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170 Cards in this Set
- Front
- Back
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SLE is more common in ___
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females
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what is the mean age of onset for SLE?
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12-13 years
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what races have highest incidence of SLE?
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hispanic, African americans, asian, native american
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SLE is characterized by ___ circulating immune complexes and T lymphocytes
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autoantibiodies
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The 10 year survival with SLE is __ % and the 30 year is __
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92%; 76%
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What are the common causes of mortality from SLE?
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infection; CNS disease; renal failure; cardiac/pulmonary; thromboembolic disease
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what are the 4 skin findings in SLE?
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1. Malar Rash
2. Discoid Rash 3. photosensitivity 4. oral ulcers |
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what are the 2 immunologic markers that are part of the SLE criteria?
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1. ANA
2. dsDNA, anti-Smith, antiphospholipid antibodies |
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what are the 5 organ systems under the SLE criteria?
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1. CNS
2. Serositis 3. Kidney 4. Arthritis 5. Hematologic |
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what are the 2 CNS issues that can occur in SLE?
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1. Seizure
2. Psychosis |
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describe discoid lupus?
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well-circumscribed, red-purplish, elevated plaques
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the SLE criteria has joint involvement without ___ __
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errosive antibodies
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The malar rash on the face of SLE spares the __ __
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nasolabial folds
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what disorders have a rash that spares the nasolabial folds?
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SLE and eczema
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what oral findings occur in SLE?
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oral ulcers
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what are the major classes of treatment for SLE?
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1. NSAIDs
2. Hydroxychloroquine (plaquenil) 3. steroids 4. Immunosuppresants 5. intravenous infusion 6. sunscreen |
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what are the side effects of plaquenil?
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ototoxic and ocular side effects (needs a visual fields test yearly)
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what are the immunosuppresants used for SLE?
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1. cyclophosphamide
2. azathioprine 3. cellcept |
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what are the drugs given via IV infusion for SLE?
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cyclophosphamide and rituximab
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what are the 3 types of inflammatory disorders that cause musculoskeletal symptoms in children?
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1. JIA
2. Connective tissue disorders 3. Vasculitis |
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what are the inflammatory connective tissue disorders that cause musculoskeletal symtpoms in children?
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SLE, Juvenile dermatomyositis, IBD, linear scleroderma, systemic sclerosis
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what are the 2 vasculitis disorders?
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1. kawasakis
2. Henoch-Schonlein purpura |
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what are the orthopedic causes of musculoskeletal symptoms in kids?
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1. benign join hypertrophy
2. Perthes disease 3. SCFE, anterior patella syndrome 4. back pain |
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what are the infectious causes of musculoskeletal pain in children?
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1. post infectious
2. septic joint, osteo |
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Marfans and ehlers danslo are __ __ disease that can cause musculoskeletal pain in children
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connective tissue disorders
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what are the neoplastic causes of musculoskeletal pain in children?
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leukemia, neuroblastoma, metastatic disease
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list the hematologic causes of musculoskeletal pain in children
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1. SCD
2. hemophilia 3. hemoglobinopathy |
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what are the metabolic disorders that have been known to cause musculoskeletal pain in children?
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1. Fabry's disease
2. Lesch-Nyhan syndrome |
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what are the diagnostic tests to do for an initial workup with joint pain and rash?
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CBC with diff; ESR; CRP; lyme titer and LDH
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what is PCT?
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procalcitonin; an acute phase reactant that goes up within 4-6 hours of infection
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what is haptoglobin?
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acute phase reactant that goes up with other markers
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A negative ___ makes diagnosis of SLE or mixed connective tissue disorder highly unlikely.
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ANA
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What is the problem with ANA?
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the prevalence of positive ANA in healthy children is 13-18%. Get a lot of false positives. Not specific for rheum disorders. Also goes up in infection, malignancy, and drug reactions
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ANA is highly ___ for SLE, but not ___ and PPV is ___
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sensitive; specific; low
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there is a higher rate of ___ diagnoses with sogren disease?
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lymphoma
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what are the 2 autoantibodies that if elevated are suggestive of sjogrens?
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Anti-dsDNA and Anti-Smith
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what are the autoantibodies that if raised are virtually diagnostic to SLE?
