Rheumatology Flashcards
Child w/ abdo pain + arthritis. Purpura on buttocks. Likely Dx? MOST common cause of long-term morbidity?
- Henoch Schonlein Purpura
2. HTN and renal Dx
What is Henoch Schonlein Purpura?
- vasculitis of small vessel
- typically 4-6 y.o.
- IgA deposition
- URTI 1-3 wk before CC
- Triad: Palpable Purpuric rash + Arthritis + Abdo Pain
- No coag
- U/A monthly till 6 mo. and F/U for HTN
- Rare complications: hematuria, HTN, V/D, intussusception, neuro (h/a, sz), pul hemm
- 1/3 cases recur
What is HSP Dx criteria?
Petechiae/purpura + min. 1 of: > arthritis/ arthralgia > abdo pain > renal > histopathology (IgA deposition)
T or F: amount of arthritis dictate long term prognosis in HSP.
False.
- Severity of nephritis key (HTN, CKD)
List 3 complication of arthritis:
- Flexion contracture
- Leg length discrepancy
- Growth retardation
- Osteoporosis
3 y.o. right knee swelling. Best initial tx?
- steroid
- MTX
- NSAID
NSAID
Mono arthritis x few weeks. Likely test result
- (+) RF
- (+) ANA
- (+) WBC
- (+) ESR
- decreased Hgb
(+) ESR
Note: RF only if poly; help with prognosis
Post reactive arthritis with painless contracture. 3 non-pharm tx:
- Stretching/ Physio
- Bracing
- Botox
- Surgical
Monoarthritis. Name 3 meds from different class for Tx:
- NSAID
- ibuprofen, naproxen - Dx Modifying AntiRheumatic Drug (DMARD)
- MTX (hep, pneumonitis, hepatitis, cytopenia) - Systemic Corticosteroids
- Cytotoxic agent= Cyclosporine
- Biologic
- Adalimumab, Infliximab - Immunosuppressant
- Mycophenolate Mofetil
- Cyclophosphamide
Which is best confirmatory test for juvenile dermatomyositis?
- EMG
- CK
- ANA
EMG or BX
Key feature= proximal muscle weak + rash (Gottron knuckle, Heliotrope)
Other feature: Nailfold capillary dilatation, myalgia
Rheumatic fever mnemonic?
JONES= prior strep + 2 major or 1 major and 2 minor
Joints= migratory polyrarthritis O = draw hear instead= carditis N= nodules, subcutaneous E= erythema marginatum S= sydenham chorea
Minor: fever, arthralgia, ESR, CRP
Migratory polyarthritis. Likely dx?
acute rheumatic fever
How do you treat acute rheumatic fever?
- bed rest and watch for carditis
- ambulate once signs of ARF subsided
- Abx: 10d pen to eradicate strep infection
- ASA x 2mo (or joint pain resolves) or steroids if carditis
Secondary prophylaxis:
- ARF carditis but no heart dx= 10 yr or until 21 y.o.
- ARF without carditis= 5 yr or until 21 (whichever longer)
Child with migratory arthritis, fever, normal echo. ASOT high. Tx:
- ASA until arthritis gone
- ASA PRN and 10d pen
- ASA until arthritis gone and 10 d pen
- ASA until arthritis resolve, 10d pen, IM monthly pen
ASA until arthritis resolves
Tx 10 d w/ pen
Then q monthly IM pen
What is Reiter syndrome? list 4 organisms that could be linked to this
Reiter Syn:
- Uveitis, Urethritis, Arthritis
- can’t see, can’t pee, can’t climb a tree
4 organisms: Chlamydia trachoma’s, Salmonella, Shigella, Ureaplasma
8 y.o. choreoathetotic movement. Handwriting worse. Emotionally labile. Throat Cx (-). Likely dx?
- Huntington’s chorea
- Sydenham’s chorea
- SLE
Sydenham’s chorea
List a ddx for chorea
chorea= brief + random + all over
Genetic: Huntington, Ataxia Telangiectasis
Structural: Stroke, Mass, MS
Parainfectious/AI: Sydenham, SLE
Infection: HIV, Toxo
Metabolic: Na, Ca, thyroid
What is sydenham chorea?
Neuro manifestation of rheumatic fever (due to Group A strep)
What is the most common acquired case of chorea?
Sydenham Chorea (from ARF)
What is Sydenham chorea classic ppt?
