bulbi and endothelial dysfunction, one eye each. Please check and confirm the author names and initials are correct. Also, kindly confirm the details in the metadata are correct.

Co-testing for detection of high-grade cervical intraepithelial neoplasia and cancer compared with cytology alone: a meta-analysis of randomized controlled trials.

BACKGROUND: Human papillomavirus (HPV) DNA testing combined with cytology has been recommended as a primary cervical cancer screening strategy. METHODS: PubMed/MEDLINE, Embase, the Cochrane Library and the NIH trial registry were searched for randomized controlled trials comparing co-testing with cytology alone for the detection of high-grade CIN lesions and cancers. Of 1156 articles identified, four met inclusion criteria. The performance of co-testing and cytology alone was compared at baseline screening, second round screening and overall. Cumulative meta-analysis, Begg's test, Egger's test and sensitivity analysis were performed. RESULTS: At baseline, co-testing was associated with a significantly higher detection rate of CIN 2+ [risk ratio (RR) = 1.41, 95% confidence interval (CI): 1.12, 1.76] and a non-significantly higher CIN 3+ detection rate (RR = 1.15, 95% CI: 0.99, 1.33). At second round screening, co-testing was associated with significantly lower detection rates of both CIN 2+ and CIN 3+ (RR = 0.77, 95% CI: 0.63, 0.93; RR = 0·68, 95% CI: 0.55, 0.85). The overall detection rate did not differ between co-testing and cytology alone for CIN 2+ (RR: 1·19, 95% CI: 0.99, 1.46) or CIN3+ (RR: 0.99, 95% CI: 0.87, 1.14). CONCLUSION: Co-testing increases the detection of CIN2+ lesions at baseline and significantly decreases the detection rates of CIN2+ or CIN3+ lesions at subsequent screening compared with cytology alone.

Clinical characteristics of a large cohort of patients with scleritis and episcleritis.

OBJECTIVE: To evaluate the demographic characteristics, clinical features, ocular complications, and disease associations of patients with scleritis and episcleritis; as well as to delineate the risk factors for decreased vision in patients with scleritis. DESIGN: Retrospective case series. PARTICIPANTS: Five hundred patients with scleritis and 85 patients with episcleritis. METHODS: The electronic health records of 500 patients with scleritis and 85 patients with episcleritis seen at 2 tertiary referral centers were reviewed and their clinical features were studied. MAIN OUTCOME MEASURES: Clinical features (pain, scleral inflammation), ocular complications (decrease in vision, anterior uveitis, peripheral ulcerative keratitis, ocular hypertension), and disease associations. RESULTS: In a series of 585 patients, 500 patients had scleritis (85.5%) and 85 patients had episcleritis (14.2%). Ocular complications were more frequent overall in patients with scleritis versus in those with episcleritis (45.0% vs. 19.0%), including decrease in vision (15.8% vs. 2.3%), anterior uveitis (26.4% vs. 16.5%), peripheral ulcerative keratitis (7.4% vs. 0%), and ocular hypertension (14.2% vs. 3.5%; P<0.0001 for each). Disease association was observed in 35.8% of patients with scleritis versus 27.1% of episcleritis patients, including connective tissue or vasculitic diseases in 24.8% versus 15.3%, respectively. Scleritis preceded systemic disease diagnosis in 38.7% of patients. Ocular complications (90.0%) and disease association (80.0%) occurred most often in patients with necrotizing scleritis (P<0.0001 for each). Risk factors for decrease in vision in patients with scleritis included necrotizing scleritis (odds ratio [OR], 6.63; P<0.001), posterior scleritis (OR, 2.33; P = 0.042), degree of scleral inflammation of more than 2+ (range, 0-4+; OR, 3.60; P<0.001), anterior uveitis (OR, 1.78; P = 0.033), ocular hypertension (OR, 3.19; P<0.001), and associated disease (OR, 2.66; P<0.001), mainly infectious (OR, 4.44; P<0.001). CONCLUSIONS: Scleritis is associated more often with ocular complications than episcleritis, and necrotizing scleritis is the type of scleritis most often associated with ocular complications and disease association. Risk factors for decrease in vision in patients with scleritis include necrotizing scleritis, posterior scleritis, scleral inflammation of more than 2+, anterior uveitis, ocular hypertension, and associated infectious disease. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Scleritis therapy.

