Rheum Flashcards
Familial Mediterranean Fever
Clinical Dx: ≥ 3 recurrent episodes lasting hours–96 hours, which include ≥ 2 of the following: - fever - abd pain - chest pain - arthritis (knees, ankles, wrists) \+ FHx Can also be rash (erysipelas like), testicular pain, PID like sx
Complication: Amyloidosis - accumulation of amyloid A during recurrent paroxysmal attacks -> kidney
Tx: Colchicine
JIA poor prognostic indicator
RF+
Psoriatic juvenile arthritis
small joints are most affected
nail pitting, onycholysis
dactylitis
periostitis (new bone formation) on radiograph
arthritis can present before skin changes (delayed up to 5-10 years)
Watch out for acute anterior uveitis
Reactive Arthritis
“Can’t see, can’t pee, can’t climb a tree”
Arthritis
Iritis/Conjunctivitis
Urethritis
+ Enthesitis
+ Dactylitis
+ Mucocutaneous manifestations - oral ulcers, genital ulcers, balanitis, papular skin lesions that look like psoriasiform-like
Usually after GI illness (Shigella, Salmonella, viridans strep, mycoplasma, yersenia) or Chlamydia/G
Seronegative: - ANA and RF
Workup: CBC, ESR, RF; consider joint aspiration
Tx: First line is NSAIDS.
Mixed Connective Tissue Disease
Overlap syndrome with features of dermatomyositis, JIA, lupus, scleroderma.
Will often present with Raynaud’s
If ANA pos, you should test for specific autoAb
But it has it’s own specific autoantibody = Anti U1 RNP
Antibodies and diseases: dsDNA SSA (Ro) Histone anti-CCP
dsDNA - lupus (also anti-smith)
SSA (Ro) - neonatal lupus
Histone - drug induced lupus
anti-CCP - Rheumatoid Arthritis
Lab findings in dermatomyositis
Elevated CK!!!
HSP treatment
Hydration and pain meds
Most cases occur after a viral infection and resolve with conservative management.
If really severe, corticosteroids.
But not like big guns like IV methylpred.
AVN
flattening and fragmentation of the L femoral head and epiphysis
RF: chronic steroid use, connective tissue d/o, chronic renal failure.
limp, thigh and/or knee pain, and hip stiffness; the patient is typically hesitant to bear weight on the involved leg when standing and fails to maintain a level pelvis—a positive Trendelenburg sign.
On physical examination, the lower extremity is held in a slightly externally rotated position; internal rotation typically produces pain.
Enthesitis-related Arthropathy
Enthesitis and/or
Arthritis
2 of the following:
- sacroiliac joint tenderness
- HLAB27
- arthritis in male > 6 yrs
- uveitis
- A 1st-degree relative with ankylosing spondylitis, ERA, sacroiliitis with inflammatory bowel disease, or reactive arthritis
HLAB27 is positive in
Ankylosing Spondylitis
JIA
Psoriatic arthritis
Enthesitis-related arthritis
NOT reactive arthritis.
Behcet’s Disease
Recurrent oral apthous ulcerations
several systemic manifestations
- genital ulcers
- uveitis
- variety of cutaneous lesions (like erythema nodosum), arthritis
- gastrointestinal ulcerations
- vasculitis of any size artery or vein—affecting the central nervous system (CNS), kidney, and/or heart
Positive Pathergy test - developing a small, red papule, pustule, or ulceration at the site of sterile needle insertion 1–2 days after the procedure
Systemic JIA labs
Increased ESR
Leukocytosis
Increased ferritin
Decreased Hgb
NORMAL ANA*** (vs. poly, oligo, psoriatic have + titers)
Negative RF
Sx: daily fever for at least 2 weeks, rash, lymphadenopathy, hepatosplenomegaly, serositis, and arthritis in 1 or more joints
Table to compare:
file:///Users/vicky/Downloads/C13.pdf
Joint Hypermobility Syndrome
Joint hypermobility syndrome is a hereditary connective tissue disorder characterized by generalized joint hypermobility and chronic pain. It is clinically indistinguishable from the hypermobility subtype of Ehlers-Danlos syndrome and may in fact be the same disorder.
Sx
- musculoskeletal abnormalities (ligament and tendon injuries, joint dislocations, problems with proprioception) - dermatologic features (hyperextensible skin, easy bruising, and stretch marks)
- gastrointestinal dysmotility
- chronic pain, chronic fatigue
- autonomic dysfunction
- and/or anxiety and depression.
Tx:
- physical therapy***
- multimodal pain control, and treatment of any associated anxiety or depression.
Neonatal Lupus
Rashes on the trunk/scalp
- discoid lesions, periorbital erythema, annular lesions, scaly atrophic patches, and/or telangiectasia.
Bradycardia
Heart block
+anti-Ro and La Ab tranferred from placenta
Other symptoms resolve, but heart block os permanent .
