Rheumatology Flashcards
What are the criteria for SLE?
4/11
- Malar rash
- Discoid rash
- Photosensitivity
- seizures or psychosis
- oral ulcers
- pleuritis or pericarditis
- positive anti-ds, anti-smith, antiphospholipid
- positive ANA
- Renal
- hematologic disorder
- nonerosive arthritis
What endocrine problem is associated with SLE?
Hypo/hyperTH, Addisonian crisis
What neurologic problems are associated with SLE?
HA, memory loss, sz, psychosis, stroke, pseudotumor cerebri, cerebral venous thrombosis, aseptic meningitis, coma, chorea, global cognitive deficits, mood disorders, transverse myelitis, peripheral neuropthay
What pulmonary problem is associated with SLE?
Bleeding, HTN, interstitial lung disease
Morbidity for SLE occurs most often in which 2 disease systems?
Renal and CNS
What drugs typically cause drug-induced lupus?
diphenylhydantoin, ehtosuximide, carbamazepine
What are the most common lab findings in 1) drug induced lupus 2) neonatal lupus
1) antihistone and anti-DNA antibodies
2) Ro and La antibodies
What systematic problem is most common in
1) SLE
2) Neonatal lupus
3) Drug-induced
1) renal and CNS
2) heart block, rash, hepatitis
3) hematologic, pleural/pericardial disease
Pattern: rash in lower trunk and legs, appears in crops, eruptions last 3 weeks, has colicky abdominal pain, nausea, vomiting, large joint arthritis
HSP
How often is kidney involvement seen in HSP patients?
20-40% hematuria, proteinuria, oliguria, ARF
So measure microalbuminemia
What GI problems in HSP
intussusception, bowel infarction, bowel perforation, hydrops of the gallbladder, pancreatitis, massive GI bleeding
What labs must you do if you suspect HSP?
CBC, PT/PTT, CMP, ESR/CRP, urine for protein, stool for occult blood
What cardiac complications are associated with Kawasaki?
Tachycardia, myocarditis, pericarditis, coronary artery aneurysms
Which constellation of lab findings are seen with Kawasaki disease?
High ESR, CRP, WBC, left shift, platelets, transaminases, TG/LDL. low HDL, sterile pyuria
What is the treatment for Kawasaki?
IVIG and mega dose aspirin for 48hr, then go to 3/-5mg/kg/day. Can discontinue with all acute phase reactants return to normal
If cANA is high, which vasculites is it most likely to be?
Wegener’s; Churg-Strauss
If pANCA is seen, which vaculities can it be?
microscopic polyarteritis, Churg-Strauss, UC, sclerosing cholangitis, Crohn’s disease
Which labs indicate active disease in SLE?
Low complement and anti-ds DNA
Which autoantibody is sensitive and which is specific for SLE?
ANA-specific
Anti-dsDNA specific
Anti-ribosomal P is a test for what?
psychosis associated with autoimmune
How do you treat HSP?
supportive, Tylenol/NSAIDs for joint pain, steroids for severe abdominal pain, steroids and immunosuppression for renal involvement
Which of the three JIA typically has highest morbidity for uveitis?
oligoarticular/pauciarticular JIA
Which JIA has highest percentage of patient with ANA+
Oligo/pauci
What is the morbidity of most concern in polyarticular JIA?
unremitting arthritis
What is the most concerning morbidity with systemic JIA?
The chronic systemic involvement. Uveitis is rare.
What complications can be seen in systemic JIA?
cardiac: pericarditis, myocarditis, endocarditis; hepatosplenomegaly, lymphadenopathy, pleuropulmonary disease, CNS disease, growth failure, osteoporosis
Pattern: unremitting fever, bruising purpura, mucosal bleeding, hepatomegaly, lymphadenopathy, liver dysfunction, CNS involvement, multiple organ failure.
Labs - pancytopenia, falling ESR, liver derangement, coag abnormalities, increased ferritin, elevated TG
macrophage activating syndrome
Pattern: male with hip, knee, ankle pain, HLA-B27+
enthesitis related arthritis
Pattern: chronic inflammatory arthritis of axial and peripheral skeleton, frequent enthesitis, seronegative, HLA-B27, b/l sacroiliac joint inflammation
Juvenile ankylosing spondylitis
What is reactive arthritis?
Response ton an infectious agent in another part of the body
Postinfectious arthritis is what?
immune complexes containing non-viable components of an initiating infectious illness
What degree of extension or flexioun would classify as hypermobility?
> 10 degrees
Criteria and associated symptoms for fibromyalgia
Generalized MSK at 3 or more sites for more than 3 months, 5+ tender points, difficulty sleeping
What is the weakness pattern for dermatomyositis?
Proximal muscle weakness
Pattern: 5-14yr old female with increased fatigue, had a cold 6 weeks ago, having trouble getting on the school bus. Parents noticed eyes loo puffy and red over the eyelids.
Dermatomyositis
Describe the rash of JDM
discoloration of the eyelids with periorbital edema; Gottron’s papules, Erythematous, scaly rash over dorsal aspects of metacarpal and proximal interphalageal joints
What enzymes are elevated in JDM?
CK, AST, ALT, LDH, Aldolase
What type of disease is JDM?
Vasculitis of skin and muscle
Pattern: rash, dysphagia/dysphonia, dyspnea, GI (micro perforation), edema, joint contractures, cutaneous ulceration, calcinosis
JDM
What is the calcinosis in JDM?
healing that happens with calcium deposition, can become very skeletal
Treatment plan for JDM
Oral pred 2mg/kg daily until serum CK normalized, slowly taper over 24 months, hydroxychloroquine (Plaquenil), MTX, IVIG, TNF inhibitors
What tissues can scleroderma affect?
Skin, bone growth, joint
Pattern: calcinosis, Raynaud, esophageal dysmotility, sclerodactylyl, telangiectasia
limited cutaneous systemic scleroderma
Pattern: sclerodactylyl and tight face
system diffuse cutaneous scleroderma
What test for scleroderma?
ANA, Scl-70, anti-centromere Ab
Treatment for complications of scleroderma
Topical Vit D, MTX, calcium channel blocker (Raynaud’s), ACE (renal)
What organ systems do you have to worry about with scleroderma?
skin, lungs, heart, renal
What gene is associated with familial sarcoidosis?
NOD2/CARD15 mutation
Pattern: polyarthritis, dermatitis, uveitis
sarcoidosis
What labs are useful for sarcoidosis?
Calcium and VitD; acute phase reactants, mild anemia, ACE
Trio of drugs for sarcoidosis
steroids, TNF inhibitors, methotrexate
Gene mutation in Ehlers Danlos
COL gene
What organ systems are affected in Ehlers Danlos?
osteopenia and cardiac
Gene mutation in Margan
AD FBN1 (fibrillin2)
Organ systems involved in Marfans
heart valves, aorta, lungs, dural sac of spine, skeleton, and eyes
Pattern: mutation of pyrin gene, amyloidosis that causes renal disease
familial Mediterranean fever
Pattern: period fever, apthathous ulcer, occurring every 28das
PFAPA
Which microorganisms can cause postinfections arthritis
parvovirus B19, influenze, hepatitis B, rubella, Epstein-barr
Which microorganisms can cause transient arthritis?
enterovirus, hepatitis B, rubella and mumps
Cause of chest pain in patient with JIA
pericarditis