OBJECTIVE: To delineate factors associated with a successful response to treatment in patients with various manifestations of scleritis. DESIGN: Retrospective case series. PARTICIPANTS: A total of 392 patients with noninfectious anterior scleritis. METHODS: We reviewed the electronic health records of 392 patients with noninfectious anterior scleritis seen at 2 tertiary referral centers and studied the factors associated with successful treatment. MAIN OUTCOME MEASURES: Patient characteristics (age, sex); ocular disease characteristics (laterality, type of scleritis, degree of scleral inflammation, ocular complications, delay in presentation, and follow-up period), systemic disease association (associated disease, potentially lethal associated disease); and anti-inflammatory and immunosuppressive medications were studied in patients with scleritis. Successful treatment response to nonsteroidal anti-inflammatory drugs (NSAIDs), steroidal anti-inflammatory drugs (SAIDs), immunosuppressive therapy drugs (immunomodulatory therapy [IMT]), or biologic response modifiers (BRMs) was assessed. RESULTS: Treatment of 392 patients with noninfectious anterior scleritis included NSAIDs in 144 (36.7%), SAIDs in 29 (7.4%), IMT in 149 (38.0%), BRMs in 56 (14.3%), and none (N = 14). Successful response to treatment with NSAIDs was associated with idiopathic diffuse or nodular scleritis with a low degree of scleral inflammation (≤ 2+) (odds ratio [OR] = 2.89, P < 0.001) and with idiopathic diffuse or nodular scleritis without ocular complications (OR = 3.13, P < 0.001). Successful treatment with SAIDs was associated with idiopathic diffuse or nodular scleritis with a high degree of scleral inflammation (>2+) (OR = 4.70, P = 0.001). Successful treatment with IMT was associated with diffuse or nodular scleritis with associated systemic disease (OR = 1.57, P = 0.047), mainly potentially lethal (OR = 17.41, P=0.007), and necrotizing scleritis (OR = 4.73, P = 0.026). Successful treatment with BRMs was associated with diffuse or nodular scleritis with associated systemic disease (OR = 3.15, P < 0.001). This study did not require institutional review board approval because the information does not contain any subject identifiers. CONCLUSIONS: Patients with idiopathic diffuse or nodular scleritis with a low degree of scleral inflammation or without ocular complications may respond to NSAIDs. Patients with idiopathic diffuse or nodular scleritis with a high degree of scleral inflammation may respond to SAIDs. Patients with diffuse or nodular scleritis with associated systemic disease may respond to IMT or BRMs. Patients with necrotizing scleritis may respond to IMT, mainly alkylating agents. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Clinical features and presentation of infectious scleritis from herpes viruses: a report of 35 cases.

PURPOSE: To describe clinical features and presentation of infectious scleritis resulting from herpes viruses. DESIGN: Retrospective case series. PARTICIPANTS: Thirty-five patients out of 500 with scleritis. METHODS: We reviewed the electronic health records of 500 patients with scleritis, 35 of whom were diagnosed with herpes virus infection, seen at 2 tertiary referral centers. We studied the clinical features and ocular complications of this subset of patient with scleritis. MAIN OUTCOME MEASURES: Correlation between classification, severity, and symptoms (i.e., pain) and diagnosis of herpetic-associated scleritis. Vision loss, presence of associated uveitis, keratitis, glaucoma, or systemic disease were documented over the follow-up period. Other outcome measures included epidemiologic data: age, gender, laterality, visual acuity, duration of symptoms, and underlying systemic or ocular diseases. RESULTS: Of 500 patients with scleritis, 47 (9.4%) had an underlying infectious cause. Thirty-five (74.4%) of these were diagnosed with herpes virus infection, 5 (10.6%) with tuberculosis, and the remaining 7 (14.8%) with other infectious disease. Patients with herpes-associated scleritis were analyzed as a group and then compared with those with idiopathic scleritis. Most patients with herpetic scleritis presented with acute (85.7%) and unilateral (80%) scleral inflammation. Pain was moderate or severe in 68.6% of the patients. The most common type of scleritis was diffuse anterior in 80% (n = 28), followed by nodular anterior 11.4% (n = 4), and necrotizing in 8.6% (n = 3). Necrotizing anterior scleritis was more commonly seen in patients with herpetic scleritis versus patients with idiopathic disease (8.6% vs 1.2%; P<0.05). Unilaterality was also more common in herpetic scleritis (80%) than in idiopathic disease (56.7%; P<0.05). Vision loss was significantly greater in herpetic than idiopathic scleritis (34.3% vs 11.5%; P<0.001). CONCLUSIONS: The association between scleritis and infectious disease may be higher than previously reported by other series. Herpes viruses account for 7% of all scleritis cases and its diagnosis may be challenging when there is not a classically diagnostic clinical picture. We present the observed clinical features of herpetic scleritis and describe the clinical differences at presentation between patients with idiopathic scleritis and those with herpes infection.

