Incidence and medical costs of lupus in Spanish hospitals: a retrospective database analysis.

BACKGROUND: This study aimed to assess the comorbidity profile, use of healthcare resources and medical costs of patients with systemic lupus erythematosus (SLE) and cutaneous lupus erythematosus (CLE) treated at the hospital level in Spain. METHODS: Admission records of patients with SLE and CLE that were registered between January 2016 and December 2020 were obtained from a Spanish hospital discharge database and analyzed in a retrospective multicenter study. RESULTS: 329 patients met the criteria; 64.44% were female and 35.56% were male, with a median age of 54.65 years. Mean Charlson comorbidity index (CCI) was 2.75 in the index admission. 31.61% of the patients suffered essential hypertension, 21.96% suffered asthma and 19.76% suffered hyperlipidemia. Mortality rate was 3.95%. The most common medical procedure was heart ultrasound (19.45%) and introduction in peripheral vein of anti-inflammatory with a percutaneous approach (17.93%). Mean admission cost was €6355.99. CONCLUSIONS: Lupus patients showed a higher incidence and prevalence in the female population, with associated cardiac diseases as the main secondary conditions.

Cutaneous Lupus Erythematosus: Review and Considerations for Older Populations.

Though more common earlier in life, increasing attention is being focused on the development of cutaneous lupus erythematosus (CLE) in patients with advancing age. Studies show that CLE is more common in older populations than previously thought, and all CLE subtypes are possible in this group. Just like patients in the third or fourth decade of life, CLE may appear alongside or independent of systemic lupus erythematosus. Older populations manifesting CLE for the first time seem to have a lower risk of progression to systemic disease than younger peers, and are more commonly White. CLE must be carefully distinguished from other skin conditions that have a predilection for presentation in older populations, including rosacea, lichen planus, and other autoimmune conditions such as dermatomyositis or pemphigus/pemphigoid. It is thought that most CLE in older populations is drug-induced, with drug-induced subacute cutaneous lupus erythematosus being the most common subtype. Management of CLE in older patients focuses on eliminating unnecessary medications known to induce CLE, and otherwise treatment proceeds similarly to that in younger patients, with a few special considerations.

Cutaneous lupus erythematosus is associated with an increased risk of cardiac and vascular diseases: a large-scale, propensity-matched global retrospective cohort study.

BACKGROUND: Autoimmune skin diseases can expedite various systemic sequelae involving other organs. Although limited to the skin, cutaneous lupus erythematosus (CLE) was noted to be associated with thromboembolic diseases. However, small cohort sizes, partially discrepant outcomes, missing data on CLE subtypes, and incomplete risk assessment limits these findings. METHODS: The Global Collaborative Network of TriNetX provides access to medical records of more than 120 million patients worldwide. We used TriNetX to elucidate the risk for cardiac and vascular diseases after diagnosis of CLE, and its subtypes chronic discoid (DLE) and subacute cutaneous lupus erythematosus (SCLE). We included 30,315 CLE, 27,427 DLE, and 1613 SCLE patients. We performed propensity-matched cohort studies determining the risk to develop cardiac and vascular diseases (ICD10CM:I00-99) following diagnosis of CLE, DLE, or SCLE. Patients with systemic lupus erythematosus were excluded. FINDINGS: We document that CLE and its subtype DLE but less so SCLE are associated with a higher risk for various cardiac and vascular diseases. This included predominantly thromboembolic events such as pulmonary embolism, cerebral infarction, and acute myocardial infarction, but also peripheral vascular disease and pericarditis. For example, the hazard ratio of arterial embolism and thrombosis was 1.399 (confidence interval: 1.230-1.591, p < 0.0001) following CLE diagnosis. The study is limited by retrospective data collection and reliance on ICD10-disease classification. INTERPRETATION: CLE and its major subtype DLE are associated with an increased risk for the development of a wide range of cardiac and vascular diseases. FUNDING: This research was funded by Deutsche Forschungsgemeinschaft (EXC 2167, CSSL/CS01-2022) and the Excellence-Chair Program of the State of Schleswig-Holstein.

