Rheumatology Flashcards
Positive ANA is seen in in which viral infection
EBV and Parvovirus B19
ANA is positive in up to __ of normal children
30
Skin reaction characterized by small hypopigmented depressed scars occuring in areas of minor skin trauma. May be caused by NSAIDs, especially naproxen
pseudoporphyria
Most significant potential adverse effect of hydroxychloroquine
Retinal toxicity leading to irreversible color blindness or loss of central vision
Recommended dosing is <6.5 mg/kg/d not to exceed 400 mg/day
Leflunomide indication classification dose adverse reaction
Leflunomide
I: rheumatoid arthritis (pJIA)
C: DMARD
D: PO, OD; 10 to <20 kg 10 mg; 20-40 kg: 15 mg; >40 kg: 20 mg
AR: hepatitis, cytopenias, mucositis, teratogenesis, peripheral neuropathy
Methotrexate Indication Classification Dose Adverse reaction
Methotrexate
I: JIA, Uveitis, SLE (arthritis, serositis, rash)
C: DMARD
D: 10-20 mg/m2/wk PO; 20-30 mg/m2/wk SC
AR: GI intolerance, hepatitis, myelosuppression, mucositis, teratogenesis, lymphoma, interstitial pneumonitis
Hydroxychloroquine Indication Classification Dose Adverse Reaction
Hydroxychloroquine
I: SLE, JDMS, APAS
C: DMARD
D: 5-6 mg/kg PO OD, not to exceed 6.5 mg/kg/d, max dose 400 mg daily
AR: retinal toxicity, Gi intolerance, rash, skin discoloration, anemia, cytopenias, myopathy, CNS stimulation, death
Sulfasalazine Indication Classification Dose Adverse Reaction
Sulfasalazine
I: Spondyloarthropathy, JIA (for poly, contraindicated in active systemic JIA)
C: DMARD
D: 30-50 mg/kg/d BID, max dose 3 g/d
AR: GI intolerance, rash, hypersensitivity reactions, SJS, cytopenias, hepatitis, headache
Mycophenolate mofetil Indication Classification Dose Adverse reaction
Mycophenolate mofetil
I: SLE, uveitis, autoimmune skin manifestations
C: Immunosuppressive
D: 600 mg/m2 BID
AR: GI intolerance, cytopenias, secondary malignancies
Etanercept Indication Classification Dose Adverse reaction
Etanercept
I: JIA
C: TNF-a antagonist
D: 0.8 mg/kg SC once weekly (max dose 50 mg) or 0.4 mg/kg SC twice weekly (max dose 25 mg)
AR: Injection site reaction, rash, demyelinating disorders, cytopenias, potential increased malignancy risk
Adalimumab Indication Classification Dose Adverse reaction
Adalimumab
I: JIA, spondyloarthropathy, psoriatic arthritis, uveitis
C: TNF-a antagonist
D: SC once every other week: 15 to <30 kg: 20 mg, ≥30 kg: 40 mg
AR: injection site reaction, infection, rash, cytopenias, lupus-like syndrome, potential increased malignancy risk
Infliximab Indication Classification Dose Adverse reaction
Infliximab
I: JIA, spondyloarthropathy, uveitis, sarcoidosis (off-label)
C: TNF-a antagonist
D: 5-10 mg/kg IV q4-8 wk
AR: Infusion reactions, hepatitis, potential increased malignancy risk
Abatacept Indication Classification Dose Adverse reaction
Abatacept
I: JIA (poly)
C: Modulator of T-cell activation
D: IV ever 2 weeks x 3 doses then monthly for ≥6 yr of age: <75 kg: 10 mg/kg; 75-100 kg: 750 mg; >100 kg: 1000 mg
AR: Infection, headache, optential increased malignancy risk
Rituximab Indication Classification Dose Adverse reaction
Rituximab
I: SLE, JIA (poly)
C: Anti-CD20 (B cell) antibody
D: 575 mg/m2, max 1000 mg IV on days 1 and 15
AR: Infusion reactions, lymphopenia, reactivation hepatitis B< rash, serum sickness, arthritis, progressive multifocal leukoencephalopathy
Belilumab Indication Classification Dose Adverse reaction
Belimumab
I: Sle
C: Anti-BLyS antibody
D: 10 mg/kg IV every 2 wk x 3 doses then ever 4 wk
AR: Infusion reactions, infection, depression
Anakinra Indication Classification Dose Adverse reaction
Anakinra I: systemic JIA, cryopyrin-associated periodic syndrome (CAPS) C: IL-1 antagonist D: 1-2 mg/kg/d, max dose 100 mg AR: injetion site reactions, infection
