Rheumatology

Question 1

Antibodies specific for SLE

Answer 1

anti-ds DNA (assoc with renal disease activity) and anti-Sm

Question 2

SLE criteris for Dx (4/11)

Answer 2

Malar rash 60%, Discoid rash, Photosensitivity 90%, Oral Ulcers 20%, Nonerosive arthritis, Pleuritis or pericarditis, Renal disorder, Seizures or psychosis, Hematologic disorder, +ve Ab (anti-dsDNA, anti-Sm or antiphospholipid/lupus anticoagulant), +ve ANA

Question 3

Antibodies NOT specific for SLE

Answer 3

ANA, antiphospholipid/lupus anticoagulant

Question 4

Oral ulcers on the hard palate associated with

Answer 4

SLE active disease

Question 5

Ab assoc with psychiatric disorders

Answer 5

Anti-ribosomal P

Question 6

Lab eval for SLE

Answer 6

CBC, ESR, CRP, C3,C4, LFTs, UA, VDRL

Question 7

Assoc with hemolytic anemia

Answer 7

Coomb’s

Question 8

Ab assoc with Neonatal lupus or cutaneous lupus

Answer 8

Anti-Ro (SS-A) and Anti-LA (SS-P)

Ab directed at RoRNP, HLADR3

Question 9

Ab assoc with Thrombosis, Thrombocytopenia, Hemolytic anemia

Answer 9

Antiphospholipid, Anti cardiolipin

Question 10

Childhood SLE more severe because

Answer 10

Renal disease 50-90%

CNS disease 40%

Question 11

SLE Mortality most frequently due to

Answer 11

1. Infections

2. MI sec to vasculitis and steroids

Question 12

Ab assoc with MCTD

Answer 12

Anti-RNP AB

Question 13

Baby born starts developing rash on exposure to Sun or UV light, Heratblock (15-30%), Hepatitis (8%), Cytopenias 6%, Hemolytic Anemia

Answer 13

Only 10% of women who cary the Ab have symptoms, so it may be their first child with Neonatal lupus

Question 14

Large vessel vasculitis

Answer 14

Giant cell - older people

Takayassu’s - teenage girls

Question 15

Medium vessel vasculitis

Answer 15

Polyarteritis Nodosa - children

Kawasaki’s

Question 16

Small vessel vasculitis

Answer 16

Wegner’s granulomatosis (Granulomatosis Polyarteritis)
Churg Straus (assoc with asthma)
HSP
Cryoglobulinemic vasculitis

Question 17

MC vasculitis in children

Answer 17

HSP, Purpura without thrombocytopenia & normal coats.
IgA deposits skin, joint, GI
Renal imvolvement may occur 3 months after disease onset

Question 18

Complications of HSP

Answer 18

Intusucception, Bowel infarction/perforation, hydrops of the gallbladder, pancreatitis, Massive GI bleeding, Proteinuria, hematuria, Renal failure

Question 19

Evaluation of HSP

Answer 19

CBC, PT/PTT, CMP. ESR/CRP (ususally normal)

UA (protein) Stool (FOCB)

Question 20

HSP treatment

Answer 20

Supportive, Tylenol/NSAIDs for joint pain, steroids for severe GI/Renal complications

Question 21

Mucocutaneous lymph node syndrome

Answer 21

Kawasaki Disease - Medium sized arteries

Question 22

Kawasaki’s disease 5 days of fever + 4/5 criteria?

Answer 22

1. Bilat. Conjuctival Injection,
2. Erythema of oropharynx, lip fissure, strawberry tongue
3. Erythema nad swelling of hands and feet
4. Erythematous rash
5. Cervical lymph node > 1.5 cm

Question 23

Kawasaki syndrome associated clinical findings

Answer 23

Aseptic Meningitis (Irritability),
Arthritis,
Hydrops of Gallbladder
Tachycardia
Myocarditis
Pericarditis
Coronary artery aneurysm

Question 24

Kawasaki disease lab findings

Answer 24

STERILE PYURIA, hi ESR/CRP, hi WBC, left shift, hi Plts, Normocytic normochromic anemia, hi transaminases, hi TG & LDL, low HDL, hyponitremia

