1. P438
Neuropsychiatric lupus with hepatitis in a child with CMV co- infection: Chicken first or the egg?
Sagar Bhattad, Vignesh P, Anju Gupta, Deepti Suri, Amit Rawat, Surjit Singh
Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh
Introduction
Hepatitis
Cytomegalovirus (CMV) infection at first presentation in pediatric SLE (pSLE) is a rare phenomenon
In a study of 670 patients with pSLE (1), CMV infection at the time of diagnosis was noted in 7 patients (incidence: 1.04%). This is the largest series reporting CMV in pSLE
Worked up extensively (table)
Liver biopsy – steatosis with hepatitis
Blood CMV PCR - Positive
Treatment
Intravenous (IV) methylprednisolone, IV cyclophosphamide monthly pulses (X 6) and ganciclovir
CMV hyper-immune globulin could not be administered due to financial constraints
Clinical details
12-year old girl
Low grade intermittent fever for 7 months
Maculopapular rash over trunk and photosensitive malar rash for 5 months
Jaundice for 1 month
Altered behavior with agitation, disorientation, fluctuating consciousness, hallucinations and altered sleep
Examination:
Small head, malar rash, icterus,hepatosplenomegaly, catatonia, mutism, low speech output, psychomotor retardation with rigidity
Outcome
Hallucinations, agitation and rigidity - passive,
improvement in psychomotor retardation.
Transaminitis gradually recovered, bilirubin normalized and on repeat testing, CMV was undetectable.
She is now doing well on maintenance immunosuppression and oral valganciclovir
Conclusion
Investigations
Pancytopenia, transaminitis, conjugated hyperbilirubinemia, normal renal functions (table)
Immunological tests: anti-nuclear antibody (ANA) - strongly positive (homogenous pattern on IIF), high anti-dsDNA with hypocomplementemia (table)
We present a challenging case of SLE with neuropsychiatric and hepatic manifestations along with CMV co-infection
Whether CMV came first or SLE remains unresolved
The ideal strategy of managing such patients is yet to be defined
1. Rozenblyum EV, Levy DM, Allen U, Harvey E, Hebert D, Silverman ED. Cytomegalovirus in pediatric systemic lupus erythematosus: prevalence and clinical manifestations. Lupus 2015;24(7):730-5
Table: Investigations in the index child
Investigation
Result
Hemoglobin (g/L) 87
ANA
3+ (homogenous)
Leucocyte counts (x 109/L) 6
Anti-dsDNA
733 IU/ml (<40)
Platelet counts (X 109/L) 20 C3 C4
68 (50-150)
6 (20-50)
Urea/creatinine
15/0.6
DCT
Anti-IgG +, C3d -
Serum protein/albumin
5.8/2.8
APL work up
negative
Total bilirubin/ direct
13/12
Serologies for HAV, HCV, HEV, HIV, EBV
AST/ALT/ALP
1650/120/455
CMV PCR
8160 copies/ml
PTI
50%
Anti-LKM/ SMA/ PCA
apTT
57 (<28)
MRI Brain
Cerebral atrophy
(DCT – Direct Coombs test, APL- anti-phospholipid, HAV- Hepatitis A, HCV- Hepatitis C, HEV- Hepatitis E, HIV- Human Immunodeficiency Virus, EBV – Epstein Barr Virus)