Intracranial hypertension with delayed puberty: a rare presentation of juvenile onset systemic lupus erythematosus

Mathew M, Cherian A
Correspondence: Dr Ajith Cherian, drajithcherian@yahoo.com

ABSTRACT
An adolescent boy presented with headache, bilateral papilloedema, growth retardation and absent secondary sexual characteristics. The diagnosis of intracranial hypertension was confirmed by increased intracranial pressure and normal neuroimaging of the brain except for partial empty sella and prominent perioptic cerebrospinal fluid (CSF) spaces. Evaluation showed an erythrocyte sedimentation rate of 150 mm/hr, positive antinuclear antibody, anti-dsDNA and antiribosomal P protein. Renal biopsy revealed diffuse segmental proliferative lupus nephritis (LN) class IV-S (A), which confirmed the diagnosis of systemic lupus erythematosus (SLE). Treatment of LN with intravenous pulse methylprednisolone and cyclophosphamide normalised the patient’s CSF pressure and symptoms. In cases of intracranial
hypertension, SLE must be considered. Growth retardation and absence of secondary sexual characteristics could coexist and may be presenting features of SLE.
These manifestations point to advanced grades of LN, which could be asymptomatic and may be missed without a renal biopsy.

Keywords: antinuclear antibody, cyclophosphamide, erythrocyte sedimentation rate, methylprednisolone, papilloedem
Singapore Med J 2012; 53(1): e15–e17