Canakinumab Indication Classification Dose Adverse reaction
Canakinumab
I: CAPS, systemic JIA
C: IL-1 antagonist
D: SC every 8 wk (CAPS) every 4 wk (JIA)
15-40 kg: 2 mg/kg (up to 3 mg/kg); >40 kg: 150 mg
AR: injection site reaction, infection, diarrhea, nausea, vertigo, headache
Tocilizumab Indication Classification Dose Adverse reaction
Tocilizumab
I: Systemic JIA, pJIA
C: IL-6 antagonist
D: ≥2 yr and ≥30 kg, 8 mg/kg/dose every 2 wk; ≥2 yr and ≤30 kg, 12 mg/kg/dose every 2 wk
AR: infusion reactions, elevated LFTs, elevated lipids, thrombocytopenia, infections
Intravenous immunoglobulin
Indication
Dose
Adverse reaction
IVIG
I: Kawasaki disease, JDMS, SLE
D: 1-2 g/kg IV. For JDMS give monthly
AR: Infusion reaction, aseptic meningitis, renal failure
Cyclophosphamide Indication Classification Dose Adverse reaction
Cyclophosphamide
I: SLE, vasculitis, JDMS, pulmonary hemorrhage
D: 0.5-1 g/m2 IV (max 1.5 g) monthly for 6 mo induction, then every 2-3 mo
Oral regimen: 1-2 mg/kg/daily, max dose 150 mg
AR: Nausea, vomiting, myelosuppression, mucositis, hyponatremia, alopecia, hemorrhagic cystitis, gonadal failure, teratogenesis, secondary malignancy
Most common rheumatic disease in children
juvenila idiopathic arthritis
Pathologic characteristic of inflammatory synovitis
villous hypertrophy and hyperplasia with hyperemia and edema of the synovial tissue
Arthritis definition
Intraarticular swelling and presence of 2 or more of the ff: limitation in range of motion, tenderness or pain on motion, warmth
ILAR definition of Juvenila Idiopathic Arthritis minimum duration age at onset ≤4 joints in 1st 6 mo after presentation >4 joints in 1st 6 mo after presentation fever, rash, arthritis
JIA (ILAR)
Min duration: ≥6 wk
Age at onset: <16 yr
≤4 joints: oligoarthritis (persistent <4 for course of disease, extended >4 after 6 mo
>4 joints: polyarthritis (RF -/+)
Systemic if with fever, rash
Includes psoriatic arthritis, enthesitis-related arthritis
Definition of systemic JIA
Arthritis in ≥1 joint with or preceded by fever of at ≥2 wk that is documented daily (quotidian) for at least 3 days and accompanied by ≥1 of the ff:
- evanescent erythematous rash (salmon-colored lesions over trunk and proximal ext, lasting <1 hr)
- generalized lymph node enlargement
- hepatomegaly and/or splenomegaly
- serositis
Definition of oligoarthritis
Arthritis affecting 1-4 joints during the 1st 6 mo of disease. Two subcategories:
- Persistent - affecting ≤4 joints throughout disease course
- Exended - affecting >4 joints after 1st 6 mo
large joints of the lower extremities
Definition of polyarthritis
Arthritis affecting ≥5 joints during the 1st 6 mo of disease
May be RF negative or
RF positive: ≥2 tests for RF at least 3 mo apart during the 1st 6 mo of disease are positive
Psoriatic arthritis
Arthritis and psoriasis, or arthritis and at least 2 of the ff:
- Dactylitis
- Nail pitting and onycholysis
- Psoriasis in a 1st-degree relative
Enthesitis-related arthritis
Arthritis and enthesitis or arthritis or enthesitis with at least 2 of the ff:
- History of sacroiliac joint tenderness or inflammatory lumbosacral pain or both
- Presence of HLA-B27 antigen
- Onset of arthritis in a male >6 yo
- Acute (symptomatic) anterior uveitis
- History of anykylosing spondylitis, enthesitis-related arthritis, sacroiliitis with IBD, Reiter syndrome or acute anterior uveitis in a 1st-degree relative
Rare but potentially fatal complication of sJIA, also referred to as secondary hemophagocytic syndrome or hemophagocytic lymphohistiocytosis
Macrophage activation syndrome
- falling platelet count
- falling leukocyte count
- extreme hyperferritinemia
- macrophage hemophagocytosis in the BM
