We describe a case of Parry Romberg syndrome/en coupe de sabre in a woman whose disease started as seizures at age 8 but was diagnosed at the age 39. During these 31 years she got married, completed a first degree at university, had two successful pregnancies and has been gainfully employed. The features of generalized tonic-clonic seizures, autoimmune abnormalities, ocular abnormalities, morphea en coup de sabre and brain imaging abnormalities were present. Areas of parietal lobe cerebral calcification were encountered on the computed tomographic scan and bilateral periventricular white matter changes on the magnetic resonance imaging with frontal, temporal and parietal lobe brain atrophy ipsilateral to the facial hemiatrophy. Clinical, immunologic and neuroradiological abnormalities are discussed. In some cases, this illness can run a benign and stable course.

Sample size n=28 (Results were not obtained for 4 patients) **Sample size n=140 ***Sample size n=139

myocardial infarction (MI) and stroke among SLE patients compared to age-matched controls. The objective of our study was to examine the rates of non-fatal MI, stroke, and the combined endpoint of non-fatal MI or stroke, overall and by race/ethnicity, among SLE patients enrolled in Medicaid. Materials and methodsWithin Medicaid Analytic eXtract (MAX), containing billing claims from 2000-10 for Medicaid patients from the 29 most populated US states, we identified patients aged 18-65 with prevalent SLE ( 3 ICD-9 codes 710.0, 30 days apart) with >12 months of continuous enrollment prior to 3 rd code (index date). Baseline data from 12 months prior to index date included age, sex, race/ethnicity, zip code, year, SLE-related and other comorbidities, including CVD risk factors (based on ICD-9 and DRG codes). Those missing race/ ethnicity were excluded. Subjects were followed from index date to first MI or stroke event, death, Medicaid disenrollment, or end of follow-up. MI, stroke, and combined outcome per 1000 person-years with 95% CIs were calculated overall and by race/ ethnicity. Subdistribution proportional hazards regression models, accounting for the competing risk of death, were used to calculate multivariable-adjusted hazard ratios (HRsd) for MI, stroke, and combined outcome. Results Among 43,448 cases with prevalent SLE, 93.6% were female. Racial/ethnic breakdown was: 41% Black, 39% White, 15% Hispanic, 3% Asian, 1% Native American. Mean follow-up was 3.48 ± 2.86 years for all SLE patients. Overall crude rates were highest among Native Americans for MI, Blacks for stroke, and Native Americans for MI or stroke. Hispanics had the lowest overall crude rates for MI, stroke, and the combined outcome. After multivariable adjustment and accounting for the competing risk of death, Hispanics had lower MI risk (HRsd] 0.59 [95% CI: 0.42-0.84]) and Blacks had elevated risk of stroke (HRsd 1.36 [95% CI: 1.15-1.60]) as compared with Whites. For the outcome of MI or stroke, Blacks had an elevated risk (HRsd 1.22 [95% CI: 1.07-1.38], whereas Hispanics had a lower risk (HR 0.82 [95% CI: 0.67 to 0.99] compared to Whites. Conclusions Marked race/ethnicity-specific variation exists in MI and stroke risks among Medicaid patients with SLE. Elevated CVD risks among Blacks and lower risks among Hispanics may account for some of the excess all-cause mortality observed among Black patients and lower overall mortality among Hispanics with SLE as previously described.

No abstract

Background Juvenile Idiopathic Inflammatory Myopathy (JIIM) is a rare autoimmune condition of which Juvenile Dermatomyositis (JDM) is the most common subtype. To this date there has been no published data focusing on this condition in Trinidad nor the English-speaking Caribbean. Methods A retrospective folder analysis was done on patients with JIIM who attended the recently formed Paediatric Rheumatology Multi-Disciplinary Clinic over a 6 months period from May 2021 at San Fernando Teaching Hospital, Trinidad and Tobago. Demographics, investigations and diagnoses were analysed. A secondary comparative analysis of two diagnostic criteria was performed and treatment approach was examined. Results 5 notes were reviewed in this period. The male: female ratio was 1:4 and the average age of onset was 6.8 years. 80% of JIIM cases were JDM. The most common presenting complaints were: skin rash and weakness (62%). The most common additional symptoms were: joint pain (60%) and pruritis (60%). Previous upper respiratory tract infection was recognized in 40% of JIIM patients and one patient had Kawasaki disease 6 years prior. Creatine Kinase showed modest elevations in JDM, whilst it was extremely elevated in one patient with myositis. There was an 80% concordance between the EULAR/ACR 2017 and the Peter and Bohan 1975 criteria. 60% tested positive for ANA. Anti PM, Anti Ku, Anti RP155, Anti Mi2A, Anti Mi2B and Anti RP11 antibodies were identified. 40% were positive for AntiRP155. 60% of patients utilized MRI imaging which detected myositis in all reports. Methotrexate and prednisolone were used in 100%. Parenteral drugs used included: intravenous immunoglobulin and methylprednisolone. 80% had vitamin D supplementation and 100% were prescribed physiotherapy. One child had intractable calcinosis. Conclusions Whilst the study size was arguably small, patterns were seen which are in keeping with international data on epidemiology and treatment. Recommendations from this study include: greater utilization of MRI in diagnostic workup, further evaluating anti RP155 antibody as a new myositis associated antibody in a Caribbean population and exploring further options for calcinosis treatment in a developing nation.