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anti-dsDNA and anti-Smith
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anticyclec citrullinated peptide antibody is directed against __ __ formed as a result of inflamed __ tissue
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citrulline residue; synovial
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what antibody may appear veofre the disease presentation of SLE?
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anticyclic citrullinated peptide antibody
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what test does NAPNAP recommend for any child presenting with arthritis?
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Anti CCP
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Anti CCP is a poor test since only __ __ children have circulating antibodies against CCP
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RF positive
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what is included in a comprehensive antiphospholipid panel?
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1. Anti-cardiolipin IgG/IgM/IgA
2. Anti-beta 2 glycoprotein I IgG/IgM/IgA 3. Lupus anticoagulant |
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what are the associated risks with antiphospholipid disorders?
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1. arterial/venous thrombosis
2. miscarraige 3. thrombocytopenia/hemolytic anemia 4. migraines 5. seizures 6. libman sacks endocarditis |
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how should a person be tested for antiphospholipid disorders?
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repeated twice 12 weeks apart
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aPL can be seen transiently following __ __
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viral illness
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a positive APL should be confirmed __
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twice
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ToF: primary aPL disease is common in children
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FALSE
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what are the pros to ordering a comprehensive aPL?
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1. appointment for subspecialist may be delayed
2. can be helpful in improving appointment time 3. early identification means earlier treatment |
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what are the cons for comprehensive aPL panels?
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1. test should only be ordered when there is a high clinical suscpicion
2. expensive 3. may add to medical cost without adding value 4. may not be able to interpret easily 5. lab reliability |
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what are UA signs of lupus nephritis?
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protein and + heme
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if a child presents with rash, palatal ulcer, arthritis, anemia and proteinuria, what are the differentials?
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SLE, crohns, JIA, cancer, and lyme
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Tof: if ANA is negative does that mean lupus is not present?
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no
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what does Ro + mean?
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sjogrens
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what does a A RASH POINTS MD mean?
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A: arthritis
R:renal disease A: ANA + S: serositis H:hematologic disorder P: photosensitivity O: oral ulcers I: immunologic disorder N: neurologic symptoms M: Malar rash D: discoid rahs |
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what are the 3 general points for SLE patients?
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1. flares are mimetic: initial presenation in first few months often dictates clinical course
2. C3, C4 and dsDNA measure disease activity 3. advances in tx improve survial yet increase risk of INFECTION and drug related toxicity |
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what is the basic treatment for SLE?
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1. Plaquenil (antimalarial)
2. NSAIDs 3. Calcium & Vit D 4. Sunscreen |
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what are the examples of steroid toxicity?
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1. immunosuppression
2. HTN 3. hyperglycemia 4. adrenal suppression 5. cataracts 6. dyslipoproteinemia 7. tremors 8. myopathies 9. avascular necrosis of bone 10. osteoporosis/growth retardation |
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ToF: avascular necrosis of bone occurs after several steroid treatments
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false, it can occur even after one treatment
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what are the skin PE findings with SLE
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1. hair loss
2. raynaud's 3. rashes (malar, vasculities, petechae, purpura) 4. abnormal nailbed capillaries |
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what are the cardiovascular findings of SLE?
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1. endocarditis
2. pericardial effusions 3. myocardial infarction 4. hyperlipidemia 5. HTN (renal issues, med related) |
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list the HEENT PE findings with SLE?
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1. palatal ulcers
2. retinal vasculitis |
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what are the issues that occur in the lungs with SLE patients?
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1.restrictive lung dz
2. pulmonary hemorrhage 3. pneumonia 4. pleuritis |
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what are the joint pain physical exam findings with SLE?
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1. arthritis
2. myositis 3. avascular necrosis (2ndary SLE vs steroids) 4. Septic arthritis |
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what are the red flags found on PE with SLE pts?
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fever, refusal to bear weight
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what are the neurologic PE findings in SLE patients?
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1. lupus cerebritis
2. cognitive dysfunction 3. migraines 4. depression 5. psychosis |
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for patients with SLE on longstanding naprosyn, what should they also be prescribed?
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GI prophylaxis: PPIs
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describe lupus arthritis.
