Welcome to the third world.
Parts of New Zealand, the Pacific Islands and outback Australia have horrendous rates of rheumatic fever and rheumatic heart disease.
So a quick refresher to those new to these islands:
Rheumatic fever is believed to be an autoimmune attack on the joints, heart, especially the valves, skin and sometimes the brain following a streptococcal throat or skin infection. The immune system in trying to attack the streptococci also attacks these other targets.
The typical patient is lower socio-economic and Maori, Pacific Islander or Aboriginee.
Sore throats and skin infections
Have a very low threshold for treating sore throats and skin infections in 3-45 year old people from populations with systemic antibiotics.
New Zealand guidelines for treating sore throats are here http://www.ttophs.govt.nz/vdb/document/364
Consider this diagnosis in any child from these populations with myalgias, arthralgias, reluctance to walk, oedema, rashes.
NZ Revised Jones Criteria for Acute Rheumatic Fever (ARF)
We don’t expect you to know the criteria per se, but rather to recognise them when you see them.
Rheumatic Heart Disease
With the first or subsequent episodes of rheumatic fever a patient may develop rheumatic heart disease which is where the heart starts to fail due to valvular disease. This is treated with diuretics, ACEI +/- valve replacement.
A 16 year old Maori girl presented with 1 week of twitching of her left arm and leg, a facial tic and intermittent slurred speech plus a swollen tender left wrist. She slept lying on her left arm to stop it from keeping her awake. She had had an episode of several sore joints earlier in the year but this was associated with starting a labouring job. She had had several sore throats over the preceding 12 months and a recent scabies infestation.
On examination she had irregular purposely writhing/twitching movements of her L hand, wrist and elbow and L leg, and occasional twitching of the left corner of her mouth and occasional slurred speech. Her left wrist was warm and tender.
Afebrile. Heart sounds normal. No rash. ECG normal.
Throat swab normal, raised antistreptococcal antibodies.
Echo showed mild aortic and mitral valvular thickening and mild MR and AR.
Diagnosis: Sydenham’s chorea (AKA St Vitus dance) secondary to rheumatic fever.
She was treated with monthly IM penicillin till age 25 and an anticonvulsant to control her chorea. 3 weeks later the anticonvulsant was stopped and she had no recurrence of chorea.
6 year old Maori boy brought to ED with sore legs and sore throat.
Fever and sore throat 2/52, sore joints 1/52. He is also more lethargic than usual. Several visits to GP.
Mum reports swollen and painful joints every 3-4 months for a period of the last two years. Several throat swabs in the past that have been positive for GAS (Group A Strep)
On examination knees and ankles tender and warm but not swollen, mild oedema of the ankles.
Temp 38.5. PR 115, BP 109/67, RR 21, SpO2 99% OA, GCS 15/15
No distress, no rashes
No subcutaneous nodules
Heart sounds: ESM loudest at apex 2-3/6, no radiation
Laterally displaced apex
Tonsils erethematous and swollen.
Throat swab grew GAS
Echo: Thickened leaflets causing moderate MVR, thickened aoritc valve leaflets and mild to moderate AVR.
He was treated with penicillin and is having monthly IM penicillin to age 25.
A 6 year old Maori girl was referred to ED by her GP with a flu like illness 2 weeks previously and one week of rash. She was generally unwell, lethargic, had intermittent fevers, a rash on her limbs and muscle and joint pain. She denied a sore throat.
HR 112, BP 106/69, RR 24, 39.0˚, CRT < 2 sec, 100% RA.
She had a macular urticarial rash on her arms, legs, abdo and buttocks. No purpura. No papules.
Cardiovascular examination was normal other than mild oedema of knees, ankles, elbows and wrists.
Urine showed 3+ blood.
The doctor who saw her, a very good doctor but from a first world country, considered post-strep glomerulonephritis, HSP and meningococcal infection.
Renal function was normal. Complement levels (apparently useful in diagnosis post-step GN) were pending.
The doctor thought this was a viral illness + maybe an allergic reaction to the child’s new house.
The child was discharged with antihistamine and steroid cream.
The child was brought back to ED 4 weeks later with painful swollen knees and ankles, blotchy intermitten tred ring-like rash of legs, buttocks and abdomen, lethargy, intermittent fevers and exertional dyspnoea and a noticable (by mum) cardiac impulse on the praecordium.
She was noted to have a pansystolic murmur. ECG normal. CXR: CCF. Antistreptococcal antibodies were raised. Throat swabs normal. Echo showed severe MR and probable AR.
She was treated with IV penicillin, started on frusemide and later had valve replacement surgery as treatment for her rheumatic heart disease.
“Is it Rheumatic Fever?” image from http://www.world-heart-federation.org/what-we-do/rheumatic-heart-disease-network/for-health-professionals/other-resources/links-to-rheumatic-heart-disease-programmes-websites-and-resources/
Music. Bernadette Seacrest and her Yes Men. Cold in My Bed https://itunes.apple.com/nz/album/no-more-music-by-the-suckers/id75310919