Rheumatology Flashcards

1
Q

Positive ANA is seen in in which viral infection

A

EBV and Parvovirus B19

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

ANA is positive in up to __ of normal children

A

30

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Skin reaction characterized by small hypopigmented depressed scars occuring in areas of minor skin trauma. May be caused by NSAIDs, especially naproxen

A

pseudoporphyria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Most significant potential adverse effect of hydroxychloroquine

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q
Leflunomide
indication
classification
dose
adverse reaction
A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q
Methotrexate
Indication
Classification
Dose
Adverse reaction
A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q
Hydroxychloroquine 
Indication
Classification
Dose
Adverse Reaction
A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q
Sulfasalazine
Indication
Classification
Dose
Adverse Reaction
A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q
Mycophenolate mofetil
Indication
Classification
Dose
Adverse reaction
A

Mycophenolate mofetil
I: SLE, uveitis, autoimmune skin manifestations
C: Immunosuppressive
D: 600 mg/m2 BID
AR: GI intolerance, cytopenias, secondary malignancies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q
Etanercept
Indication 
Classification
Dose
Adverse reaction
A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q
Adalimumab
Indication
Classification
Dose
Adverse reaction
A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q
Infliximab
Indication
Classification
Dose
Adverse reaction
A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q
Abatacept
Indication
Classification
Dose
Adverse reaction
A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q
Rituximab
Indication
Classification
Dose
Adverse reaction
A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q
Belilumab
Indication
Classification
Dose
Adverse reaction
A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q
Anakinra
Indication
Classification
Dose
Adverse reaction
A
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q
Canakinumab
Indication
Classification
Dose
Adverse reaction
A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q
Tocilizumab
Indication
Classification
Dose
Adverse reaction
A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Intravenous immunoglobulin
Indication
Dose
Adverse reaction

A

IVIG
I: Kawasaki disease, JDMS, SLE
D: 1-2 g/kg IV. For JDMS give monthly
AR: Infusion reaction, aseptic meningitis, renal failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q
Cyclophosphamide
Indication
Classification
Dose
Adverse reaction
A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Most common rheumatic disease in children

A

juvenila idiopathic arthritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Pathologic characteristic of inflammatory synovitis

A

villous hypertrophy and hyperplasia with hyperemia and edema of the synovial tissue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Arthritis definition

A

Intraarticular swelling and presence of 2 or more of the ff: limitation in range of motion, tenderness or pain on motion, warmth

