Rheumatology

Question 1

Symptoms suggestive of rheumatic disease

Answer 1

joint pain
fever
fatigue
rash

Question 2

Arthralgia WITHOUT physical findings of arthritis suggests

Answer 2

infection
malignancy
orthopedic conditions
benign syndromes
pain syndromes like fibromyalgia

Question 3

Kawasaki Disease (Mucocutaneous Lymph Node Syndrome)

Answer 3

FEVER for at least 5 days + 4 of the ff

CRASH

Conjunctivitis - bilateral, bulbar injection (-) exudate

Rash

Adenopathy - solitary, cervical adenopathy > 1.5 cm

Strawberry tongue and other oropharyngeal changes

Hand changes - brawny edema, induration, peeling around the nail beds

Question 4

Common in ACUTE phase especially in younger then 1 y/o patients

Answer 4

PERINEAL DESQUAMATION

Question 5

Begins 1-3 weeks AFTER the onset of illness

Answer 5

PERIUNGUAL DESQUAMATION

Question 6

Develop in the 2nd-3rd week of illness in untreated cases

Answer 6

Coronary Artery Aneurysms

Question 7

Clinical phases of Kawasaki Disease

Answer 7

Acute Febrile
Subacute
Convalescent

Question 8

Lasts 1-2- weeks; fever and other acute signs of illness

Answer 8

Acute Febrile Phase

Question 9

Begins when fever and other signs have abated

Highest risk of sudden death in those who developed aneurysms

Lasts until 4 weeks

Answer 9

Subacute Phase

Question 10

Begins when all clinical signs have disappeared and continues until ESR and CRP return to normal

Answer 10

Convalescent Phase

Question 11

Any infant or child with 5 or more days of fever with 2-3 of the principal clinical features but have compatible laboratory findings

May have manifestations such as – renal failure, unilateral facial nerve palsy, pulmonary infiltrates

Answer 11

Atypical Kawasaki Disease

Question 12

Considered in all children with unexpected fever for 5 or more days with 3 or less of the clinical criteria

More common in young infants

Answer 12

Incomplete Kawasaki Disease

Question 13

This should be performed at diagnosis of Kawasaki and repeated after 2-3 weeks of illness

If both are normal – repeat study after 6-8 weeks

Answer 13

2D-Echo

Question 14

Treatment of Kawasaki Disease

Acute Stage

Answer 14

IVIg 2 g/kg over 10-12 hrs

Aspirin 80-100

Question 15

Treatment of Kawasaki Disease

Convalescent Stage

Answer 15

Aspirin 3-5 mg/kg OD daily until 6-8 weeks

Question 16

Long term therapy for patients w/ Coronary Abnormalities

Answer 16

Aspirin 3-5 mg
Clopidogrel 1 mg/kg/day
Warfarin or LMWH

Question 17

Persistent of recrudescent fever 36 hr after completion of the initial IVIG infusion

INCREASED RISK FOR CAA

Answer 17

IVIG Resistant KD

give another dose of IVIG at 2 g/kg

methylprednisolone - if poor response pa din

Question 18

Serious, rare condition in children in which the body’s own immune system overreacts to a stimulus

Results in inflammation of multiple organ systems → leads to impaired organ function and organ failure

Diagnosed in <21 years of age in mostly Europe and the USA

Unknown cause

Mounting evidence that is linked to COVID-19

Answer 18

Multisystem Inflammatory Syndrome in Children (MIS-C)

HEART - commonly affected

Question 19

MIS-C

Answer 19

Patient is 21 years of age or younger and has had a fever of 38°C for at least 24 hours

blood work shows indications of inflammation

requires treatment at a hospital (often in the intensive care unit)

due to severe illness that includes dysfunction of two or more organs, particularly the heart, blood vessels, GI organs, lungs, kidneys, skin, eyes, or nervous system

Question 20

The MC of pediatric inflammatory myopathies

Systemic vasculopathy with characteristic cutaneous findings and focal areas of myositis – progressive proximal muscle weakness

Answer 20

JUVENILE DERMATOMYOSITIS

Gottron papules
Heliotrope eyelids/Heliotrope rash
Shawl sign

Question 21

Clinical manifestations of Juvenile Dermatomyositis

Answer 21

Neck flexor or abdominal muscle weakness

Unable to sit up or head lag during infancy, and Gower sign (use of hands on thighs to stand from a sitting position)

Derangement of upper airway function

Dysphagia - severe prognostic sign

Constipation, abdominal pain, or diarrhea

Dilated cardiomyopathy

Question 22

Diagnostic Criteria for Juvenile Dermatomyositis

Answer 22

Classic rash (Heliotrope rash of the eyelids, Gottron papules)

PLUS 3 of the ff

weakness (symmetric, proximal)

muscle enzyme elevation equal or >1 (CK, aspartate aminotransferase, LDH, aldolase)

electromyographic changes (myopathy, denervation)

muscle biopsy (necrosis, inflammation)

Question 23

Diagnosis and Lab Findings in Juvenile Dermatomyositis

Answer 23

PE - nailfold of capillaries show periungual avascularity with capillary dropout and vessel dilatation with terminal bush formation

ALANINE AMINOTRANSFERASE

Antibodies to Pm/Scl

MRI

Question 24

Confirms diagnosis of Juvenile Dermatomyositis if the clinical and lab manifestations are inconclusive