Immunohistochemical differences between normal and chronically inflamed conjunctiva: diagnostic features.

PURPOSE: There is widespread misinterpretation of normal conjunctival fibrinogen. In differentiating between normal conjunctiva and cicatrizing conjunctivitis, including ocular cicatricial pemphigoid, atopic keratoconjunctivitis, and lichen planus, it is important to properly evaluate and characterize the histologic appearance of the structures seen and not base a diagnosis on just the presence or absence of certain features. One feature of conjunctival histology prone to misinterpretation and misdiagnosis is the presence of subepithelial fibrinogen, particularly when the diagnosis of lichen planus is being considered. Although the presence of subepithelial fibrinogen in oral mucous membranes and in skin can be indicative of lichen planus, such is not the case for conjunctiva. An erroneous diagnosis of lichen planus based on the presence of conjunctival subepithelial fibrinogen can initiate prolonged treatment with topical steroids leading to avoidable, blinding, complication, and further, delay therapy for the real cause of the conjunctivitis. We conducted a cross sectional, controlled, blinded and prospective Institutional Review Board-approved study on the occurrence and pattern of fibrinogen at the epithelial basement membrane zone (BMZ) of normal and inflamed conjunctiva. METHODS: Bulbar conjunctiva was obtained from 10 cases of undiagnosed chronic conjunctivitis of at least 6 months duration and 8 patients with normal conjunctiva. Immunofluorescent staining with antifibrinogen antibodies, periodic acid-schiff stain (PAS), and Giemsa staining were performed. RESULTS: BMZ fibrinogen was found in all cases. This layer was linear, smooth, and continuous in normal conjunctiva and 7 cases of chronic conjunctivitis. It was fragmented and lumpy in 1 case of ocular cicatricial pemphigoid (OCP) and showed spikes and spurs in 2 cases of lichen planus. CONCLUSIONS: BMZ fibrinogen is a normal component of the conjunctiva and its morphological features rather than its mere presence should be assessed as a diagnostic tool.

Understanding Risk Factors for Poor Outcomes in a Multiethnic Longitudinal Cohort: The LUMINA (Lupus in Minorities: Nature vs. Nurture) Experience (LUMINA LXXXII).

The Lupus in Minorities: Nature Vs Nurture (LUMINA) cohort has placed Hispanics on the lupus map in the United States. Texan Hispanic and African American patients experience, overall, worse outcomes than the Caucasian and Puerto Rican Hispanic patients. The genetic component of ethnicity is important early in the disease course whereas socioeconomic factors become more important subsequently. The role of hydroxychloroquine in preventing damage accrual and reducing mortality in lupus patients is a major contribution of LUMINA.

Novel clinical presentation of a CRX rod-cone dystrophy.

We describe a novel clinical presentation of a CRX rod-cone dystrophy in a single family. Two boys ages 6 and 12 years presented with clinical and optical coherence tomography features suggestive of X-linked retinoschisis, but with optic nerve swelling without increased intracranial pressure. One patient had an electronegative electroretinogram (ERG) and the other had rod-cone dysfunction. Neither had retinoschisin (RS1) gene mutations. Biological mother and sister presented with retinal pigment epithelium (RPE) changes and abnormal cone-rod ERG responses. On further testing, next generation sequencing with array comparative genomic hybridisation showed a deletion in exon 4 of the CRX gene. Cystoid maculopathy in young male children can be difficult to distinguish from RS1-associated schisis. Phenotypic variants within a family must prompt a thorough retinal dystrophy evaluation even with electronegative ERG in the presenting child. This novel phenotype for CRX presents with optic nerve swelling and cystoid maculopathy in men, and RPE changes in women.

