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Pedia > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

Symptoms suggestive of rheumatic disease

A

joint pain
fever
fatigue
rash

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2
Q

Arthralgia WITHOUT physical findings of arthritis suggests

A 
infection
malignancy
orthopedic conditions
benign syndromes
pain syndromes like fibromyalgia
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3
Q

Kawasaki Disease (Mucocutaneous Lymph Node Syndrome)

A

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

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4
Q

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

A

PERINEAL DESQUAMATION

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5
Q

Begins 1-3 weeks AFTER the onset of illness

A

PERIUNGUAL DESQUAMATION

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6
Q

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

A

Coronary Artery Aneurysms

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7
Q

Clinical phases of Kawasaki Disease

A

Acute Febrile
Subacute
Convalescent

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8
Q

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

A

Acute Febrile Phase

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9
Q

Begins when fever and other signs have abated

Highest risk of sudden death in those who developed aneurysms

Lasts until 4 weeks

A

Subacute Phase

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10
Q

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

A

Convalescent Phase

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11
Q

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

A

Atypical Kawasaki Disease

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12
Q

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

A

Incomplete Kawasaki Disease

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13
Q

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

A

2D-Echo

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14
Q

Treatment of Kawasaki Disease

Acute Stage

A

IVIg 2 g/kg over 10-12 hrs

Aspirin 80-100

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15
Q

Treatment of Kawasaki Disease

Convalescent Stage

A

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

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16
Q

Long term therapy for patients w/ Coronary Abnormalities

A

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

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17
Q

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

INCREASED RISK FOR CAA

A

IVIG Resistant KD

give another dose of IVIG at 2 g/kg

methylprednisolone - if poor response pa din

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18
Q

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

A

Multisystem Inflammatory Syndrome in Children (MIS-C)

HEART - commonly affected

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19
Q

MIS-C

A

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

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20
Q

The MC of pediatric inflammatory myopathies

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

A

JUVENILE DERMATOMYOSITIS

Gottron papules
Heliotrope eyelids/Heliotrope rash
Shawl sign

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21
Q

Clinical manifestations of Juvenile Dermatomyositis

A

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

22
Q

Diagnostic Criteria for Juvenile Dermatomyositis

A

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)

23
Q

Diagnosis and Lab Findings in Juvenile Dermatomyositis

A

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

ALANINE AMINOTRANSFERASE

Antibodies to Pm/Scl

MRI

24
Q

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

A

Muscle Biopsy

25
Q

Treatment for Juvenile Dermatomyositis

A

Corticosteroids
Methotrexate
Folic acid
IVIG

26
Q

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

A

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

27
Q

Treatment of HSP

A

symptomatic
self limiting

STEROIDS - severe abdominal pain and joint pain

SERIAL URINALYSIS for 6 mos after diagnosis

28
Q

Skin biopsy result of HSP

A

leukocytoclastic angiitis

29
Q

Renal biopsy result of HSP

A

IgA mesangial deposition

30
Q

Systemic JIA

fever
rash
joint pain
swelling

A

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
31
Q

Oligoarthritis JIA

A

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

32
Q

Pharmacologic Treatment of Juvenile Idiopathic Arthritis

A

NSAIDS
Naproxen
Ibuprofen
Meloxicam

DMARDS
MTX
Sulfasalazine

ANTI TUMOR NECROSIS FACTOR
Etanercept

IL-1 INHIBITOR
Canakinumab

IL 6 RECEPTOR
Tocilizumab

33
Q

Autoantibody production against self-antigens – inflammatory damage to target organs

FEMALE

skin, joints, kidneys, blood-forming cells, blood vessels and CNS

A

SYSTEMIC LUPUS ERYTHEMATOSUS

34
Q

Criteria for diagnosis of SLE

A

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

35
Q

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

36
Q

More specific for lupus and reflects the degree of disease activity

A

Anti-DS DNA

37
Q

Specific for diagnosis of SLE

A

Anti Smith AB

38
Q

MC affected organ in MIS-C

39
Q

More likely to be correlated with ANA POSITIVITY

A 
anterior uveitis
younger age at disease onset
female sex
asymmetric arthritis
lower number of involved joints over time
40
Q

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

A

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

41
Q

Clinical manifestations of SPONDYLOARTHRITIS

A 
arthritis of sacroiliac joints
arthritis of the hips
enthesitis
symptomatic uveitis
GI inflammation
42
Q

Reactive arthritis typically follows ENTERIC infection with

A 
Salmonella sp
Shigella flexneri
Yersinia enterolitica
Campylobacter jejuni
Organitourinary tract infection w. C. trachomatis
43
Q

The skin manifestation MOST suggestive of SLE in children

A

Discoid rash

44
Q

Recommended for all individuals with SLE if tolerated

Prevents SLE flares, improves lipid profiles and may have beneficial impact in mortality and renal outcomes

A

HYDROXYCHLOROQUINE

45
Q

The most serious complication d.t. neonatal lupus

A

Congenital Heart Block

46
Q

Common manifestations of Juvenile Dermatomyositis

A 
Heliotrope rash
Photosensitivity
Facial erythema crossing the nasolabial folds
Gottron papules
Periungual telangiectasias
47
Q

Adolescence

Symmetric occurrence

(-) tissue necrosis and gangrene

A

Raynaud Disease

48
Q

Hallmark of Behcet disease

A

ORAL ULCERATION

very painful
recurrent
involve any location in the oral cavity
last 3-10 days
heal without scarring
49
Q

Treatment for Behcet Disease

A

(-) major organ involvement - COLCHICINE

(+) vascular involvement - CYCLOPHOSPHAMIDE

50
Q

Primary Sjogren Syndrome

A

CHILDREN
*recurrent parotitis and recurrent parotid gland enlargement

ADULT
*SICCA SYNDROME - dry mouth, painful mucosa, sensitivity to spicy foods, halitosis, widespread dental caries)

51
Q

Subtype of JIA with HIGHEST PREVALENCE of AA amyloidosis

A

Systemic JIA

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