A 13 year old girl with history of lethargy, joint pain and cough....

A 13 years old girl presented to accident and emergency department with a history of lethargy, joint pain and cough. Both of her parents are from Jamaica. Her problem started one month ago with an upper respiratory infection. She was seen by her family doctor and diagnosed to have a viral infection. She has a past history of skin rashes that was never seen by her family doctor.
On examination she has a palpable cervical lymph nodes of various sizes, a congested throat, generalized myalgia and a swollen ankle joint on the left side.
Her BP is 120/75 mmHg, HR 90 bmp, RR 22/min.
A urine test shows protein+ve with red cells.
other tests are as follows:
Hb = 9.7 g/dl
TLC = 4x10-9/l with neutrophils and lymphopenia.
Platelets =100x10-9/l
Retic count = 2.6%
CRP = 20

Questions:
1. Which three other investigations should be carried out?
2. What treatments should you prescribe?
3. What are three possible differential diagnosis?

Answers:

1. Which three other investigations should be carried out?
The three investigations that need to be carried out are:
i- Blood film
ii- ESR
iii- Mycoplasma titres

2. What treatments should you prescribe?
- IV antibiotics
- Ibuprofen.

3. What are three possible differential diagnosis?
i- Juvenile chronic arthritis
ii- Mycoplasma infection
iii- Viral infection

She was admitted for further investigations and on the second night her oxygen requirement increased to 3 l/min via face mask to maintain a saturation of 93%.All cultures were negative.

Question:
4. Which single investigation should you carry out now?
 Answer: 
Chest X ray

Her condition got worse and was admitted in pediatric intensive care unit, she required ventilation and other procedures. Her BP was high 130/90mm Hg and she was treated with nifedepine. A kidney ultrasound scan was normal as well as hormonal studies.She was treated with antibiotics for 7 days. A viral titre shows IgG for EBV. A Mantoux test is very weakly positive although she has never received a BCG vaccine.

Questions:
5. Which three other investigations may help with the diagnosis?
6. What other treatment should be added at this stage?

Answers:
5. Which three other investigations may help with the diagnosis?
i- DNA double strand
ii- Pleural fluid biopsy
iii- Lymph node biopsy

6. What other treatment should be added at this stage?

i- Steroids

ii-NSAIDS

Discussion:

Systemic Lupus Erythmatosus (SLE)

SLE is a multisystem disorder with many manifestations. Patients may present with different signs and symptoms. There is a high chance of multi organ involvement. 

The commonest presenting symptom is joint swelling and looks like juvenile chronic arthritis. Skin rashes vary from  a butterfly rash on face to purpura , urticaria or just erythema of hands and soles of feet. Other features include renal involvement with glomerulonephritits, high BP, Myocarditis, pleural effusions, pericardial effusions, CNS involvement like seizures, confusion and focal neurological deficits. Blood disorders like hemolytic anemia ,thrombocytopenia and lymphopenia canalso be seen.

Autoantibodies are positive including ANA and anti-double stranded DNA. 

Corticosteroids can be given at a dose of 1-2 mg/kg/day with analgesia for pain relief.

Prognosis is not known but in general children do better than adults.