Epidemiology and demographics of cutaneous lupus erythematosus in Colombia between 2015 and 2019.

Cutaneous lupus erythematosus (CLE) is classified into three groups - acute, subacute, and chronic - based on clinical and histopathological characteristics. The risk of systemic manifestations varies among these groups. There are few studies on CLE epidemiology. For this reason, this paper aims to describe CLE prevalence and demographics in Colombia between 2015 and 2019. This descriptive, cross-sectional study used the international classification of diseases, tenth revision, for CLE subtypes, utilizing official data from the Colombian Ministry of Health. In people older than 19 years, 26,356 CLE cases were registered, yielding a prevalence of 76 cases per 100,000 population. CLE was more frequent in females, at a 5:1 ratio compared to males. The most common clinical presentation was discoid lupus erythematosus, in 45% of cases. The majority of cases occurred in people between 55 and 59 years old. This is the first study that describes CLE demographics in adults in Colombia. Findings regarding clinical subtypes and female predominance are consistent with those in the medical literature.

Hepatitis B and C virus infection in patients with Systemic and Cutaneous Lupus Erythematosus.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by a multifactorial etiology. The primary aim of this study was to estimate HCV and HBV infection prevalence in a cohort of SLE and Cutaneous Lupus Erythematosus (CLE). We assessed the frequency of these infections in our cohort and the possible associations with disease clinical/laboratory features and disease activity status. The prevalence of chronic HBV infection was 2.2% in the CLE group, while no HBsAg positive patients were identified in the SLE group. Conversely, the prevalence of anti-HCV positive was 2.2% in the SLE group while no anti-HCV positive patients were identified in the CLE group. We found no significant association between anti-HBc positive status and clinical manifestations or disease activity status in either group of patients. Hemodialysis resulted significantly associated with anti-HBc positivity in SLE. In the present study, we found HBsAg positivity in CLE patients but not in the Systemic form (SLE); conversely, a similar prevalence of anti-HBc antibodies in both groups was observed. A possible protective role exerted by SLE in HBV infection may be hypothesized. A higher frequency of HCV infection in SLE compared to CLE suggests a possible involvement of HCV in some SLE-related clinical and immunological features.

Epidemiology of Cutaneous Lupus Erythematosus Among Adults Over Four Decades (1976-2018): A Lupus Midwest Network (LUMEN) Study.

OBJECTIVE: To characterize the epidemiological trends and mortality of cutaneous lupus erythematosus (CLE) between 1976 and 2018 in Olmsted County, Minnesota. PATIENTS AND METHODS: In this retrospective population-based cohort study, all incident and prevalent CLE cases among adult residents in Olmsted County, Minnesota, between January 1, 1976, and December 31, 2018, were identified and categorized by subtype through medical record review using the resources of the Rochester Epidemiology Project. RESULTS: The overall incidence rate of CLE between 1976 and 2018 was 3.9 (95% CI, 3.4 to 4.5) per 100,000. The incidence of CLE was relatively stable, with no major trend across sexes or age groups. The age- and sex-adjusted prevalence of CLE was 108.9 per 100,000 on January 1, 2015. Mortality in CLE patients was similar to that of the general population, with a standardized mortality ratio of 1.23 (95% CI, 0.88 to 1.66) with no observed trends in mortality over time. CONCLUSION: In the past 4 decades, the incidence of CLE remained stable. Patients with CLE have mortality comparable to that of the general population.

Late-onset cutaneous lupus erythematosus patients have distinctive clinical features and demographics versus early-onset patients.

