Objective: To study the clinical features and laboratory parameters of neonatal lupus erythematosus (NLE) from India.
Patients and methods: We analyzed case records of children diagnosed with NLE in the Pediatric Rheumatology Clinic at tertiary care centre from North India during the period January 1999 - December 2023.
Results: Twenty-four babies are diagnosed with NLE during the study period. Median age at diagnosis was 60 days with a female predisposition (Male to female- 1:2). Cutaneous manifestations were reported in 14 (58%) patients. Hepatomegaly was noted in 15 (62.5%), and splenomegaly in 2 (8%). Elevated transaminases were noted in 14 (58%) patients, and cholestatic jaundice in one. Hematological manifestations were noticed in 20 (83.3%), and neurological manifestations were noticed in 8 patients. Most non-cardiac manifestations were self-limiting. Intravenous immunoglobulin was used for phrenic nerve palsy and autoimmune hemolytic anemia. Oral corticosteroid was used in a patient with refractory cytopenia, and four patients required transfusions. Cardiac involvement was reported in 13 (54%) patients - 3rd-degree heart block in 9 patients, and 6 were managed with epicardial pacemaker insertion. One patient required pacemaker reimplantation due to infective endocarditis. Congenital hydrops was seen in 3 patients, and required respiratory support and recovery was uneventful.
Conclusion: NLE is associated with significant morbidity. More than half had cardiac manifestation, of which 70% had 3rd-degree heart block requiring pacemaker insertion. Other rare clinical manifestations like phrenic nerve palsy and seizures are noticed in our cohort.
Keywords: anti-La; anti-Ro; antinuclear antibodies; bradycardia; congenital heart block; cytopenia; hydroxychloroquine; neonatal lupus erythematosus; pacemaker.