Welcome to PICU Doc On Call, A Podcast Dedicated to Current and Aspiring Intensivists.
I'm Pradip Kamat and I'm Rahul Damania. We are coming to you from Children's Healthcare of Atlanta - Emory University School of Medicine.
Welcome to our Episode of 17-year old with h/o of SLE and now acute liver failure.
Here's the case presented by Rahul:
A 17-year old teenage female year old presents to the PICU with acute liver failure. Important past h/o includes a diagnosis of SLE on therapy with prednisone, mycophenolate (cellcept), and plaquenil.
4 days prior to this admission, patient presented to an OSH with RUQ pain, vomiting (non bloody & no bilious), fever & malaise. Initially due to concern for "lupus Flare" patient was given steroids at the OSH.
At the OSH notable initial labs included a mild transaminitis and an INR of 1.5. She suddenly at the OSH developed fluid refractory hypotension and was started on a pressor. Due to continued worsening of her transaminitis well as a rising INR on her repeat labs she was referred to our tertiary PICU for further management.
Pertinent history also includes a negative urine pregnancy test. No recreational drug use, and only as needed use of Tylenol.
She now is in the PICU. She generally appears tired and ill. She is tachypneic on 4 LPM of nasal canulla and her oxygen saturation is 98%. She has a non-focal lung exam.
Her cardiac exam is notable for tachycardia, and pertinently no gallop, rub or murmur.
Her abdominal exam is non-focal except for mild discomfort on palpation of the RUQ with a palpable liver edge. Her extremities are cool with 3-4 capillary refill time. She is able to answer questions but intermittently doses off. No rash is noted.
To summarize key elements from this case, this patient has:
H/o of lupus and is on immunosuppressive medications
New onset fever/malaise
This sounds like a LUPUS flare as she has a clinical picture of generalized inflammation.

Rahul: Lets pause right here and take a look at key history and physical exam components in a patient who has a chronic auto-immune condition:
Fever, malaise and feeling tired all signs of constitutional symptoms.
She has abdominal pain and vomiting that could again be related to systemic inflammation but also an intra-hepatic lesion.

Are there some red-flag symptoms or physical exam components which you could highlight?
This patient has signs of shock!
Tachycardia with delayed cap refill and cool extremities
Tachypnea
& hepatomegaly which could indicate increased central venous pressures.
Initially her outside presentation of fluid refractory shock is of utmost concern!
Fluid refractory shock with multi organ presentation involving liver, kidney and the blood/coagulation systems
All of these elements bring up a concern for some acute life threatening process such as sepsis, or even immune dys-regulation due to her h/o of Lupus
To continue with our case, the patients labs were consistent with:Acute liver dysfunction (Elevated AST and ALT in the thousands, Total bilirubin 1.6, GGT 56) although the total bilirubin is not elevated to a degree I would expect.
AKI (creatinine 2.18)
An uptrending Coagulopathy with elevated PT and INR: PT 120 and a peak INR of 16
Thrombocytopenia: Platelets < 50K
She had a peak lactate 9.2
and concurrent Metabolic acidemia: serum HCO3 7, and pH 7.18.
A Pertinent negative: Normal serum ammonia <38 micromol/L (nl < 50)
Finally, she had an elevated WBC 20.5K/ Hgb 9.7, Platelet 42. CRP 4.2/ESR 5

OK to summarize, we have: a 17 yr old female with SLE on mycophenolate (cellcept) who presents with fever, hypotension, AKI and liver dysfunction with severe coagulopathy, although we do not have other labs- This brings up the concern for acute macrophage activation syndrome (MAS) the topic of our discussion today.
Let's start with a short multiple choice question:
12 year old male with h/o systemic onset juvenile idiopathic arthritis (JIA) presents