Factors affecting quality of life in patients with systemic lupus erythematosus: important considerations and potential interventions.

INTRODUCTION: Patients with systemic lupus erythematosus (SLE) have a better survival than decades ago; nevertheless, they still experience a low health-related (HR) quality of life (QoL). Areas covered: After defining QoL and HRQoL, we review the need to assess it, its elements, how to measure it, its predictors, and its impact and potential interventions to improve it. Expert commentary: Physicians assessments of disease activity and damage do not capture the patients' perspective of their health, and these differences could lead to nonadherence to therapy. Based on that, a comprehensive evaluation of SLE should include the assessment of HRQoL or the sum of the physical, psychological, and social perception of wellbeing, influenced by the patient's illness. The most consistent predictors of low HRQoL are older age, poverty, lower educational level, behavioral issues, some clinical manifestations, and comorbidities. HRQoL impacts negatively on dealing with stress, intimal relationship, home and job-related activities, and treatment adherence. At the present, there are no successful specific therapeutic strategies aimed at improving it.

Pseudotumor cerebri syndrome in a pregnant woman with systemic lupus erythematous.

Systemic lupus erythematous is a chronic multi-systemic autoimmune disease that affects multiple organ systems, including the central nervous system. Pseudotumor cerebri is a disorder associated with increased intracranial pressure in the absence of a space-occupying lesion or other identifiable cause that affects young and obese women.We present the case of a pregnant woman with both pseudotumor cerebri and a new diagnosis of active systemic lupus erythematous.

The evolving concept of SLE comorbidities.

INTRODUCTION: The survival of SLE patients has improved significantly over the past few decades placing them at increased risk of cardiovascular disease (CVD), malignancies, and osteoporosis, among other comorbidities. The aim of this review was to assess the incidence and prevalence of comorbidities in these patients as well as their prevention and treatment focusing in CVD, malignancies and osteoporosis. Areas covered: We focused on CVD, malignancies and osteoporosis as SLE comorbidities. A literature search (PubMed database) was performed using the words 'comorbidities', 'cardiovascular disease', 'osteoporosis', 'malignancy', 'cancer' and 'lupus' between January 1976 and December 2016. No language restrictions were placed. More than 100 full-length articles were reviewed. Expert commentary: The therapeutic approach in SLE should aim not only at achieving disease remission but also at treating all conditions affecting the patients and, consequently, their outcomes. These patients should be treated as coronary artery disease (CAD) equivalent with rigorous modifiable CV risk factors management in addition to the optimal treatment of their lupus. Furthermore, modifiable osteoporosis traditional risk factors and SLE-related risk factors should be modified to ameliorate bone loss and fracture risk. Cancer preventive measures (smoking cessation and screening programs for cervical cancer) constitute also essential components of the management of these patients.

Organ damage in systemic lupus erythematosus / Daño orgánico en lupus eritematoso sistémico

ABSTRACT Damage reflects the irreversible changes that occur in systemic lupus erythematosus (SLE) patients as a consequence of the disease, its treatment or comorbidities. The pattern of damage increases in a steady linear fashion over time. At least half of all patients with SLE will have some form of organ damage 10 years after their diagnosis. Factors associated with the occurrence of damage include older age, disease duration, male gender, non-Caucasian ethnicity, disease activity, corticosteroid use, poverty, hypertension and abnormal illness behaviors. In contrast, antimalarials are protective against damage. Since damage predicts further damage and mortality, prevention of damage accrual should be a major therapeutic goal in SLE. Novel therapies for SLE that achieve better control of the disease and with corticosteroid-sparing properties, may lead to improved outcomes in patients as they will reduce damage accrual and improve survival.