- increased liver enzymes
- persistent continuous fever ≥38C
- falling ESR
- hypofibrinogenemia
- hypertriglyceridemia
Naproxen Indication Classification Dose Adverse reaction
Naproxen
I: JIA (poly, systemic, oligo), spondyloarthropathy, pain, serositis, cutaneous vasculitis, uveitis
C: NSAID
D: 15 mg/kg/d PO in 2 divded doses, max 1000 mg/day
AR: GI intolerance, gastritis, hepatitis, tinnitus, anemia, pseudoporphyria, aseptic meningitis, headache, renal disease
Etodolac Indication Classification Dose Adverse reaction
Etodolac I: JIA, spondyloarthropathy, pain, serositis, cutaneous vasculitis, uveitis C: NSAID D: PO once-daily dose: 20-30 kg: 400 mg 31-45 kg: 600 mg 46-60 kg: 800 mg >60 kg: 1000 mg AR: GI intolerance, gastritis, hepatitis, tinnitus, anemia, pseudoporphyria, aseptic meningitis, headache, renal disease
Celecoxib Indication Classification Dose Adverse reaction
Celecoxib
I: JIA, spondyloarthropathy, pain, serositis, cutaneous vasculitis, uveitis
C: NSAID
D: 10-25 kg: 50 mg PO BID;
>25 kg 100 mg PO BID
AR: GI intolerance, gastritis, hepatitis, tinnitus, anemia, pseudoporphyria, aseptic meningitis, headache, renal disease
Ibuprofen Indication Classification Dose Adverse reaction
Ibuprofen
I: JIA, spondyloarthropathy, pain, serositis, cutaneous vasculitis, uveitis
C: NSAID
D: 40 mg/kg/d PO divided 3 times daily
max 2400 mg per day
AR: GI intolerance, gastritis, hepatitis, tinnitus, anemia, pseudoporphyria, aseptic meningitis, headache, renal disease
Joint involvement highly suggestive of enthesitis-related arthritis
tarsitis
Joint involvement highly suggestive of psoriatic arthritis
distal interphalangeal joints
Modified NY criteria for diagnosing ankylosing spondylitis
- Radiographic evidence of sacroiliitis
- sacroiliitis of grade2 or greater bilaterally or at least grade 3 unilaterally - Meets at least 1:
- limitation of motion in the lumbar spine
- limitation of chest expansion
Axial spondyloarthritis criteria (Assessment of Spondyloarthritis International Society)
- At least 3 months of back pain
- Sacroiliitis on imaging (MRI)
plus 1 feature of spondyloarthritis
- inflammatory back pain
- arthritis
- enthesitis (heel)
- uveitis
- dactylitis
- psoriasis
- IBD
- good response to NSAID
- family history
OR HLA-B27 positive with at least 2 features
Gold standard for early visualization of sacroiliitis
Evidence of bone marrow edema adjacent to the joint on MRI with short T1 inversion recover (STIR) sequences
Arthritis that occurs following enteropathic or urogenital infections
Reactive arthritis
Probable etiologic microorganisms of reactive arthritis
Chlamydia Shigella Salmonella Yersinia Campylobacter
Symptoms of reactive arthritis begin approximately __ following infection
2-4 weeks
Nonsuppurative arthritis, usually in adolescent boys, in association with severe truncal acne.
Pyogenic (sterile) arthritis, pyoderma gangrenosum and acne (Cystic) syndrome
__ of patients with reactive arthritis are HLA-B27-positive
75%
Deposition of immune complexes within the derma-epidermal junction on immunofluorescence examination
lupus band test
SLICC 2012 Criteria
4 criteria, at least 1 clinical, 1 immunologic Serositis Oral ulcers Arthritis (synovitis) P Blood Renal Alopecia Immunologic criteria Neurologic
Hematologic values in SLE clinical criteria
Leukopenia <4,000 or lymphopenia <1,000
Thrombocytopenia <100,000
Immunologic criteria
ANA Anti-dsDNA Anti-Smith Antiphospholipid antibody Low complement Direct Coomb's in the absence of hemolytic anemia
Blue-violet discoloration of the eyelids that may be associated with periorbital edema
Diagnosis?
Heliotrope rash
Juvenile dermatomyositis
Erythema seen over the chest and neck after UV light exposure
Diagnosis?