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q
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
A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
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: 1. evanescent erythematous rash (salmon-colored lesions over trunk and proximal ext, lasting <1 hr) 2. generalized lymph node enlargement 3. hepatomegaly and/or splenomegaly 4. serositis
26
Definition of oligoarthritis
Arthritis affecting 1-4 joints during the 1st 6 mo of disease. Two subcategories: 1. Persistent - affecting ≤4 joints throughout disease course 2. Exended - affecting >4 joints after 1st 6 mo large joints of the lower extremities
27
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
28
Psoriatic arthritis
Arthritis and psoriasis, or arthritis and at least 2 of the ff: 1. Dactylitis 2. Nail pitting and onycholysis 3. Psoriasis in a 1st-degree relative
29
Enthesitis-related arthritis
Arthritis and enthesitis or arthritis or enthesitis with at least 2 of the ff: 1. History of sacroiliac joint tenderness or inflammatory lumbosacral pain or both 2. Presence of HLA-B27 antigen 3. Onset of arthritis in a male >6 yo 4. Acute (symptomatic) anterior uveitis 5. History of anykylosing spondylitis, enthesitis-related arthritis, sacroiliitis with IBD, Reiter syndrome or acute anterior uveitis in a 1st-degree relative
30
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
31
``` 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
32
``` 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 ```
33
``` 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
34
``` 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
35
Joint involvement highly suggestive of enthesitis-related arthritis
tarsitis
36
Joint involvement highly suggestive of psoriatic arthritis
distal interphalangeal joints
37
Modified NY criteria for diagnosing ankylosing spondylitis
1. Radiographic evidence of sacroiliitis - sacroiliitis of grade2 or greater bilaterally or at least grade 3 unilaterally 2. Meets at least 1: - limitation of motion in the lumbar spine - limitation of chest expansion
38
Axial spondyloarthritis criteria (Assessment of Spondyloarthritis International Society)
1. At least 3 months of back pain 2. 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
39
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
40
Arthritis that occurs following enteropathic or urogenital infections
Reactive arthritis
41
Probable etiologic microorganisms of reactive arthritis
``` Chlamydia Shigella Salmonella Yersinia Campylobacter ```
42
Symptoms of reactive arthritis begin approximately __ following infection
2-4 weeks
43
Nonsuppurative arthritis, usually in adolescent boys, in association with severe truncal acne.
Pyogenic (sterile) arthritis, pyoderma gangrenosum and acne (Cystic) syndrome
44
__ of patients with reactive arthritis are HLA-B27-positive
75%
45
Deposition of immune complexes within the derma-epidermal junction on immunofluorescence examination
lupus band test
46
SLICC 2012 Criteria
``` 4 criteria, at least 1 clinical, 1 immunologic Serositis Oral ulcers Arthritis (synovitis) P Blood Renal Alopecia Immunologic criteria Neurologic ```
47
Hematologic values in SLE clinical criteria
Leukopenia <4,000 or lymphopenia <1,000 | Thrombocytopenia <100,000
48
Immunologic criteria
``` ANA Anti-dsDNA Anti-Smith Antiphospholipid antibody Low complement Direct Coomb's in the absence of hemolytic anemia ```
49
Blue-violet discoloration of the eyelids that may be associated with periorbital edema Diagnosis?
Heliotrope rash | Juvenile dermatomyositis
50
Erythema seen over the chest and neck after UV light exposure Diagnosis?
Shawl sign | Juvenile dermatomyositis
51
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
52
Thickened erythematous and scaly rash over the palms
mechanic's hands Juvenile dermatomyositis Associated with anti-Jo-1 antibodies
53
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
54
Most common subtype of localized scleroderma
linear scleroderma
55
Triphasic sequence of Reynaud phenomenon
1. Blanching (vasoconstriction) 2. Cyanosis (venous stasis) 3. Erythema (vasodilation)
56
Most common pharmacologic intervention for Reynaud phenomenon
calcium channel blockers nifedipine 30-60 mg sustained release daily amlodipine 2.5-10 mg daily
57
Most frequent initial symptom of Behcet disease
oral ulcer
58
Diagnostic criteria for Behcet disease
Oral ulcers at least 3 times per year plus 2 other major features: 1. recurrent genital ulcers 2. eye lesions 3. characteristic skin lesions 4. pathergy
59
Most frequent vasculitic feature of Behcet disease
deep venous thrombosis of the lower limbs
60
Most common CNS manifestation of Behcet disease in children
dural sinus thrombosis
61
Definition of giant coronary artery aneurysm
>8 mm internal diameter | greatest risk for rupture, thrombosis, stenosis, MI
62
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) ```
63
3 clinical phases of Kawasaki disease
1. Acute febrile phase (1-2 wk) 2. Subacute phase (3 wk) - desquamation, thrombocytosis, CAA, sudden death 3. Convalescent phase (6-8 wk) - until ESR becomes normal
64
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.
65
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
66
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
67
Long-term therapy for patients with coronary abnormalities
1. Aspirin 3-5 mg/kg/d | 2. Clopidogrel 1 mg/kg/d (max 75 mg)
68
Live vaccines should generally be deferred until __ after IVIG therapy
11 months
69
Most common vasculitis of childhood
Henoch-Schonlein purpura
70
Diagnostic criteria for HSP (American College of Rheumatology)
Two of the ff: 1. palpable purpura 2. age of onset ≤20 yr 3. bowel angina (postprandial abdominal pain, bloody diarrhea) 4. biopsy demonstrating intramural granulocytes in small arterioles and/or venules
71
Diagnostic criteria for HSP (European)
Palpable purpura (in absence of coagulopathy or thrombocytopenia) and 1 or more of the ff: 1. abdominal pain 2. arthritis or arthralgia 3. biopsy of affected tissue demonstrating predominant IgA deposition 4. Renal involvement (proteinuria <3 g/24 hr), hematuria or red cell cast
72
Diagnostic criteria for Takayasu arteritis
Angiographic abnormalities (angio) of the aorta or its main branches and at least one of the ff: 1. decreased peripheral artery pulses and .or claudication 2. blood pressure difference between arms or legs of >10 mmHg 3. bruits over the aorta and/or its major branches 4. hypertension 5. elevated ESR/CRP
73
Polyarteritis nodosa | Histopathology
necrotizing vasculitis in medium or small arteries
74
Polyarteritis nodosa | Angiographic abnormalities
"beads on a string" | aneurysm, stenosis or occlusion of a medium or small size artery
75
Polyarteritis nodosa | Cutaneous findings
livedo reticularis, tender subcutaneous nodules, superficial skin ulcers, deep skin ulcers, digital necrosis, nail bed infarctions or splinter hemorrhages
76
Polyarteritis nodosa | Muscle involvement
Myalgia or muscle tenderness
77
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
78
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