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1. non-erosive
2. often in small joints of hands but can be anywhere 3. jaccoud's arthropathy (deforming dur to particular fibrosis, ligament laxity and joint subluxation |
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those with class I minimal mesagnial lupus nephritis will have __ __
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mild proteinuria
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Class 2 lupus nephritis is called __ __ and associated with __
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mesagnial proliferative; asymptomatic hematuria/proteinuria
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focal segmental glomerulonephritis (active or chronic) is associated with what?
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active SLE, and moderate proteinuria
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what class of lupus neprhritis is associated with nephrotic syndrome, renal insufficiency and HTN?
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class IV: diffuse proliferative glomerulonephritis
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what symptom occurs in membranous (class V) lupus nephritis?
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nephrotic syndrome
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advanced glomerulosclerosis is characteristic of what symptoms?
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renal insufficiency or ESRD
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what are the etiologies of a pt with SLE complaining of chest pain?
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cardio (pericard; tamponade; MI); pulmonary (pleuritis, infection, alveolar hemorr, pulm embolism); costochondritis
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what are the red flags for a person with SLE and chest pain?
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1. abnormal vital signs
2. muffled heart sounds 3. won't lie flat 4. hemoptysis 5. hx of postiive aPL, antibodies or nephrotic syndrome |
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what is the work up for pts with SLE and chest pain?
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ekg; CXR; ct scan, VQ scan; CBC; chem, LFTs, urine protein, C3, C4, dsDNA, Cardiac enzyme
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what is the tx for pts with SLE and serositis?
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NSAIDs/steroids
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a pt with alveolar hmeorrhage and SLE will need ___
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plasmaphoresis
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what are the etiologies for SLE and headache?
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lupus cerebritis/vasculitis; thrombosis (CVA/venous sinus throm); HTN emergencies; pseduotumor cerebri; migraines; meningitis; depression
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what are the red flags associated with SLE and headache?
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Severe, unremitting HA; abnormal vital signs; abnormal neurologic exam; papilledema
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what are the characteristics of lupus cerebritis?
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1. HA
2. decreased concentration 3. poor school performance 4. psychosis 5. seizures 6. stroke 7. chorea 8. vertigo 9. transverse myelitis 10. peripheral neuropathy |
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what are the labs to be done for SLE and headache?
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cbc, chem, lft's C3, C4, dsDNA, UA, Anti-ribosomal P antibodies
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what imaging is usually ordered for SLE and headache?
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MRI/MRV/MRAk
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what do they look for with an LP for SLE and headache?
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oligoclonal bands and anti-neuronal antibodies: elevated WBC/protein with cerebritis
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what are the treatments for SLE and headache?
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1. if patient is well apearing start by treating as migraine
2. if no improvement in HA, further workup required |
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what are the etiologies of SLE and Fever/Infection?
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1. immunosupression (PO, SC, or IV)
2. functionally asplenic 3. Meds always on the differential 4. Never stop prednisone |
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What is the work up for a patient with SLE and fever/infection that looks well?
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Likey viral but, CBC, Blood culture, no change in meds
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for patients with SLE and mid baterial infection, what is likely and what should be done?
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uncomplicated UTI, strep throat, mild pneumonia. Give Abx, stop immunosuppression but continue prednisone
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what is done for severe infection/sepsis with a pt with SLE?
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1. abx
2. supportive care 3. hold immunosuppression (can leave on plaquenil, don't stop pred) 4. stress dose of steroids to prevent adrenal crisis |
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what is an adrenal crisis?
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1. hypotension/shock
2. abdominal pain, vomiting, diarrhea 3. electrolyte abnormalities (low NA and glucose; high potass; met acidosis) |
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what is the typical stress dose of steroids?
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prednisone 0.5-1mg/kg/day Hydrocortisone 100mg = prednisone 25mg
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what are the etiologies for SLE and ctyopenias?
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1. anemia of chronic disease
2. ITP 3. hemolytic anemia (coombs +) 4. TTP/HUS 5. macrophage activation syndrome |
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what are the red flags for SLE and cytopenia?
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1. abnormal vital signs
2. bruising 3. abnormal coagulation tests |
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what is MAS?
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similar to hemophagocytic lymphohistocytosis (HLH): reactive process of antigen presenting cells (macrophages, histiocytes and CDR+ T cells)
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what are the symptoms of MAS?