Shawl sign
Juvenile dermatomyositis
Bright pink or pale, shiny, thickened or atrophic plaques over the proximal interphalangeal joints and distal interphalangeal joints and occasionally on the knees, elbows, small joints of the toes and ankle malleoli
Grotton papules
Juvenile dermatomyositis
Thickened erythematous and scaly rash over the palms
mechanic’s hands
Juvenile dermatomyositis
Associated with anti-Jo-1 antibodies
Diagnostic criteria for JDM
Classic rash: heliotrope rash of the eyelids, Grotton papules
Plus 3 of the ff:
1. Weakness - symmetric, proximal
2. Muscle enzyme elevation (≥1) - CK, AST, LDH, aldolase
3. EMG changes - short, small polyphasic motor unit potentials; fibrillations; positive sharp waves; insertional irritability; bizarre, high-frequency repetitive discharges
4. Muscle biopsy - necrosis, inflammation
Most common subtype of localized scleroderma
linear scleroderma
Triphasic sequence of Reynaud phenomenon
- Blanching (vasoconstriction)
- Cyanosis (venous stasis)
- Erythema (vasodilation)
Most common pharmacologic intervention for Reynaud phenomenon
calcium channel blockers
nifedipine 30-60 mg sustained release daily
amlodipine 2.5-10 mg daily
Most frequent initial symptom of Behcet disease
oral ulcer
Diagnostic criteria for Behcet disease
Oral ulcers at least 3 times per year plus 2 other major features:
- recurrent genital ulcers
- eye lesions
- characteristic skin lesions
- pathergy
Most frequent vasculitic feature of Behcet disease
deep venous thrombosis of the lower limbs
Most common CNS manifestation of Behcet disease in children
dural sinus thrombosis
Definition of giant coronary artery aneurysm
> 8 mm internal diameter
greatest risk for rupture, thrombosis, stenosis, MI
Diagnostic criteria of Kawasaki
Conjunctival injection, nonexudative Rash, polymorphous Adenopathy, cervical, >1.5 cm Strawberry tongue, lip cracking Hand and feet edema, erythema, desquamation (2-3 weeks Burn (fever ≥38.3, at least 5 days)
3 clinical phases of Kawasaki disease
- Acute febrile phase (1-2 wk)
- Subacute phase (3 wk) - desquamation, thrombocytosis, CAA, sudden death
- Convalescent phase (6-8 wk) - until ESR becomes normal
Timing of echocardiography in Kawasaki disease
2Decho should be performed at diagnosis and again after 2-3 of illness. If results are normal, a repeat study should be performed 6-8 wk after onset of illness. If normal repeat after 1 year. If normal repeat every 5 years.
Treatment of acute Kawasaki disease
IVIG 2g/kg. If IVIG resistant, (still febrile after 36 hr), give another dose
high dose aspirin 80-100 mg/kg/d divided q6 until afebrile for at least 48 hr
within 10 days of disease and ideally as soon as possible after diagnosis
Treatment of convalescent stage Kawasaki disease
Aspirin 3-5 mg/kg/d once daily PO until 6-8 wk after illness onset if normal coronary findings throughout course
Long-term therapy for patients with coronary abnormalities
- Aspirin 3-5 mg/kg/d
2. Clopidogrel 1 mg/kg/d (max 75 mg)
Live vaccines should generally be deferred until __ after IVIG therapy
11 months
Most common vasculitis of childhood
Henoch-Schonlein purpura
Diagnostic criteria for HSP (American College of Rheumatology)
Two of the ff:
- palpable purpura
- age of onset ≤20 yr
- bowel angina (postprandial abdominal pain, bloody diarrhea)
- biopsy demonstrating intramural granulocytes in small arterioles and/or venules
Diagnostic criteria for HSP (European)
Palpable purpura (in absence of coagulopathy or thrombocytopenia) and 1 or more of the ff:
- abdominal pain
- arthritis or arthralgia
- biopsy of affected tissue demonstrating predominant IgA deposition
- Renal involvement (proteinuria <3 g/24 hr), hematuria or red cell cast
Diagnostic criteria for Takayasu arteritis
Angiographic abnormalities (angio) of the aorta or its main branches and at least one of the ff:
- decreased peripheral artery pulses and .or claudication
- blood pressure difference between arms or legs of >10 mmHg
- bruits over the aorta and/or its major branches
- hypertension
- elevated ESR/CRP
Polyarteritis nodosa
Histopathology
necrotizing vasculitis in medium or small arteries
Polyarteritis nodosa
Angiographic abnormalities
“beads on a string”
aneurysm, stenosis or occlusion of a medium or small size artery
Polyarteritis nodosa
Cutaneous findings
livedo reticularis, tender subcutaneous nodules, superficial skin ulcers, deep skin ulcers, digital necrosis, nail bed infarctions or splinter hemorrhages
Polyarteritis nodosa
Muscle involvement
Myalgia or muscle tenderness
Granulomatosis with polyangiitis
Wegener granulomatosis
3/6 criteria
1. histopathology showing granulomatous inflammation
2. upper airway involvement
3. laryngeal, tracheal or bronchial involvement
4. ANCA positivity
5. renal involvement
6. Proteinuria, hematuria, red blood cell casts, necrotizing pauci-imune glomerulonephritis
Management of ANCA vasculitides
corticosteroids 2 mg/kg/d oral or 30 mg/kg/d x 3 days IV
in conjunction with cyclophosphamide 2 mg/kg/d
transition to MTX, azathioprine, MM within 3-6 months once remission is achieved
PCP prophylaxis