Rheumatology Flashcards
What are the features of Growing Pains (6)
- age 3 - 10
- Bilateral Leg pain
- Occurs at night
- Normal physical exam
- Normal Lab tests
- Normal Imaging
What are the 2 different Pain Amplification Syndromes?
1) Generalized
- Fibromyalgia
- specific trigger points
- associated psychiatric symptoms
2) Localized
- Reflex sympathetic dystrophy
- Complex regional pain syndrome
Autonomic changes
- acute: swelling
- chronic: atrophy/cold extremities
Girls> boys
Txt: Interdisciplinary assessment and therapy
Most common cause of limp according to age range?
Most common cause is often in the Hip
2 - 6 yrs: Transient Synovitis
4 - 10 yrs: Legg-Calve-Perthe’s
10 - 14 yr: Slipped Capital Femoral Epiphysis
What are the cardinal features of an acute joint infection and what is its management?
Cardinal features:
- fever
- severe pain/tenderness
- swelling
- redness
Surgical emergency!
- joint aspirate and culture
- irrigate joint
- Antibiotics
What is the DDX for Monoarthritis
Trauma/Mechanical (overuse)
Orthopedic
- AVN
- SCFE
Infection:
- acute (septic arthritis, osteomyelitis)
- chronic ( TB and Lyme)
- reactive arthritis
Inflammatory:
- JIA
- non-bacterial osteomyelitis
- FMF
Tumor (localized)
Hemarthrosis
DDX for Polyarthritis
Inflammatory:
- JIA
- SLE, other connective tissue diseases
- Vasculitis - HSP
Infection:
- Acute: viral (parvo), bacterial (gonococcal)
- Post-infectious (reactive/RF)
Malignancy (systemic)
Mechanical
-Hypermobility, sketetal dysplasia
Pain syndrome
-fibromyalgia
What is the Major and Minor Criteria of ARF
Need 2/5 Major Criteria or 1/5 Major and 2/4 Minor Criteria pluse evidence of of GAS infection - temporal relationship
Except chorea - don’t need to meet whole criteria
Major: SPACE S ubcutaneous nodules P ancarditis A rthritis, migratory, poly C horea E rythema
Minor: FLAP F ever L ab abnormalities (increased ESR, CRP) A rthralgia P rolonged PR interval
Treatment for ARF
- penicillin for 10 days
- ASA for arthritis
- ASA/steriods for carditis
- Prophylactic penicillin for at least 5 yrs or until 21 years, lifelong for carditis
What is the definition of JIA
Arthritis before 16 yrs
Duration x 6 weeks
Exclude other causes
What level of ANA should you consider you do specific antibodies
Specific antibodies (Anti-double stranded DNA) only if ANA + at higher levels (>1:160) and disease other than JIA suspected
Low titres of nonspecific ANA in JIA (eg ANA 1:160
What is Rheumatoid Factor and how is it used in JIA
IGM autoantibody reacts to Fc portion of IgG antibodies
- 85% in adults with RA
- only 5 - 10% of children with JIA (poly)
- no diagnostic utility but classification/prognostic factor (suggest worse prognosis)
- positive in other rheum disease: SLE
What is HLA B27
HLA B27 is a class I gene only present in 10% of ppl
HLA class I and II associated with Rheumatic disorders
- 90% of Caucasians with spondylarthritis
- 70-90% of enthesitis related JIA
What are the components of Oligoarthritis
- Girls > Boys
- max 4 joints in the first 6 months
- Persistent - only ever 4 joints after the first 6 monts
- Extended - more than 4 joints after the first 6 months
- as
What are some of the long term effects from untreated Oligoarthritis
- malalignment of joints
- muscle atrophy
- growth disturbances
- delay of motor development
- motor and intellectual development are closely related
Which type of JIA is uveitis most commonly associated with?
- most common in oligoarticular JIA (10 - 20%)
- associated with positive ANA
- usually ASYMPTOMATIC except with enthesitis related arthritis
- does not parallel arthritic course
What are the complications of uveitis?
