Complete resolution of juvenile neuropsychiatric lupus with low dose rituximab

Nervous system involvement leads to high mortality in juvenile systemic lupus erythematosus. We present the case of a 12-year-old girl who was admitted with a history of recurrence of fever, cervical swellings, seizure, and delirium. Approximately 6 months back, she was started on antitubercular therapy (ATT) for fever and granulomatous lymphadenitis. She developed a recurrence of fever, new cervical swellings, seizures, and delirium while she was continuing ATT. Magnetic Resonance Imaging brain revealed multiple hyperintensities in bilateral basal ganglia, temporal, and hippocampal regions. The patient was diagnosed to have lupus based on clinical features, positive antinuclear antibody, positive double-stranded DNA antibody, and hypocomplementemia. To avoid cyclophosphamide-related gonadotoxicity in this young girl, Rituximab was given. She was treated with two doses of Rituximab and there was complete resolution of her neuropsychiatric SLE. Rituximab can be considered over cyclophosphamide in children to avoid gonadotoxicity.

Antinuclear Autoimmunity Potentially Makes the Middle-aged and Female with COVID-19 Prone to Severe Progression: A Case-control Study

BackgroundElderliness is known risk factor for severe progression of COVID-19 due to compromised immunity, however aberrant hyperactive immune response including autoimmunity might be responsible for younger patients.Methods162 patients tested with autoimmunological detections were enrolled, and study of “Severe” cases and “Non-severe” controls was retrospectively analyzed. ResultsMultivariable analysis involving antinuclear autoimmunity manifests correlation of disease severity with middle age and attenuates the risk of age older than 65. Middle age (45≤age≤65) and female turn out to be the risk factors after hierarchical cluster analysis, before which however sex was not correlated. We find antinuclear autoimmunity to be strongly correlated with severity for the middle-aged (OR= 21.000, 95% CI 4.893- 90.126, p< 0.001) and female (OR= 16.044, 95% CI 4.717- 54.568, p< 0.001), especially for the middle-aged female (Pearson R= 0.770, p< 0.001). Incidence of symptoms fever and chest distress, and complication myocardial injury are statistically more frequent in patients with positive antinuclear antibody, compared with those negative. Severe patients with positive antinuclear antibody possess significantly shorter onset of symptoms to severity time (p= 0.021), indicating quicker progression, and interestingly, present more incidence (21%) of post-remission aggravation, compared with those negative (6%). ConclusionsThe presence of antinuclear autoimmunity potentially makes COVID-19 prone to severe progression, especially for the middle-aged and female, probably even quicker.

Autoinflammatory/Autoimmunity Syndrome Induced By Adjuvants (ASIA) Due to Silicone Incompatibility Syndrome

The adjuvant-induced autoimmune syndrome (ASIA) is associated with a dysregulation of the innate and adaptive immune system after exposure to chemical compounds, including liquid paraffin, silicone gel, acrylamides, and hyaluronic acid. Due the increase of the use of these compounds in cosmetic procedures, the prevalence of this syndrome is increasing. We present the first report in Ecuador associated to ASIA after an elective silicone breast prosthesis procedure, manifested as polyarthralgia, positive antinuclear antibody, anticentromere antibody, and a moderate positive Sclero-70.

Lupus enteritis as a lone manifestation of SLE: rare but possible

A 28-year-old Southeast Asian non-pregnant woman with asthma and prior cholecystectomy presented to the emergency department with acute watery diarrhoea, intermittent abdominal pain and vomiting. Apart from abdominal tenderness, the rest of the physical examination was unremarkable. She had leucocytosis, alkaline phosphatase elevation and exudative ascites. Radiological imaging ruled out biliary leak and was only significant for circumferential small and large bowel thickening. Upper endoscopy and colonoscopy showed normal duodenal and colonic mucosae. Both infectious and malignancy workup were also unremarkable. Bereft of other systemic symptoms, autoimmune pathology was initially deemed unlikely; however, autoimmune workup revealed positive antinuclear antibody, double-stranded DNA, anti-Smith antibody, antinuclear ribonucleoprotein and hypocomplementaemia. With multidisciplinary collaboration, the patient was initiated on high-dose steroids, which dramatically improved her symptoms. She was discharged home with a steroid taper, and at 3 months of follow-up with her rheumatologist, she was continued on steroids and hydroxychloroquine.