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1. potentially life threatening
2. Fever, HSM 3. consumptive coagulopathy (DIC) 4. severe cytopenias * plts 5. extreme hyperferritinemia (>10,000) 6. high CRP and LOW ESR |
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what is the work up for SLE and cyptopenias?
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cbc, chem, lft's \UA, ESR CRP; retic, haptoglobin, LDH, Coags, ferriting, triglycerides
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what is the tx for MAS
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high dose steroids and cylcosporine
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what are the take home points for SLE?
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1. always ask about immunosupression
2. always consider infection/thromboembolitic even in diff3. Never stop prednisone |
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what is the maternal antibody that is transferred with neonatal lupus>?
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anti-Ro (SS-A)
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what are the complications of neonatal lupus
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1. rash
2. Heart block (3rd degree) 3. hepatitis 4. neutropenia/thrombocytopenia 5. hydrops fetalis |
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__% of babies born to mothers with lupus with have heart block
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50
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the damage to the heart in children with mothers with lupus occurs in the __ semester
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2nd
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what is the treatment for neonatal lupus?
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supportive care and possibly pacemaker
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what are the symptoms of neonatal lupus?
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racoon eyes, annular plaques and annular scaling
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what does D-SLE stand for?
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drug induced lupus D: drugs for the heart (procainimide) S: sulfonamides L: lithium E: epilepsy medications
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of ther DSLE, what drugs can induce lupus?
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INH, minocylcine
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ToF: drug induced SLE is usually irreversible
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FALSE
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what is the hallmark for rhumatologic disease due to lyme?
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morning stiffness, limp or falling
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what are the symptoms of JIA?
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1. morning stiffness
2. easity fatigued 3. joint swelling 4. minimal pain 5. Joint is NEVER red or tender 6. loss of function |
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a patient with lyme disease will have swelling that is ____
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out of proportion to pain
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what is the ACR classification criteria for JIA?
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1. under age 16 at onset
2. lasts 6 weeks 3. arthritis in at least one joint 4. exclusion of other rheum diseases |
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what are the 3 subgroups of JIA named after 6 months
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1. systemic: arthritis and fever
2. pauciarticular: 4 or fewer joints 3. polyarticular: 5 or more joints |
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what is oligoarticular JIA?
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persistent and extended (>4 joints after 6 months)
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children with RF ___ __ JIA must be followed by ophtho.
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positive polyarticular
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enthesistis-related arthritis is usually ___
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errosive
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what is the largest subgroup of JIA?
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pauci
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ToF: pauci JIA involves the large joints including the hip
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false: no hip
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what are the serologic findings with pauci JIA?>
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positive ANA; negtive RF
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what are the main morbitdities from pauci JIA?
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1. asymptomatic anterior uveitiis (associated with pos ANA) can lead to blindness
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what are the labs to draw for pauci JIA?
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cbc: high inflammatory markers, plt ct, ESR, CRP, Lyme to r/o other causes ANA
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what are the characteristics of polyarticular arthritis>?
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1. >5 joints
2. more in girls 3. symmetric involvement of joints 4. usually involves TMJ and cervical spine |
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what is the lab work up for polyarticular?
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1. cbc
2. RF/CCP 3. ESR 4. CRP 5. Lyme 6. Ophtho |
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what are the joints involved in poly JIA?
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small and large: the PIP, MCP and wrist, can also have rheum nodules with positive ANA RF can be +- or-
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Tof: males are more likely to get systemic JIA than females?
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false, =
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what are the characteristics of systemic JIA?
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1. quotidian fever
2. arthritis 3. HSM 4. LAD 5. Serositis 6. leukocytosis 7. evanescent, salmon colored rash 8. ana and rf neg |
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in systemic onset JIA (so JIA), how does it present
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with high spiking fevers and arthritis within 6 months after onset of fever. Athralgia and arthritis worsen with temp, salmon pink macular lesions, HSM, pleuritis and pericarditis in 50% of cases
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ToF: iritis often occurs in soJIA patients
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false, never
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what happens to the blood counts of in soJIA?
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inc WBC, platelets, ESR and CRP; decreased hgb/hct; neg ANA and Rh factor
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what are the outcomes of soJIA?
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50% of children with soJIA completely recover and other 50% go on to chronic illness
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what is the new recommmendations for children with soJIA?
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new TNF inhibitors have been approved for use. Must get a PPD before treatment.