- synechaie - irregular pupil
- glaucoma
- cataract
- visual loss
What are X-ray changes seen in JIA
- acceleration of growth (knee)
- acceleration of maturation (wrist)
- growth inhibition (TMJ)
- osteoporosis
- erosions
- loss of cartilage
MRI changes
- synovitis
- bone marrow edema
- cartilage
- erosions
Features of JIA?
- Girls - 75%
- typically: symmetric joint involvement, small and large joints
- slow progression (except for RF/anti CCP pos)
- Uveitis in 5-10%
Labs:
ANA + 30 -40%
RF/anti CCP- if present = more aggressive course
Features of Enthesitis associated Arthritis
- Boys = 75%
- typically: asymmetric joint involvement esp lower extr
Enthesitis Tarsitis Daktylitis Sakrolitis Uveitis (acute) - red and painful
Labs:
HLA- B27 70-90%
ANA and RF negative
What is the criteria for Psoriatic Arthritis
Arthritis AND Psoriasis:
-search for ‘hidden’ psoriasis (scalp, umbilicus)
OR
Arthritis plus 2 of the following:
- nail pits
- dactylitis
- family hx of psoriasis
What is the criteria for Systemic JIA
- > 2 weeks spiking fever
- arthritis
+ at least 1 of:
- rash
- generalized lymphadenopathy
- HSM
- serositis
Course sJIA
- monocyclic
- polycyclic
- persistent
What are the 2 categories of HLH
Primary HLH (familial)
Secondary HLH (acquired)
- infection associated
- malignancy associated
- rheumatic disease associated: MAS
What is the therapy for JIA?
Traditionally step up
- NSAID and joint infection for oligo JIA
- NSAID then MTX and/or SSZ for poly JIA then biologics
- NSAID then systemic glucocorticoids and/or MTX for sJIA then biologics
- Anti TNF does not work well in SJIA
- sJIA often now treated initially with anti IL-1 or anti IL-6
JIA therapy side effects
- monitor CBC, transaminases and creatinine
- increased risk for infections
- no live vaccines with MTX and biologics
- monitor for TB with biologics
- Varicella can be severe
- no Sulfa Abx with MTX
What are the features of FMF?
- fever, brief episodes (1 - 3 days)
- painful polyserositis: peritonitis, pleuritis, arthritis (70%)
dx is important because of untreated Amyloidosis in 60% > 40 yrs
-also can get renal failure
What is the txt for FMF
prophylaxis with colchicine (needs to be constant prophylaxis)
- up to 96% Efficiency
- not helpful in acute phase
What are the features of PFAPA
Periodic Fever, Aphtous stomatitis, pharingitis, adenitis (PFAPA)
-regularly recurring fevers with an early age of onset (< 5 yrs of age)
- symptoms in the absence of URTI with at least one of the following:
- aphtous stomatitis
- cervical lymphadenopathy
- pharyngitis
- exclusion of cyclic neutropenia
- completely asymptomatic btw episodes
- normal growth and development
What are the autoantibodies for SLE
- ANA (97%)
- dsDNA (70%)
- Sm (32%)
- RoSSA/LaSSb (20 - 30%)
- Antiphospholipid - Ab (35%)
What is the most Sensitive test for SLE
What is the most specific test for SLE
Most sensitive test: ANA
Most specific test: anti double stranded DNA antibodies
What are the features of Dermatomyositis
- symmetric proximal muscle weakness
- cutaneous changes - heliotrope or Gottron’s papules
- elevated muscle enzymes
- CK, AST, LDH, aldolase
- Abnormal EMG
- muscle biopsy
- Difficulty swallowing
- Resp failure with severe weakness
- Lung involvement
- Arthritis
- Joint contractures
- cutaneous and mucosal ulcerations
What are the features of HSP (7)
- palpable purpura
- arthritis
- abdominal pain, bleed, intussusception
- IgA nephropathy, renal failure < 5%
