Poly JIA Flashcards
Important HLA Alleles
Class I alleles HLA A2 and class II alleles DRB108 and DPB102 confer susceptibility in RF negative poly
HLA DRB1*11:03/11:04
Confers susceptibility to oligo and younger onset poly JIA
HLA DRB1*08:01
Confers susceptibility in both younger and older onset RF negative poly
Important non HLA genes
PTPN22–> encodes tyrosine kinase which negatively regulates T cells
What predicts progression to polyarthritis in oligo?
Symmetric involvement
Wrist and ankle involvement
Elevated ESR
Differential Dx of RF negative poly
SLE ERA Psoriatic JIA Scleroderma Sarcoid Blau Sjorgens MCTD Infection (Septic, Neisseria, ARF, Poncet) Malignancy Nutritional def IBD Scurvy Relapsing polychondritis Sickle cell
Which autoantibodies could be positive?
Anti-CCP are uncommon
ANA present in one half
also have seen Anti Carp and anti mutated citrullinated vimentin (anti MCV
Unfavorable prognostic factors
Hip and cervical spine involvement Joint space narrowing on radiograph with or without body erosions Anti CCP antibodies Subcutaneous nodules High inflammatory markers High number of affected joints Poor global assessments
Felty Syndrome
Persistent neutropenia, Splenomegaly, and RA and is associated with frequent infections
Most common cardiac lesion in RF positive poly
Aortic Insufficiency
Pulmonary manifestations in RF positive poly
Lymphoid interstitial pneumonitis
Bronchiolitis obliterans organizing pneumonia (BOOP)
Rheumatoid nodules
- Innermost zone is a core of necrotic tissue containing cellular material surrounded by fibrinoid
- Next layer is a palisade of radially arranged elongated mononuclear cells
- Outermost is CT later which is a vascular region containing a lymphocytic infiltrate
Joint Pattern in RF negative Poly JIA
Usually insidious with progressive accumulation of joints
Morning stiffness
Knees, wrists, ankles most common
Can also see small joints of hands and feet ( not DIPS)
TMJ involvement
C spine with long standing diseases
RF negative tends to be fewer joints and more asymmetric than RF positive
What is Dry synovitis?
Joint contractures, limited ROM, stiffness, lab indicative of inflammation, polyarthropathy, minimal signs of joint effusion or synovial hypertrophy
Joint pattern in RF positive poly JIA
Upper and lower extremity and small joints
Symmetrical arthritis affective MCP, PIP, wrists, MTPs, PIP of feet
