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Consultant Rheumatologist at the Blackrock Clinic, Dublin, Dr Nicola Ambrose discussed cases of junior systemic lupus erythematosus (JSLE), junior dermatomyositis (JDM) and associated difficulties experienced in “emerging adulthood” during her presentation at the ISR meeting.
Dr Ambrose, in her presentation entitled, “Juvenile onset SLE and Dermatomyositis – A young adult experience”, highlighted cases treated during her time at University College London Hospitals (UCLH) NHS Foundation Trust, where she was a clinical lead in adolescent rheumatology.
Patients suffering from JSLE experience frequent flares and their conditions are often complicated, both psychosocially and medically, Dr Ambrose, who has published 23 peer review papers and six book chapters, explained. In terms of mortality, individuals are more likely to die from SLE if they are older; however, standardised mortality ratios were significantly increased relative to the baseline population for those aged between 10-30 years. The standardised morality rate in JSLE patients was 18.3 versus 3.1 in the whole adult group, she said.
The first case examined involved an 18-year-old female, who was diagnosed with SLE at the age of 12, where non-adherence to medication was a significant issue. On presentation to the adult service at UCLH, the patient said they had been hallucinating. The patient also presented with a vasculitic rash; malar rash; widespread erythematous rash; mouth ulcers; alopecia; and was afebrile. Further questioning revealed the patient had been suffering from irritability and grandiose thinking. A diagnosis of neuropsychiatric lupus was reached.
The long-term outlook of JSLE shows that remission appears to be uncommon and that a lifetime of flares and medications is likely, Dr Ambrose explained. In SLE, most pregnancies are successful, Dr Ambrose said, but SLE with active disease and especially with nephritis has poorer pregnancy outcomes, including: Spontaneous abortion; intrauterine fetal death; preterm birth; neonatal lupus; and intrauterine growth restriction. Maternal complications in SLE patients include SLE flares, preeclampsia and arterial hypertension.
Turning to JDM, Dr Ambrose outlined that the condition is rare, affecting 3.2 per million per year, but that it is the most common myositis in children. The average age of onset of JDM is seven years. A definite diagnosis is made if at least three of the following are identified: Symmetrical proximal myopathy; raised muscle enzymes; abnormal EMG; and characteristic muscle biopsy.