- edema
- orchitis
- leukocytoclastic vasculitis IgA deposition
What is the treatment for HSP
- supportive
- analgesia/NSAIDS
- systemic steroids: treat early with steroids in (severe abdo pain, severe renal disease)
-recurrences common in approx 30% usually w/n the first 1 - 2 months
What is the criteria for Classical Kawasaki Syndrome
Fever >/ 5 days and at lest 4 of the following:
- Polymorphous rash
- conjunctivitis
- cervical lymphadenopathy > 1.5 cm
- Oromucosal changes (lips; strawberry tongue, pharyngitis (nonexudative)
- extremity changes (palmar pantarerythema (edema, scaling)
What are other helpful clues for diagnosis of KD not part of the criteria
- peeling in the perineal area (acute phase)
- aseptic meningitis
- anterior uveitis in up to 80%
- hydrops of the gallbladder
- sterile pyuria
- lung involvement
- diarrhea in approx 25%
- Gamma GT elevation
What are findings supporting a diagnosis OTHER THAN KD
- exudative conjunctivitis
- generalized lymph node swelling
- intraoral ulceration or vesicles
- exudative pharyngitis
- normal ESR
- Leucocytes < 10, 000/uL
What is the treatment for KD
-IVIG (2g/kg)
-ASA (80-100 mg/kg)
10- 20% don’t respond => IVIG (2 g/kg)
Steroids?
-low dose ASA (3 - 5 mg/kg/day) until follow up 2D echo is normal and platelets are normal
-be careful of hemolysis
what are the ANCA-related vasculitis?
Microscopic polyangitis
Granulomatosis with polyangitis (wegner)
Eosinophillic granulotosis with polyangitis (churg strauus)
Criteria for Takayasu artheritis
Angiographic abN of aorta or main branch(PA etc) AND 1of 5 1) peripheral deficit or claudication 2) 4limb - BP > 10mmHg in any limb 3) bruits 4) HTN (>95%) 5) Acute phase reactant (ESR/CRP)
neonatal lupus
name 4 complications
1) Cardiac - complete heart block
2) Skin - annular erythema, erythematous papulomacular rash (usually head), telaniectasia (later)
3) Heme - thrombocytopenia
4) GI - cholestatic hepatitis
5) Neuro - macrocephaly, hydrocephalus
antiphospholipid antibodies - list
lupus anticoagulant
anticardiolipin
anti b2 - glycoprotein I
KD - when and how much does IVIG change risk for Coronary aneurysms?
IVIG given within first 1- days
25% risk to 4%
Criteria for HSP
Purpura/purpura + 1 of 4
1) abdominal colicky pain
2) arthritis/arthralgia
3) renal (proteinuria, hematuria, etc)
4) biopsy - skin or renal
JDM exam and investigations (4)
1) proximal muscle weakness(+/-nasal voice, dysphagia)
2) Gottron papules (dorsal knuckles)
3) heliotrope rash (eyelids)
4) elevated muscle enzymes - CK, ALT, LDH, Aldolase
5) EMG - denervation & myopathy
6) biopsy - necrosis & inflammation
7) ANA+ (70%)
JDM complications
muscle weakness - joint contractures
soft tissue calcinfication/calcinosis
lipoatrophy +/- hyperinsulin, TG, acanthosis nigricans, T2DM
Systemic sclerosis 5 complications
1) Raynauds
2) sclerodactyly (thickening of skin around fingers)
3) calcium deposits (skin)
4) polyarthritis
5) Resp - PulHTN, ILD
6) GI - dysmotility, bacterial overgrowth, malabsorption
7) GU - HTN, proteinuria
Systemic sclerosis investigations
ANA, anti Scl70, anticentromere (rare RF) BP, U/A ECHO, ECG - PulHTN CT chest, PFT - ILD UGIS - dysmotility
Raynaud treatment
1) avoid triggers - cold, caffeine, smoking, stress
2) nifiedipine - peripheral vasodilator
3) if severe - IV prostaglandin
4) topical nitroglycerin if ulcers
Sjogren syndrome
2 main manifestations
keratoconjuntivitis sicca (dry eyes) xerostomia (dry mouth)
