AB0949 IS PEDIATRIC ONSET LUPUS MORE SEVERE IN BOYS? OUR EXPERIENCE AT A TERTIARY CARE CENTER IN NORTH-WEST INDIA

Abstract

Background There is diversity in clinical presentation of pediatric onset SLE (pSLE) and the manifestations are more severe as compared to adults. Males comprise 4-22% of all SLE patients. Gender distribution of pSLE is 4.5-5: 1 (female-to-male) as opposed to 9-10: 1 (female-to-male) in the adult population. However male patients have been known to have a more severe disease with higher morbidity, especially due to renal causes. Objectives To evaluate clinical and immunological profile and outcomes in a follow up series of males with pediatric onset SLE Methods We analyzed the clinical and immunological profile and outcome of male patients diagnosed with SLE at less than 16 years of age. These children were followed-up in Pediatric Rheumatology Clinic, Advanced Pediatrics Centre, Postgraduate Institute of Medical education and research, Chandigarh, India. Details on demographic data, clinical presentation, laboratory findings, immunological profile, treatment regimens and outcomes of these children were retrieved from clinic files. Results Forty-three boys were diagnosed to have SLE between January 1998 to December 2018. Mean age at presentation was 9.7 years (range: 9 months-12 years). Total patient years of follow up was 192 years. The most common clinical presentation was fever in 38 (88%) patients; rash in 27(63%); pallor in 21(49%); edema with urinary abnormalities in 17(40%) and photosensitivity in 16 (37%). A diagnosis of lupus nephritis was made in 25 (58%) patients out of which 17 (39.5%) had nephritis at presentation. Renal biopsies were performed in 18 patients; 11 had class IV disease, 2 had class 5 disease, 2 had class IV/V. Neuropsychiatric manifestations were seen in 11 (26%) patients- 7 of these had symptoms at presentation. Seizures were the predominant manifestation in 9 patients (21%)-6 of these had MRI changes consistent with posterior reversible encephalopathy syndrome while 3 had cerebral infarcts. Other central nervous system abnormalities included psychosis (1 patient) and chorea (1 patient). We also noted a family history of lupus like illness in 4 patients and 3 (6.9%) were found to have early complement deficiencies. Antiphospholipid antibodies (aPLA) were detected in 8 (18.6%)patients - anticardiolipin antibody was positive in 8 and lupus anticoagulant was positive in 6. Dual aPLA positive was seen in 6(13.9%) and triple positive was positive in none. Infections were seen in 16 (37%) patients during follow-up. All patients received steroids in gradually tapering doses along with hydroxychloroquine following a diagnosis of SLE. Cyclophosphamide was given for induction in 13 patients who had severe forms of lupus nephritis. Remission was maintained through azathioprine in 8 patients and 8 required Mycophenolate mofetil. Ten patients (23%) had a relapse on therapy -1 had CNS relapse, 3 had renal relapse, 2 had muco-cutaneous relapse and 4 had hematological relapse. Six fatalities (14%) were recorded during follow-up -1 with severe disease activity and neuropsychiatric manifestations, 1 had disseminated tuberculosis, 1 had CNS flare with status epilepticus, 4 had sepsis. Conclusion This is one of the largest series on boys with pediatric onset SLE from a developing country. It appears that while the severity of lupus nephritis in boys is no different from that in girls, neurological disease is more severe in the former. Further, boys appeared to have earlier onset of neuropsychiatric lupus as compared to girls. The incidence of complement deficiency lupus was also more in boys. Mortality in boys with SLE appears to be comparable to our previously published mortality data on pSLE(1).