Question 25

Kawasaki disease management

Answer 25

IVIG 2 gm/kg over 8-12 hrs, ASA 100 mg/day div QID, Decrease ASA o 3-5mg/kg/day 48hrs after resolution of fever, Continue ASA until all acute phase reactants return to normal unless coronary aneurysms detected. If no adequate response Repeat IVIG, consider steroids, TNF inhibitors

Question 26

Three phases of Kawasaki disease

Answer 26

1. Acute, febrile 10-14 days 2. Subacute 2- 4 weeks until Plt + ESR return to normal 3. convalescent/recovery period months-years, vessels undergo healing, remodeling, scarring

Question 27

c - ANCA

Answer 27

Wegeners (GPA) 30 -90% | Churg-Strauss 25-50%

Question 28

p - ANCA

Answer 28

``` Microscopic polyarteritis 25-75% Churg-Strauss 50-75% Ulcerative Collitis 40-80 % Sclerosing Collangitis 65-85% Crohn's Disease 10-40% ```

Question 29

poly arteritis nodosa

Answer 29

small and medium vessel vasculitis

Question 30

wegners/ granulomatosis + polyarteritis

Answer 30

small vessel vasculitis, c- ANCA, kidney, lung,

Question 31

Bechet-Occlusive small/medium vessel vasculitis

Answer 31

Uveitis, Pathergy

Question 32

Takayasu arteritis (TA), pulseless disease,

Answer 32

large-sized vessel vasculitis, aorta and its branches - nonspecific, such as fever, fatigue, and weight loss,

Question 33

Laboratory findings of Takayasu arteritis

Answer 33

elevated ESR and CRP, leukocytosis, and moderate anemia. Diagnosis is made by angiography or MRA demonstrating segmental involvement

Question 34

Heat, swelling of joint; Pain, limitation of motion

Answer 34

Juvenile Idiopathic Arthritis | 6 weeks

Question 35

Oligo/Pauci articular JIA

Answer 35

<=4 joints (hips rare), 2-3 yrs, F:M 5:1, lack of systemic involvement, UVEITIS, RF-ve, ANA +ve, excelent prognosis except eyesight, 50-60% of all JIA

Question 36

Oligo/Pauci articular JIA Eye findings

Answer 36

Anterior Uveitis, Band Keratopathy, Catracts, Glaucoma, Needs optho consultq3months

Question 37

Oligo/Pauci articular JIA - Xray indiacted to ---

Answer 37

r/o infection / malignancy. affected limb grows longer due to increased blood supply

Question 38

Poly articular JIA

Answer 38

> 5 joints, symmetric, throughout childhood, F:M 3:1, systemic involvement mild but there, ARTHRITIS, rare (5%) chronic uveitis, RF+ve (10%) increases with age, prognosis gaurded to moderately good.

Question 39

Poly articular JIA complications

Answer 39

short stature/FTT, | micrognathia, osteopenia, flexion contractures

Question 40

Systemic JIA | Very high fever, Rash (salmon colored) that comes and goes

Answer 40

Variable# joints, throughout childhood, F:M 1:1, 50% systemic involvement, rare uveitis, RF, ANA+ 10 %, moderate to poor prognosis

Question 41

Systemic JIA clinical symptoms

Answer 41

HSM, lymphadenopathy, Peri/myo/endocarditis, pleuropulmonary disease, CNS disease, growth failure, Osteoporosis, CERVICAL spine (intubation?)

Question 42

initial meds to treat JIA

Answer 42

NSAIDs, MTX, DMARDs, Hydroxychloquine, Sulfasalazine, Steroids, Cytoxic/immunosuppresive therapy, PT, OT, splinting

Question 43

Juvenile Ankylosing Spondylitis

Answer 43

axial and peripheral skeleton, enthesitis, -ANA, -RF, (HLA-B27+) bilateral inflammation of sacroiliac joints.

Question 44

enthesitis related artheritis

Answer 44

males more common, LE hips, knees, ankles. Enthesitis/Tendenitis (achilles) HLA B27

Question 45

Puffy eyelids. heliotropic rash, proximal muscle weakness, fatigue

Answer 45

Juvenile dermatomyositis, small vessel vasculitis (skin+muscles), MC pediatric myopathy 5-14 yrs F:M 1.7:1, Treated with steroids to prevent calcinosis