E-consults: an effective way to decrease clinic wait times in rheumatology

Background To evaluate the effect of E-consults on wait times and resource utilization for positive antinuclear antibody (ANA) referrals in outpatient rheumatology. Methods We conducted a pre-post study of E-consult implementation for positive ANA referrals. We retrospectively reviewed “positive ANA” referrals from 1/2015–3/2017. A statistical process control chart was created to display monthly average wait times for in-person clinic visits and to identify special cause variation. Final diagnoses, wait times and resource utilization were recorded and compared between E-consults and in-person referrals. Results There were 139 referrals for positive ANA with 126 occurring after E-consult implementation in August 2015. Forty-four percent (55/126) of referrals were E-consults; 76% did not have an in-person visit after initial electronic rheumatology recommendation. A control chart demonstrated special cause variation in the form of a shift from June 2016 – January 2017, suggesting a temporal association between decreased wait times and the implementation of E-consults. Eleven patients were diagnosed with ANA-associated rheumatic disease; the majority of patients (73%, 86/139) did not have a rheumatologic diagnosis. Overall E-consults utilized more labs than in-person visits, but this was not statistically significant. In-person visits utilized more imaging studies, which was statistically significant. Conclusion E-consults are an effective way to address positive ANA consults without significant increase in resource utilization and were temporally associated with decreased wait times for in-person visits.

Rowell’s syndrome: a rare but distinct entity in rheumatology

Rowell’s syndrome is a rare disorder characterised by an association of lupus erythematosus with erythema multiforme (EM)-like skin lesions. EM as the initial clinical presentation of systemic lupus erythematosus is also atypical and even rarer. We report the case of an 18-year-old girl admitted to our hospital with fever and polyarthralgia along with multiple discrete ill-defined target lesions with crust formation over forehead, cheek, external ears, scalp, upper chest and back (predominantly over sun-exposed areas) with ulceration over hard palate. Investigations revealed pancytopaenia, a positive rheumatoid factor, positive antinuclear antibody with a speckled pattern, anti-Smith antibody and strongly positive anti-Ro. Patient was diagnosed with Rowell’s syndrome as per clinical and laboratory features. Majority of skin lesions including oral ulcerations subsided gradually after treatment with steroids and hydroxychloroquine.

E-Consults: an effective way to decrease clinic wait times in Rheumatology

Background: To evaluate the effect of E-consults on wait times and resource utilization for positive antinuclear antibody (ANA) referrals in outpatient rheumatology.Methods: We conducted a pre-post study of E-consult implementation for positive ANA referrals. We retrospectively reviewed “positive ANA” referrals from 1/2015-3/2017. A statistical process control chart was created to display monthly average wait times for in-person clinic visits and to identify special cause variation. Final Diagnoses, wait times and resource utilization were recorded and compared between E-consults and in-person referrals.Results: There were 139 referrals for positive ANA with 126 occurring after E-consult implementation in August 2015. Forty-four percent (55/126) of referrals were E-consults; 76% did not have an in-person visit after initial electronic rheumatology recommendation. A control chart demonstrated special cause variation in the form of a shift from June 2016 – January 2017, suggesting a temporal association between decreased wait times and the implementation of E-consults. Eleven patients were diagnosed with ANA-associated rheumatic disease; the majority of patients (73%, 86/139) did not have a rheumatologic diagnosis. Overall E-consults utilized more labs than in-person visits, but this was not statistically significant. In-person visits utilized more imaging studies, which was statistically significant. Conclusion: E-consults are an effective way to address positive ANA consults without significant increase in resource utilization and were temporally associated with decreased wait times for in-person visits.

E-Consults: an effective way to decrease clinic wait times in Rheumatology

Background: To evaluate the effect of E-consults on wait times and resource utilization for positive antinuclear antibody (ANA) referrals in outpatient rheumatology.Methods: We conducted a pre-post study of E-consult implementation for positive ANA referrals. We retrospectively reviewed “positive ANA” referrals from 1/2015-3/2017. A statistical process control chart was created to display monthly average wait times for in-person clinic visits and to identify special cause variation. Final Diagnoses, wait times and resource utilization were recorded and compared between E-consults and in-person referrals.Results: There were 139 referrals for positive ANA with 126 occurring after E-consult implementation in August 2015. Forty-four percent (55/126) of referrals were E-consults; 76% were resolved after initial electronic rheumatology recommendation. A control chart demonstrated special cause variation in the form of a shift from June 2016 – January 2017, suggesting a temporal association between decreased wait times and the implementation of E-consults. Eleven patients were diagnosed with ANA-associated rheumatic disease; the majority of patients (73%, 86/139) did not have a rheumatologic diagnosis. Overall E-consults utilized more labs than in-person visits, but this was not statistically significant. In-person visits utilized more imaging studies, which was statistically significant. Conclusion: E-consults are an effective way to address positive ANA consults without significant resource utilization and were temporally associated with decreased wait times for in-person visits.