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biologic agents used in soJIA improve …
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the way the joint looks and prevents severe errosive arthritis
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what is the first line tx for soJIA?
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NSAIDs
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ho many weeks of nsaid therapy is needed for soJIA?
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4 to 6
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___ ___ injections suppress arthritis rapidly without systemic treatment of soJIA
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intra-aurticular steroid
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When are steroid injections beneficial for soJIA?
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when oral meds are not tolerated
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the effects of intra-articular steroids are felt within ___ and last for __ to __ months
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24-48 hours; 3-9
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what ist he key to JIA?
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1. follow up and monitoring
2. 33% grow out of it 3. 33% have waxing and waning course 4. 33% will continue to have it t/o childhood into adulthood |
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what are the common complications of JIA?
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1. joint damage
2. bony deformities 3. joint laxity 4. iritis and blindness |
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what are the common findings of JIA?
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1. swollen/stiff/painful/erythematous joints
2. morning stiffness 3. limp 4. decreased activity level 2ndary to pain 5. pain alone is not enough |
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what is spondyloarthritis?
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enthesitis-related JIA; asymmetrical arthritis affected 4 or fewer joints
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what is meant by enthesis?
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involves insertion of ligaments and tendons into bone
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what gender most often gets sponlyoarthritis?
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males
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how do children with ankylosing spondylitis present?
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usually males with back pain and morning stiffness relieved with exercise
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what is seen on xray of spine with ankylosing spondylitis
|
bamboo spine
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what are the lab findings of ankylosing spondylitis?
|
1. HLA-B27 positive
2. increased ESR 3. ANA and RF are neg |
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what is the tx for spondylitis?
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NSAIDs, sulfasalazine and mtx
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what are the constitutional symptoms associated with JDM?
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1. fatigue
2. fever 3. weight loss 4. muscle weakness |
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list the physical exam findings with JDM.
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1. heliotrope rash
2. photosensitive rash 3. nail fold telangiectasiasis 4. gottron papules 5. gowers sign 6. dysphagia/dysphonia/dyspnea 7. nodular calcifications |
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what can the heliotrope rash of JDM be confused with?
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eczema of the eyelids
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what do gottons sign look like?
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red, thickened, scaly skin overlying PIP
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what is the photosenstive rash of JDM resemble?
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striae
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describe calcinosis?
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hard and they move
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what are the labs done in a JDM workup?
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increased CK, aldolase, LDH, AST, ALT; increase vWF antigen; usually nl ESR/CRP (can have +ANA)
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what is seen on radiology for JDM?
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increased T2 signal on MRI b/l thighs
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what are the treatment options for JDM?
|
1. sunscreen
2. steroids 3. MTx 4. IVIG |
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what are the complications of JDM?
|
at high risk for gastric perf
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what is the most common small vessel vasculitis in children?
|
Henoch-schonlein purpura
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HSP is a __ mediated __ vasculitis
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IgA; leucocytoclastic
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HSP is usually preceded by a __ or __
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URI or strep infections
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what is the age and disease course for HSP
|
2-13 year olds and is usually self limiting
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what is the clinical manifestation of HSP?
|
palpable purpura; angioedema; colicky pain (may procede rash); intussesception; arthritis; hematuria and proteinuria
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where is the rash of HSP often occuring?
|
buttocks and lower extremities
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what are the lab findings for HSP?
|
normal plts; mild/mod increase in WBC; UA can be nml or nephrotic; increase ESR; ANA/RF neg; C3 and C4 normal; ANCA neg
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how long does hsp usually last?>
|
6-8 weeks
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the prognosis of hsp depends on __ involvement
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renal
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a small % of HSP pts experience…
|
massive GI hemorrhage, renal insuffiency or severe glomerulonephritis
|
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what is the tx for HSP?
|
supportive therapy for joint and abdominal; NSAIDs may aggravate abdominal pain; controversal role of steroids
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what are the symptoms of scleroderma?
|
linear bands of hard transluscent, shiny skin; flexion contracture; muscle atrophy
|
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what is morphea?
|
type of localized scleroderma that has flesh colored, erythematous or purplish patches on skin
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what is CREST?
|
symptoms of systemic scleroderma: Calcinosis; raynaud sclerodactyly telangectasia
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