BACKGROUND: Cutaneous lupus erythematosus (CLE) can present later in life, but frequency and risk factors of late-onset CLE patients are not well characterized. The study determined frequency of late-onset CLE and compared the demographic and disease characteristics between early-onset and late-onset CLE in a cohort of patients with CLE. OBJECTIVES: To determine the frequency and compare clinical features of early-onset and late-onset CLE. METHODS: This was a cross-sectional study of CLE patients seen in outpatient dermatology clinics at University of Texas Southwestern Medical Center (UTSW) and Parkland Health and Hospital System, Dallas, TX, from April 2009 to May 2019. The primary outcome was the age of CLE onset, stratified by early-onset (<50 years) and late-onset CLE (≥50 years). Predictor variables significantly associated with CLE onset groups were identified by univariate and multivariable logistic regression analyses. RESULTS: Of the 291 CLE patients studied, 79% were early-onset, and 21% were late-onset. Multivariable logistic regression analyses identified that Caucasian race (odds ratio (OR): 2.23, 95% Confidence Interval (CI): 1.19-4.19, p = 0.013), having a CLE subtype other than chronic (OR: 2.18, 95% CI: 1.02-4.65, p = 0.044), and drug-induced cases (OR: 4.65, 95% CI: 1.18-18.24, p = 0.028) were significantly associated with late-onset CLE. Early-onset CLE patients were more likely to have oral ulcers (OR: 3.58, 95% CI: 1.46-8.78, p = 0.005) and renal disorders (OR: 4.02, 95% CI: 1.10-14.71, p = 0.036). LIMITATIONS: This was a single center study. Age of onset was self-reported and late-onset CLE cohort has a small sample size. CONCLUSIONS: Our diverse cohort of CLE patients had about one out of five patients with CLE experiencing disease onset after 50 years old. These patients have distinct demographic and clinical presentations compared to early-onset CLE patients. Providers should remain mindful of CLE in older patients with photosensitive rashes and mild systemic symptoms.

Long-term risk of adverse cardiovascular outcomes associated with cutaneous lupus erythematosus: a nationwide cohort study.

BACKGROUND: Autoimmune diseases, including systemic lupus erythematosus, have been associated with a substantial risk of cardiovascular morbidity and mortality. However, data on the long-term risk of incident heart failure and other adverse cardiovascular outcomes among patients diagnosed with cutaneous lupus erythematosus (CLE) are limited. METHODS: In this cohort study, all patients ≥ 18 years with newly diagnosed CLE between 1996 and 2018 were identified through Danish nationwide registries and matched 1:4 by age, sex, and comorbidity with individuals without CLE. Incident adverse cardiovascular outcomes, including heart failure, were compared between the matched groups, overall, and according to sex. RESULTS: Of 2085 patients diagnosed with CLE, 2062 patients were matched with 8248 control subjects from the Danish background population (median age 50 years [25th-75th percentile: 37-62 years]; 22.3% men). The median follow-up was 6.2 years. The 10-year cumulative incidences and adjusted hazard ratios (HR) of outcomes were as follows: heart failure: 3.29% (95% CI, 2.42-4.36%) for CLE patients versus 2.59% (2.20-3.02%) for the background population, HR 1.67 (95% CI, 1.24-2.24); atrial fibrillation or flutter: 5.15% (3.99-6.52%) versus 3.84% (3.37-4.36%), HR 1.40 (1.09-1.80); the composite of ICD implantation, ventricular arrhythmia, or cardiac arrest: 0.72% (0.34-1.40%) versus 0.44% (0.29-0.64%), HR 1.71 (0.85-3.45); the composite of pacemaker implantation, atrioventricular block, or sinoatrial dysfunction: 0.91% (0.48-1.59%) versus 0.54% (0.37-0.76%), HR 1.32 (0.72-2.41); myocardial infarction: 3.05% (2.18-4.15%) versus 1.59% (1.29-1.93%), HR 2.15 (1.53-3.00); ischemic stroke: 3.25% (2.38-4.32%) versus 2.50% (2.13-2.93%), HR 1.56 (1.16-2.10); and venous thromboembolism: 2.74% (1.94-3.75%) versus 2.05% (1.71-2.44%), HR 1.60 (1.16-2.21). Sex did not modify the association between CLE and adverse cardiovascular outcomes (Pinteraction ≥ 0.12 for all outcomes). CONCLUSIONS: Patients with CLE had a higher associated risk of adverse cardiovascular outcomes compared with the background population, irrespective of sex. Key Points • Findings: In this nationwide cohort study, including 2062 patients with cutaneous lupus erythematosus and 8248 matched controls, cutaneous lupus erythematosus was associated with an increased long-term risk of heart failure, cardiac arrhythmias, and thromboembolic events, irrespective of sex.