Answer 24

Muscle Biopsy

Question 25

Treatment for Juvenile Dermatomyositis

Answer 25

Corticosteroids Methotrexate Folic acid IVIG

Question 26

7 y/o rash - raised, pinkish to erythematous purpuric lesions -- buttocks to the lower extremities R knee pain

Answer 26

HENOCH SCHONLEIN PURPURA IgA mediated vasculitis of small vessels (dermal capillaries and postcapillary venules) 3-10 y/o linked with NEPHRITIS: HLA B34 and HLA DRB1*01 PALPABLE PURPURA IN THE DEPENDENT PARTS

Question 27

Treatment of HSP

Answer 27

symptomatic self limiting STEROIDS - severe abdominal pain and joint pain SERIAL URINALYSIS for 6 mos after diagnosis

Question 28

Skin biopsy result of HSP

Answer 28

leukocytoclastic angiitis

Question 29

Renal biopsy result of HSP

Answer 29

IgA mesangial deposition

Question 30

Systemic JIA fever rash joint pain swelling

Answer 30

Arthritis in equal or > 1 joints with or preceded by fever for at least 2 weeks in duration documented to be daily for at least 3 days and accompanied by 1 or more of the ff 1. Evanescent erythematous rash 2. Generalized lymph node enlargement 3. Hepatomegaly or splenomegaly or both 4. Serositis

Question 31

Oligoarthritis JIA

Answer 31

Arthritis affecting 1-4 joints during the 1st 6 mos

Question 32

Pharmacologic Treatment of Juvenile Idiopathic Arthritis

Answer 32

NSAIDS Naproxen Ibuprofen Meloxicam DMARDS MTX Sulfasalazine ANTI TUMOR NECROSIS FACTOR Etanercept IL-1 INHIBITOR Canakinumab IL 6 RECEPTOR Tocilizumab

Question 33

Autoantibody production against self-antigens -- inflammatory damage to target organs FEMALE skin, joints, kidneys, blood-forming cells, blood vessels and CNS

Answer 33

SYSTEMIC LUPUS ERYTHEMATOSUS

Question 34

Criteria for diagnosis of SLE

Answer 34

SOAP BRAIN MD Serositis (pleuritis, pericarditis) Oral ulcers (painless) Arthritis (2 or more joints) Photosensitivity Blood changes (anemia, leukopenia, low platelet) Renal disorder (persistent proteinuria, cellular casts) ANA abnormal titer Immunological changes (anti-DNA Ab, anti-Sm) Neurological signs (seizures, frank psychosis) Malar rash Discoid rash

Question 35

Excellent screening tool for SLE but can be (+) in other rheumatic conditions

Answer 35

ANA

Question 36

More specific for lupus and reflects the degree of disease activity

Answer 36

Anti-DS DNA

Question 37

Specific for diagnosis of SLE

Answer 37

Anti Smith AB

Question 38

MC affected organ in MIS-C

Answer 38

Heart

Question 39

More likely to be correlated with ANA POSITIVITY

Answer 39

``` anterior uveitis younger age at disease onset female sex asymmetric arthritis lower number of involved joints over time ```

Question 40

Risk factors for devt of uveitis in various types of juvenile idiopathic arthritis

Answer 40

oligoarthritis subtype female ANA (+) onset of arthritis earlier than 6 y/o

Question 41

Clinical manifestations of SPONDYLOARTHRITIS

Answer 41

``` arthritis of sacroiliac joints arthritis of the hips enthesitis symptomatic uveitis GI inflammation ```

Question 42

Reactive arthritis typically follows ENTERIC infection with

Answer 42

``` Salmonella sp Shigella flexneri Yersinia enterolitica Campylobacter jejuni Organitourinary tract infection w. C. trachomatis ```

Question 43

The skin manifestation MOST suggestive of SLE in children

Answer 43

Discoid rash

Question 44

Recommended for all individuals with SLE if tolerated Prevents SLE flares, improves lipid profiles and may have beneficial impact in mortality and renal outcomes

Answer 44

HYDROXYCHLOROQUINE

Question 45

The most serious complication d.t. neonatal lupus

Answer 45

Congenital Heart Block

Question 46

Common manifestations of Juvenile Dermatomyositis

Answer 46

``` Heliotrope rash Photosensitivity Facial erythema crossing the nasolabial folds Gottron papules Periungual telangiectasias ```

Question 47

Adolescence Symmetric occurrence (-) tissue necrosis and gangrene

Answer 47

Raynaud Disease

Question 48

Hallmark of Behcet disease

Answer 48

ORAL ULCERATION ``` very painful recurrent involve any location in the oral cavity last 3-10 days heal without scarring ```

Question 49

Treatment for Behcet Disease

Answer 49

(-) major organ involvement - COLCHICINE (+) vascular involvement - CYCLOPHOSPHAMIDE

Question 50

Primary Sjogren Syndrome

Answer 50

CHILDREN *recurrent parotitis and recurrent parotid gland enlargement ADULT *SICCA SYNDROME - dry mouth, painful mucosa, sensitivity to spicy foods, halitosis, widespread dental caries)

Question 51

Subtype of JIA with HIGHEST PREVALENCE of AA amyloidosis

Answer 51

Systemic JIA