RESUMEN El daño refleja los cambios irreversibles que se producen en los pacientes con lupus eritematoso sistémico (LES) como consecuencia de la enfermedad, de su tratamiento o por causa de comorbilidades. El patrón de dano aumenta de forma lineal, constante a lo largo del tiempo. Al menos la mitad de todos los pacientes con LES presentará alguna forma de daño orgánico 10 años después de haber sido diagnosticados. Entre los factores asociados con el desarrollo de dano encontramos la edad avanzada, la duración de la enfermedad, el sexo masculino, la etnia no caucásica, la actividad de la enfermedad, el uso de corticoesteroides, la pobreza, la hipertensión y comportamientos anormales de la enfermedad; por otra parte, los antimaláricos protegen contra el dano de la enfermedad. Puesto que la presencia de daño es un predictor de danno adicional y de mortalidad, la prevención de acumulación de dano deberá ser un objetivo terapéutico fundamental en LES. Los tratamientos novedosos para el LES que logren un mejor control de la enfermedad y que tengan propiedades ahorradoras de corticoesteroides, podrían lograr mejores desenlaces en los pacientes, pues reducirían el daño acumulado y mejorarían la sobrevida.

Scleritis in patients with granulomatosis with polyangiitis (Wegener).

AIMS: To describe and compare clinical features, complications and outcomes in patients with granulomatosis with polyangiitis (GPA)-associated scleritis with those seen in idiopathic and other autoimmune-associated scleritis, and to further describe the features that may serve as an indicator of life-threatening systemic disease. METHODS: We retrospectively reviewed electronic health records of all patients with scleritis seen at two tertiary care centres. Of 500 patients, 14 had GPA-associated scleritis and were included in this analysis. Measures included were age, gender, laterality, visual acuity and underlying systemic or ocular diseases. Clinical features (location, pain, inflammation) and ocular complications of these patients (decrease of vision, concomitant anterior uveitis and ocular hypertension) were studied and correlated. RESULTS: Fourteen of 500 patients with scleritis were GPA associated. Most of the patients with GPA-associated scleritis presented with sudden onset, bilateral, diffuse anterior scleral inflammation, with moderate-or-severe pain. Vision loss was not significantly different, and pain was more severe in these patients than in those with idiopathic scleritis. When compared with patients with other underlying autoimmune diseases, there were no significant differences found in epidemiological or clinical signs. Necrotising scleritis and corneal involvement were more commonly observed in GPA than in idiopathic scleritis and other autoimmune diseases and are often the presenting feature of the disease. CONCLUSIONS: The presence of necrotising changes or corneal involvement in the setting of scleral inflammation is highly suggestive of an underlying systemic vasculitis, of which GPA is the most common. These features should alert the doctor/optometrist and prompt a thorough diagnostic approach and an aggressive treatment given that it could reveal a life-threatening disease.

Scleritis associated with relapsing polychondritis.

AIMS: To evaluate ocular disease characteristics and successful therapeutic regimens in patients with scleritis associated with relapsing polychondritis (RP). To compare these features with those seen in patients with scleritis associated with other systemic immune-mediated diseases (SIMD). METHODS: Electronic health records of 13 scleritis patients associated with RP were analysed and compared with those of 113 scleritis patients associated with other SIMD seen at two tertiary referral centres. RESULTS: Scleritis in patients with RP was often bilateral (92.3%), diffuse (76.9%), recurrent (84.6%), sometimes with decreased vision (46.2%), anterior uveitis (38.5%), peripheral keratitis (15.4%) and ocular hypertension (30.8%). Patients with scleritis associated with RP more often had bilateral scleritis (p=0.001), necrotising scleritis (23.1%; p=0.02), recurrences (p=0.001) and decreased vision (three of the six with legal blindness; p=0.012), as compared with patients who had scleritis associated with other SIMD. Nine patients (69.2%) had one or more SIMD other than RP, including systemic vasculitis (4) or other autoimmune disease (8); they antedated RP by 9 years (range 2-21 years). Successful therapy included cyclophosphamide (5), methotrexate (3), azathioprine (3), mycophenolate mofetil (2), infliximab (2) and adalimumab (1). CONCLUSIONS: Scleritis may be the first manifestation whose study leads to the diagnosis of RP. Scleritis associated with RP is more often bilateral, necrotising, recurrent and associated with decrease of vision than scleritis associated with other SIMD. About 69.2% of patients will have an additional SIMD disorder. Scleritis associated with RP most often will require immunomodulatory therapy. Occasionally, scleritis with RP may appear while using antitumor necrosis factor α agents.