Unusual severe radiation-induced toxicity in a patient with discoid lupus erythematosus: A case report and critical review of the literature.

Data on the incidence and severity of radiation-induced toxicity in patients with systemic and/or cutaneous lupus erythematosus (SLE/CLE) are very limited. After reporting the case of a patient who experienced major toxicity and CLE flare in the irradiated area following breast irradiation, we conducted a comprehensive literature review of available data in this setting. The few retrospectives studies which have evaluated both the risk of toxicity in SLE/CLE patients and/or the potential induction or reactivation of SLE/CLE with radiotherapy have not shown differences between SLE/CLE patients and controls. Several other factors such as concurrent chemotherapy, a particular genetic background, or lupus treatments (essentially hydroxychloroquine) can explain severe radiation-induced toxicity. Therefore, patients with SLE/CLE should be irradiated like patients without SLE/CLE, with close monitoring during radiotherapy if other risk factors exist. Further studies examining a larger number of patients would probably allow a better understanding of the radiosensitivity of these patients.

Long-Term Risk of Autoimmune Diseases other than Systemic Lupus Erythematosus in Cutaneous Lupus Erythematosus-Alone Patients: A 10-Year Nationwide Cohort Study.

BACKGROUND: Up to 25% of patients with cutaneous lupus erythematosus (CLE) can develop systemic lupus erythematosus (SLE). However, the risk of autoimmune diseases other than SLE in CLE patients who have only skin manifestations (CLE-alone) has rarely been explored. OBJECTIVE: To investigate the long-term risk and independent factors of non-SLE autoimmune diseases among CLE-alone patients. METHOD: A nationwide cohort study using the Taiwanese National Health Insurance Research Database 1997-2013. CLE patients and matched subjects were included. Cumulative incidences of autoimmune diseases after 1 year of CLE-alone diagnosis were compared. Cox proportional hazard model was also performed. RESULTS: A total of 971 CLE-alone patients and 5,175 reference subjects were identified. The 10-year cumulative incidence of autoimmune diseases other than SLE was significantly elevated in the CLE-alone cohort (9.00%, 95% confidence interval [CI] 6.72-11.29) than in the reference cohort (4.20%, 95% CI 3.53-4.87%) (p < 0.001). CLE-alone was independently associated with non-SLE autoimmune diseases (adjusted hazard ratio 1.55, 95% CI 1.10-2.18). Among CLE-alone patients, females and those taking long-term systemic corticosteroids (a proxy for extensive disease) were associated with non-SLE autoimmune diseases after adjusting for the number of repeated autoimmune laboratory tests. CONCLUSION: CLE-alone is independently associated with future non-SLE autoimmune diseases.

Clinical characteristics of itch in cutaneous lupus erythematosus: A prospective, multicenter, multinational, cross-sectional study.

OBJECTIVE: Pruritus is an important symptom frequently accompanying various inflammatory skin conditions and some recent data indicated that it may be associated with autoimmune connective tissue diseases. The aim of this study was to assess the frequency and clinical presentation of itch in CLE. METHODS: A multinational, prospective, cross-sectional study was performed to assess the prevalence, intensity and clinical characteristic of pruritus in various subtypes of CLE. A total of 153 patients with active CLE lesions were included. Their age ranged between 17 and 82 years (mean 49.8 ± 15.4 years), and 115 patients (75.2%) were women. The disease activity and damage were assessed according to the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI). Pruritus severity was assessed with Numeric Rating Scale (NRS) and the 12-Item Pruritus Severity Scale. Dermatology Life Quality Index and EQ-5D questionnaire were used to measure quality of life. RESULTS: Pruritus was present in 116 (76.8%) of patients of whom half had NRS scoring equal or above 4 points indicating moderate or severe pruritus. Most commonly itch was localized on the scalp, face (excluding ears and nose) and arms (40.5%, 36.2%, 31.9%, respectively). Sensations connected with pruritus were most frequently described as burning, tingling and like ants crawling feeling, but 31.9% patients described it as "pure itch". More than half of patients reported that pruritus was present every day, and it was most frequent during the evenings. The pruritus scoring and the CLASI activity score were significantly correlated (r = 0.42, p = 0.0001), while no correlation was found with the CLASI damage score (p = 0.16). Both the maximum and average itch intensity were correlated with systemic lupus erythematosus (SLE) activity measured with the Systemic Lupus Erythematosus Disease Activity Index. CONCLUSIONS: Pruritus is a common, but frequently overlooked symptom of CLE. Its intensity correlates with the activity of CLE, but not with the skin damage. In more than a half of patients it occurs on a daily basis. The correlation between the intensity of pruritus and the activity of the skin lesions and the systemic involvement indicate that pruritus could be an individual indicator of both SLE and CLE activity.

