RHEUMATOLOGY

Question 1

DIAGNOSTIC CRITERIA (BOHAN & PETER, 1975) of JUVENILE DERMATOMYOSITIS

Answer 1

• Classic rash (heliotrope rash of the eyelids, Gottron papules) PLUS 3 of the ff:

1. Weakness (symmetric, proximal)
2. Muscle enzyme elevation equal or >1 (CK, aspartate aminotransferase, LDH, aldolase - most specific)
3. Electromyographic changes (myopathy, denervation) - Classic finding: short, small, polyphasic motor unit potential)
4. Muscle biopsy (necrosis, inflammation)

Question 2

children aged 3-10 years with a history of episodic pain that occurs at night after increased daytime physical activity and is relieved by rubbing; no limp or complaints in the morning

Answer 2

Growing pains or benign nocturnal pain of childhood

Question 3

commonly affects teen-aged males and presents with low back pain; inflammation of joints of axial skeleton and limbs

Answer 3

ANKYLOSING SPONDYLOSIS

Question 4

A 4 year old female patient presents to the clinic with 5 day history of fever with temperatures between 38-39°C. There was bilateral redness of the eyes with no discharge, decreased appetite and intake, physical exam reveals dry cracked lips, erythematous oral and pharyngeal mucosal areas, (+) cervical lymphadenopathy, maculopapular and scarlatiniform rashes most prominent at the groin areas, there was also noted mild swelling and erythema of the hands and feet, HR 108, RR 28 T > 38.8°C what is the most likely diagnosis?

Answer 4

Kawasaki disease

Question 5

• medium-sized vasculitis that affects more males and is most common in the 1 st 5 years of life
• aneurysm of the major coronary arteries is the most characteristic finding
• Perineal desquamation is common in the acute phase especially in younger than 1 year-old patients
• Periungual desquamation begins 1-3 weeks after the onset of illness
• Coronary artery aneurysms develop in the 2nd-3rd week of illness in untreated cases

Answer 5

KAWASAKI DISEASE (MUCOCUTANEOUS LYMPH NODE SYNDROME)

Question 6

• Pattern of crops of palpable purpura in the dependent parts
• Lab tests are neither specific nor diagnostic
• inc ESR, anemia, 50% have ↑ IgA/IgM, (-) ANA & ANCAs
• Skin biopsy: leukocytoclastic angiitis

Answer 6

HSP

Question 7

• 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 is a severe prognostic sign
• Constipation, abdominal pain, or diarrhea
• Dilated cardiomyopathy

Answer 7

JUVENILE DERMATOMYOSITIS

Question 8

AKA pulseless disease

Answer 8

takayasu arteritis

Question 9

intermittent pain esp. in a girl aged 3-10 years that is increased with activity and is associated with hyperextensible joints

Answer 9

Benign hypermobility syndrome

Question 10

Phase of Kawasaki Disease that begins when all clinical signs have disappeared & continues until ESR & CRP return to normal about 6-8 wks after the onset

Answer 10

Convalescent phase

Question 11

A 10 year-old female patient presents with morning stiffness and joint pain later in the day particularly in the knees and sometimes in the ankles it has been bothering her for the past 2 months. PE revealed joint swelling with limitation of range of motion of the knees and ankles. No other complaints noted, HR 90, RR 20, T37.2°C. What is the most likely the diagnosis?

Answer 11

Pauciarticular JRA

• The disease is usually associated with ANA(+)

Question 12

In a patient with known Kawasaki disease, which phase of the illness gives the highest risk for sudden death?

Answer 12

Subacute

Question 13

Treatment of kawasaki disease

Answer 13

Question 14

Which of the ff. is the preferred treatment of Kawasaki in the acute stage of the disease?

Answer 14

IV Ig

Question 15

Acute febrile phase of Kawasaki

Answer 15

last 1-2 wks; fever and other acute signs of illness

Question 16

specific for the diagnosis of SLE

Answer 16

anti-Smith Ab found only in SLE

Question 17

Laboratory findings in kawasaki disease

Answer 17

1. WBC is normal to elevated
2. Elevated ESR, CRP for 4-6 weeks
3. Normocytic, normochromic anemia
4. Platelet count usually normal in the 1st wk of illness but rapidly increases in the 2nd-3rd wk
5. Sterile pyuria
6. Mildly high liver transaminases

Question 18

symmetric thickening and hardening of the skin with fibrous and degenerative changes of the viscera

Answer 18

SCLERODERMA

Question 19

Antibodies to Pm/Scl is associated with

Answer 19

Dermatomyositis

Question 20

Phase of kawasaki disease that begins when fever & other acute signs have abated; associated with desquamation, thrombocytosis, development of coronary aneurysms, and highest risk of sudden death in those who have developed aneurysms; lasts until the 4 th week

Answer 20

Subacute phase

Question 21

• IgA-mediated vasculitis of small vessels (dermal capillaries and postcapillary venules)

• Most common cause of nonthrombocytopenic purpura in children
• Unknown cause; typically follows an URTI
• 3-10 yrs old; male:female ratio 1.2-1.8:1
• Linked with nephritis: HLA-B34 and HLA-DRB1*01

Answer 21

HENOCH-SCHÖNLEIN PURPURA

Question 22

• The most common of pediatric inflammatory myopathies
• Systemic vasculopathy with characteristic cutaneous findings and focal areas of myositis resulting in progressive proximal muscle weakness

Answer 22

JUVENILE DERMATOMYOSITIS

Question 23

CRITERIA FOR DIAGNOSIS OF SLE (1997): 4 of 11 criteria

Answer 23

• 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 24

KAWASAKI DISEASE diagnostic criteria

Answer 24

FEVER for at least 5 days +4 of the following features:

• Conjunctivitis
  
  • Bilateral, bulbar injection WITHOUT EXUDATE
• Rash
  
  • Any form of rash EXCEPT VESICLES/BULLAE
• Adenopathy
  
  • Cervical adenopathy, non-suppurative; > 1.5 cm usually unilateral
• Strawberry tongue and other oropharyngeal changes
  
  • Red cracked, swollen lips
• Hand changes
  
  • Brawny edema, induration
  • Peeling around the nail beds - Periungal desquamation

Question 25

more specific for lupus & reflects the degree of disease activity

Answer 25

Anti-double-stranded DNA

Question 26

A 13 y/o female patient presents to the clinic with complaints of fever, rash, and joint pains. It started 2 months ago with pain and swelling over her knees and sometimes her ankles. She has also been having intermittent fever 1-2x/day ranging from 38-39°C returning to normal temperatures in between. She would also has faint, macular, salmoncolored evanescent rash especially at the height of the fever. What is the most likely diagnosis?

Answer 26

Systemic onset JRA

* Quotidian pattern fever (intermittent fever 1-2x/day ranging from 38-39°C returning to normal temperatures in between)

Question 27

adolescent girl with knee pain aggravated by walking upstairs and on patellar distraction

Answer 27

Patellofemoral syndrome

Question 28

Mainstay of treatment for dermatomyositis

Answer 28

Corticosteroids – Methylprednisolone for more severe cases

Question 29

most commonly elevated on initial presentation in dermatomyositis

Answer 29

Alanine aminotransferase (ALT)