Chorea
+ Emotional Lability
+ Hypotonia
When does sydenham chorea usually resolve
6-9 month
if impairing f’n= VPA, carbmazepine
Rheumatic fever. What causes long-lasting problems
Carditis
- arthritis and chorea resolve without sequelae
- longterm= heart
Recurrent fever. Evanescent rash. Lymphadenopathy. MSK normal. Dx?
Systemic JIA
How do you define systemic JIA
arthritis w/ or preceded by fever min. 2 wk
(note quotidian= daily fever x 3 d)
\+ min 1 OF > evanescent red rash > node > HSM > serositis
T or F: systemic JIA is typically easy to control.
False.
Esp poor prognosis if
- polyarticular
- fever x 3 mo
- BW high x 6 mo.
Features of KD include all except:
- ++ WBC
- ANA (-)
- Plt low
- urine 10-15
Plt low
BW:
- WBC ++
- Anemia
- ++ plt after wk 1
- ++ CRP, ESR
- low albumin
- low Na
- sterile pyuria
12 y.o. 2 wk fever, arthralgia, myalgia. Pale. Blood and protein urine. Plt normal.
- ARF
- Wegner’s granulomatosis
- SLE
- Juvenile rheum arthritis
SLE
What is a SLE Mnemonic?
Need 4 of 11 "SOAP BRAIN MD" S= serositis (pleuritis, pericarditis) O= oral or nasal ulcer (painless) A= arthritis (oligo or poly) P= photosensitivity, pul fibrosis B= blood (all line low) R= renal, raynaud A= ANA (+) I= immune (anti-Sm, anti-dsDNA, antiphospholipid) N= neuro (Sz, psychosis) M=malar rash D= discoid rash
3 tests to confirm dx of Juvenile Dermatomyositis
Muscle enzyme (CK, AST, ALT, LDH)
EMG change (short small motor unit, (+) sharp wave)
Muscle Bx (necrosis, inflammation)
Mainstay treatment of juvenile dermatomyositis?
Steroids
Next:
- MTX
- IVIG
- Plaquenil
- Cyclosporine
- MMF
- Rituximab
What are 5 criteria for KD
CRASH + Burn
> Fever x 5 d
Conjunctivitis (b/l, non purulent)
Rash (polymorph)
Adenopathy (cervical, > 1.5 cm, unilateral)
Strawberry tongue (cracking of lips, mucosa injection)
Hands (extreme red, swelling and then desquamation)
2 findings of P/E for ankylosing spondylitis? Two tests to help with dx.
- Sacroiliac tenderness (FABER)
- Schober test (< 5 cm increase with forward flexion)
Tests to Dx:
- HLA B27 (+)
- MRI (bone marrow edema next to joint)
Rash over back of hands, muscle pain, teased for weakness.
- DMD
- JDM
- SLE
Juvenile Dermatomyositis
Boys with Fever, rash, b/l conjunctivitis. Dry cracked lips. Most likely lab finding?
- low albumin
- neutropenia
- EBV (+)
- (+) ASOT
Low albumin
Child w/ pulmonary findings, eosinophilia, slightly high Ca2+.
- Miliary TB
- sarcoidosis
- cryptococcus
- blastomycosis
Sarcoidosis
What are the dx criteria of JIA
age < 16
+ arthritis (swelling or effusion or 2 of: ROM, warmth, tender on pain or motion)
+ duration min. 6 wk
Oligo= max 4 Poly = min. 5
Which complication are you at risk for if JIA?
Uveitis
** kids with oligo (max 4 joint), ANA (+), and onset earlier than 5 at risk for chronic uveitis
Three ways maternal SLE affect neonate:
Due to passive transfer of maternal immunoglobulin IgG autoantibody to fetus.
- Heart block (from prolonged PR to complete block; permanent)
- Rash (annular or macular; esp face; last 4mo.)
- Low Plt
- Hepatitis
Sunburst Pic on XR. Dx?
Osteosarcoma
- teen (versus Ewing <10 or > 20)
- long bone
- local pain, limp
- chemo
Periosteal reaction on XR. Another word for that and likely CA dx?
Onion skinning
Ewing Sarcoma
What puts kid with JIA at higher risk of uveitis:
- being 9 y.o.
- course severity
- M
- ANA (+)
ANA (+)
List complications of uveitis
- Synechiae (irregular pupil)
- Glaucoma
- Cataract
- Vision Loss