Increased cancer risk in patients with cutaneous lupus erythematosus and systemic lupus erythematosus compared with the general population: A Danish nationwide cohort study.

OBJECTIVES: To investigate if patients with cutaneous lupus erythematosus (CLE) or systemic lupus erythematosus (SLE) have an increased risk of cancer compared with the general population, and furthermore to identify specific cancer types associated with increased risk. METHODS: This is an observational cohort study of 5310 patients with CLE or SLE identified in the Danish National Patient Register from 1 January 1995 to 31 December 2014. The cohort was followed up for cancer by linkage to the Danish Cancer Registry. Based on the age, sex, and calendar specific cancer rates of the general population of Denmark, standardised incidence ratios (SIRs) were calculated. RESULTS: The patients with CLE or SLE were followed for 40.724 person-years, each group's average duration of follow-up being 6.9 and 8.1 years. The SIR for overall cancer (except non-melanoma skin cancer (NMSC)) was increased in patients with CLE 1.35 (95%CI 1.15 to 1.58) and patients with SLE 1.45 (95%CI 1.30 to 1.62). Both groups had high risks of hematological - including a 3-4-fold increased risk of non-Hodgkin lymphoma -, pancreatic, and lung cancers. Several cancers associated with oncogenic viruses as liver and tongue/mouth/pharynx were increased in the SLE group, while the risk of ovarian cancer was increased 2-4-fold only in the CLE group. CONCLUSION: The overall risk of cancer was significantly increased in both patients with CLE and SLE. SIRs for hematological, pancreatic and lung cancers were elevated in both groups. Extra awareness of cancer in patients with SLE and patients with CLE should be considered.

Prevalence of antimitochondrial antibodies in subacute cutaneous lupus erythematosus.

BACKGROUND: In approximately 13% of systemic lupus erythematosus (SLE) patients, a hallmark of primary biliary cirrhosis (PBC) can be detected: antimitochondrial M2 antibodies (AMA-M2). It has not been determined if the presence of AMA-M2 in SLE patients results in a higher risk of PBC in comparison to those with AMA but no SLE. Until now, there have been no such analyses among individuals with subacute cutaneous lupus erythematosus (SCLE). METHODS: To assess the seropositivity rates for AMA-M2 and autoantibodies associated with autoimmune hepatitis in patients with newly diagnosed SCLE and to determine the coexistence and risk of development of autoimmune liver disease in these patients within 1 year of follow-up, data from 33 patients with newly diagnosed SCLE were analyzed. RESULTS: AMA-M2 was found in 20% of SCLE patients. Patients from the AMA-M2-positive group were characterized by significantly higher levels of cholestatic liver enzymes when compared to those without AMA-M2 (P < 0.05). After introducing therapy with hydroxychloroquine and prednisone, the levels of hepatocellular enzymes increased significantly only in AMA-M2 positive patients (P < 0.05). CONCLUSIONS: A high prevalence of AMA-M2 was found in patients with SCLE. Patients with SCLE and AMA-M2 had significantly higher values of cholestatic enzymes than patients without AMA. Newly diagnosed patients with SCLE should be screened for the presence of AMA and should be clinically followed up. Avoiding drugs with potential liver toxicity should be recommended in patients with SCLE and AMA.

Use of hydroxychloroquine and risk of major adverse cardiovascular events in patients with lupus erythematosus: A Danish nationwide cohort study.

