Neonatal lupus erythematosus: 24 years of experience from a tertiary centre at Chandigarh,North India

Abstract

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.