Dr. Rithwik Sunil , Dr.Vinitha Prasad, Dr.Stefy, Dr. Shela, Dr C Jayakumar
Thirteen year old female child presented with throbbing headache more on the right side since 5 days with photo -phono phobia. To begin with she had 2 to 3 episodes non projectile, non billous, non blood stained vomiting and passage of orange coloured urine . Treatment for migraines run outside AIMS didn’t have any improvement
Labs done
Haemoglobin 4.1g/dl
Child was then referred to AIMS for further management.
Negative history
No fever, fast breathing, grunting,bleeding manifestations, arthritis, rash ,abdominal pain ,history ,drug intake fall or trauma
Past History: no history of previous admissions in the past
Perinatal ,development, is moral and immunized for age
History of migraine is noted for mother
Examination
Vitals stable
BP: 1108/70mmhg
Afebrile, tired and pale looking
icteric , Right cervical lymphadenopathy.
No cyanosis, clubbing or edema
Systemic examination
Liver palpable , Liver span of 12 centimetres ,spleen palpable
Other systems : Within normal limits
Labs done
Vitamin B12: 377
CRP: 3
Hb: 4g/dl
ESR:140
Platelet :114K/uL
Tc;5.93K/uL
N:79%
L:14%
DCT:+4
C3:53mg/dl(90-180)
C4:4mg/dl(10-40)
LDH:765U/L
MCV:124
MCH: 41
MCHC: 33.1
TSB: 2.30
DSB:0.51
Peripheral smear : macrocytic anemia
Differentials that can be considered;
IDA
Mycoplasma pneumonia causing AIHA
EBV
Parvovirus B19
SLE associated AIHA
Cold AIHA
Kikuchi-Fujimoto disease
Given the history that it is a female child with low complement levels, high MCV anemia, we should consider hemolytic anemia. Since the CRP is negative, an infectous patology is less likely. DCT is strongly positive, an underlying hemolytic anemia is confirmed.
ANA IF sent was +3 positive showing mixed pattern
Child was treated as a case of childhood onset SLE. She was started on Pulse steroids
Childhood Onset SLE
The presentation of SLE in childhood or adolescence differs somewhat from that seen in adults. The most common presenting complaints of children with SLE include fever, fatigue, hematologic abnormalities, arthralgia, and arthritis.
A positive ANA test is present in 95–99% of SLE patients. ANA has poor specificity for SLE, however, because up to 20% of healthy individuals also have a positive ANA test result, making the ANA a poor screen for SLE when used in isolation. High titers are more suggestive of underlying autoimmune disease, but ANA titers do not correlate with disease activity, so repeating ANA titers after diagnosis is not helpful. Antibodies to dsDNA are specific for SLE, and in many individuals, anti-dsDNA levels correlate with disease activity
Corticosteroids are a treatment mainstay for significant manifestations of SLE and work quickly to improve acute deterioration
Take home message
It is vital to consider SLE in adolescents with AIHA.
It is important to limit dose and length of exposure to corticosteroids whenever possible. Potential consequences of corticosteroid therapy include growth disturbance, weight gain, striae, acne, hyperglycemia, hypertension, cataracts, avascular necrosis, and osteoporosis.