BACKGROUND: Limited data suggest that hydroxychloroquine may affect risk of cardiovascular disease in patients with lupus erythematosus (LE). OBJECTIVE: To investigate whether hydroxychloroquine treatment is associated with major adverse cardiovascular events (MACE) (myocardial infarction, ischemic stroke, or cardiovascular-associated death) in patients with cutaneous LE (CLE) or systemic LE (SLE). METHODS: Based on the Danish nationwide registers, an observational cohort study was conducted including patients with first-time diagnosis of CLE or SLE (between 1997 and 2017). Cox regression models calculating the hazard ratio (HR) analyzing the risk of MACE were performed comparing time on and off hydroxychloroquine (including never users). The models were adjusted for age, sex, socioeconomic status, concomitant treatment, and cardiovascular risk factors. RESULTS: Among 4587 patients with LE, 51% (n = 2343) were treated with hydroxychloroquine during the study period. An inverse association between use of hydroxychloroquine and MACE risk was observed among patients with SLE (adjusted HR, 0.65; 95% confidence interval, 0.46-0.90) and patients with CLE (adjusted HR, 0.71; 95% confidence interval, 0.42-1.19). Consistent results were found in sensitivity analyses including a case-time control design. LIMITATIONS: No information on disease activity/severity was available. CONCLUSION: Our findings indicate an opportunity to reduce the risk of cardiovascular events in patients with LE through use of hydroxychloroquine.

Systematic Review: Monoclonal Antibody-Induced Subacute Cutaneous Lupus Erythematosus.

BACKGROUND: Subacute cutaneous lupus erythematosus (SCLE) lacks consensus diagnostic criteria and the pathogenesis is poorly understood. There are increasing reports of SCLE induced by monoclonal antibodies (mAbs), but there are limited data on the aetiology, clinical characteristics and natural course of this disease. METHODS: We devised a set of diagnostic criteria for SCLE in collaboration with a multinational, multispecialty panel. This systematic review employed a two-layered search strategy of five databases for cases of mAb-induced SCLE (PROSPERO registered protocol CRD42019116521). To explore the relationship between relative mAb use and the number of SCLE cases reported, the estimated number of mAb users was modelled from 2013 to 2018 global commercial data and estimated annual therapy costs. RESULTS: From 40 papers, we identified 52 cases of mAb-induced SCLE, occurring in a cohort that was 73% female and with a median age of 61 years. Fifty percent of cases were induced by anti-tumour necrosis factor (TNF)-ɑ agents. A median of three drug doses preceded SCLE onset and the lesions lasted a median of 7 weeks after drug cessation. Oral and topical corticosteroids were most frequently used. Of the licensed mAbs, adalimumab, denosumab, rituximab, etanercept and infliximab were calculated to have the highest relative number of yearly users based on global sales data. Comparing the number of mAb-induced SCLE cases with estimated yearly users, the checkpoint inhibitors pembrolizumab and nivolumab showed strikingly high rates of SCLE relative to their global use, but ipilimumab did not. CONCLUSION: We present the first systematic review characterising mAb-induced SCLE with respect to triggers, clinical signs, laboratory findings, prognosis and treatment approaches. We identify elevated rates associated with the use of checkpoint inhibitors and anti-TNFɑ agents.

Association Between Drug Use and Subsequent Diagnosis of Lupus Erythematosus.

Importance: It has been estimated that up to 30% of all subacute cutaneous lupus erythematosus (CLE) cases and up to 15% of systemic lupus erythematosus (SLE) cases are drug induced. Based on numerous case reports and several epidemiologic studies, more than 100 drugs from more than 10 drug classes are suspected to cause drug-induced lupus erythematosus. Objective: To examine the association between drug use and a subsequent diagnosis of CLE or SLE based on a systematic screening process of the drugs in the Anatomical Therapeutic Chemical classification system in a nationwide setting. Design, Setting, and Participants: A matched case-control study was conducted using all incident cases of CLE and SLE registered in the Danish National Patient Register between January 1, 2000, and December 31, 2017. Patients with CLE and patients with SLE were matched (1:10) on age and sex, with individuals from the general population serving as controls. Exposures: To select which drugs to examine for an association with CLE or SLE, a screening process of all drugs was performed, including drugs filled at pharmacies and drugs administered in hospitals. Main Outcomes and Measures: Odds ratios (ORs) were calculated for the association between exposures to certain drugs and the subsequent diagnosis of CLE or SLE. Results: In all, 3148 patients with CLE (n = 1298; 1022 women [78.7%]; median age at diagnosis, 50.5 years [interquartile range, 39.4-62.2 years]) or SLE (n = 1850; 1537 women [83.1%]; median age at diagnosis, 45.0 years [interquartile range, 33.6-56.4 years]) and 31 480 controls (25 590 women [81.3%]; median age, 47.5 years [interquartile range, 35.9-59.5 years]) were found. Many significant associations between drug use and a subsequent diagnosis of CLE and SLE were observed. Many associations were likely due to protopathic bias. However, new plausible causal associations were observed between CLE or SLE and some drugs, including fexofenadine hydrochloride (SLE: OR, 2.61 [95% CI, 1.80-3.80]; CLE: OR, 5.05 [95% CI, 3.51-7.26]), levothyroxine sodium (SLE: OR, 2.46 [95% CI, 1.97-3.07]; CLE: OR, 1.30 [95% CI, 0.96-1.75]), metoclopramide hydrochloride (SLE: OR, 3.38 [95% CI, 2.47-4.64]; CLE: OR, 1.47 [95% CI, 0.85-2.54]), and metronidazole hydrochloride (SLE: OR, 1.57 [95% CI, 1.09-2.27]; CLE: OR, 1.93 [95% CI, 1.25-2.97]). Conclusions and Relevance: The study's findings suggest that physicians should be cognizant about whether a new case of CLE or SLE could be drug induced. Furthermore, the results highlight that the reported associations in the published literature may be due to publication or protopathic bias.

Clinical features, disease activity and outcomes of Malaysian children with paediatric systemic lupus erythematosus: A cohort from a tertiary centre.

BACKGROUND: Paediatric systemic lupus erythematosus is a rare autoimmune disease with a wide spectrum of clinical presentation in different populations. We present a cohort of paediatric systemic lupus erythematosus in Malaysia where the disease features and outcomes are still largely unknown. METHODS: A retrospective review of all paediatric systemic lupus erythematosus patients with at least 6 months follow-up at Selayang Hospital from 2004 to 2016. Epidemiological, clinical and outcome data were collected and analysed. RESULTS: A total of 141 paediatric systemic lupus erythematosus patients, 87.9% females, were followed up for a median 6.3 years (interquartile range 3.6-9.0). The median age at diagnosis was 10.8 years (interquartile range 9.0-12.0 years), positive family history of systemic lupus erythematosus was present in 12.1% and the majority (61.7%) were of Malay ethnicity. Common presentations included fever (87.2%), vasculitic rash (72.3%) and lethargy (69.5%). At diagnosis, leukopenia (51.1%), thrombocytopenia (41.8%) and cutaneous lupus (56%) predominate with significant renal involvement (39.7%). Renal (45.4%), liver (26%) and the central nervous system (17%) were important major organs involved during the course of the disease. At diagnosis, almost all (99.3%) patients had high disease activity (mean Systemic Lupus Erythematosus Disease Activity Index score 20.1 ± 9.6). The majority (62.4%) achieved remission or low disease activity after 6 months, maintained over the next 10 years. Damage occurred early (39.1% at 1 year) and increased with time. Ocular damage was the most common side effect (29%) and was predominantly corticosteroid related (93%). Growth retardation was significant (38.2%) with no gonadal failure or secondary malignancies. End-stage renal disease occurred in 3.1% patients whereas 53.1% had sustained renal remission. Overall mortality was 1.4%. CONCLUSION: Despite high disease activity at diagnosis, the majority had good sustained response to treatment with low overall mortality. However, there was progressive accrual of organ damage, highlighting the need for further research and refinements into therapies for paediatric systemic lupus erythematosus.