Rheumatology

Question 1

A 4 year old female patient presents to the clinic with 5 day history of fever with temperatures between 38-39C, there was bilateral redness of the eyes with no discharge, decreased appetite and intake, physical exam revealed 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>38.8C, what is the most likely diagnosis?
A.  Rubeola
B. SJS
C. Ritter disease
D. Kawasaki
E. Scarlet fever

Answer 1

D. Kawasaki

Question 2

In a patient with known Kawasaki disease, which of the illness gives the highest risk for sudden death?
A. Prodromal
B. Acute febrile
C. Subacute
D. Convalescence

Answer 2

C. Subacute

Question 3

Which of the ff is the preferred treatment of Kawasaki in the acute stage of the disease?
A. IV Ig
B. Low dose aspirin
C. Warfarin
D. Both A and B

Answer 3

D. Both A and B

Search if low dose or high dose

Question 4

Kawasaki Disease: FEVER + 4 of the ff features:

Clue: C-R-A-S-H

Answer 4

Conjunctivitis
Rash
Adenopathy
Strawberry tongue and other oropharyngeal changes
Hand changes (brawny edema, induration, peeling around nail beds)

Question 5

Treatment of kawasaki disease in the acute stage

Answer 5

Acute stage: IV Ig (2g/kg) and Aspirin (80-100mg/kg/day) q6h until afebrile

Convalescent: Aspirin 3-5mg/kg OD

Long term therapy w/ coronary abnormalities: Aspirin & Clopidogrel

Question 6

8 year old female presents with 3 month history of fever ranging from 37.8 to 38.5C, weight loss, irritability, and joint pains. She also has some muscle weakness and has difficulty climbing the stairs and combing her hair sometimes. She also started having a violaceous erythematous rash near the periorbital area that extends to the nose, current PE: HR 90, RR18, T>37.5C, characteristic rash over the face, (+) muscle weakness 4/5 over biceps and hips, (+) reddish pink colored skin over the dorsal surfaces of the metacarpal joints, what is the most likely diagnosis?
A. dermatomyositis
C. photosensitive dermatitis
D. vitamin C deficiency
E. allergic contact dermatitis

Answer 6

A. dermatomyositis -most common pediatric myopathy

Question 7

Systemic vasulopathy with characteristic cutaneous findings and focal areas of myositis resulting in progressive proximal muscle weakness.

Answer 7

Juvenile Dermatomyositis

Gottron Papules- alligator skin appearance
Heliotrope eyelids/rash- periorbital violaceous erythema that may cross over the bridge of the nose

Question 8

Clinical manifestation of Juvenile Dermatomyositis:

Answer 8

• Neck flexor and abdominal muscle weakness
• unable to sit up or head lag during infancy, and Gower sign
• derangement of upper airway function
• dysphagia (severe prognostic sign)
• constipation, abdominal pain, diarrhea
• dilated cardiomyopathy

Question 9

What is not a diagnostic criteria for juvenile dermatomyositis?
A. Heliotrope rash
B. Asymmetric weakness
C. Muscle enzyme elevation
D. Myopathy
E. None of the above

Answer 9

B. Asymmetric weakness

Diagnostic criteria:
Classic rash (heliotrope rash of the eyelids, Gottron papules) PLUS 3 of the ff:
1. Weakness (symmetric, proximal)
2. Muscle enzyme elevation:
CK, aspartate, aminotransferase (most commonly elevated), LDH, aldolase
3. Electromyographic changes (myopathy, denervation)
4. Muscle biopsy (necorsis, inflammation)

Question 10

TRUE or FALSE. IVIG is given for severe cases in Juvenile Dermatomyositis

Answer 10

TRUE

Corticosteroids also given: methylprednisolone for more severe cases

Methotrexate decreases the length of treatment with steroids

Question 11

A 7 year old male  presents to the clinic with a rash extending from the buttocks to the lower extremities. The rash is characterized as raised pinkish to erythematous purpuric rashes. The mother recalled that he had just gotten well from flu 1 week ago. He also has right knee pain with slight swelling of the right knee but not warm and non erythematous. He is presently afebrile. Urinalysis reveals 6-8RBC, protein +2, WBC 0-2, glucose (-), bacteria +1. What is
the most likely diagnosis in this case?
A. hemolytic uremic syndrome
B. Henoch schonlein purpura
C. dengue fever
D. meningococcemia

Answer 11

B. Henoch schonlein purpura

Question 12

What is the pathophysiologic mechanism involved in Henoch-Schonlein Purpura?
A. deposition of IgA and immune complexes
B. microvascular thrombi leading to hemolysis
C. immune reaction against platelet with increased vascular permeability
D. endotoxin release resulting to DIC and shock

Answer 12

A. deposition of IgA and immune complexes

Question 13

What vessels are commonly affected in HSP?
A. coronary and other muscular arteries
B. large arteries 
C. medium arteries
D.small arteries

Answer 13

D.small arteries (dermal capillaries and postcapillary venules)

Large arteries- Takayasu
Medium arteries- Kawasaki

Question 14

What is the best approach to treatment for HSP?
A. supportive therapy
B. mycophenolate or cyclophosphamide
C. oral prednisone
D. plasmapheresis and IV Ig

Answer 14

A. supportive therapy

Question 15

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 reveals: joint swelling with limitation of range of motion of the knees and ankles, no other complaints noted, HR
90, RR 20, T>37.2C, what is the most likely the diagnosis?
A. systemic onset JRA
B. pauciarticular JRA
C. polyarticular JRA
D. juvenile spondyloarthropathy

Answer 15

B. pauciarticular JRA

Question 16

Which of the ff. statements pertain to Pauciarticular JRA
A. the disease is usually associated with RF positive
B. the disease is usually associated with ANA(+)
C. it is the most aggressive or disabling form
D. it is the least cornmon type

Answer 16

B. the disease is usually associated with ANA(+)

Question 17

A 13 y/o female patient presents to the clinic with complaints of fever, rash and joint pains. It started 2 mons ago with pain and swelling over her knees and sometim her ankles. She has also been having intermittent fevers 3x/day ranging from 38-39C returning to normal temperatures in between. She would also have faint, erythematous macular salmon colored evanescent rash especially at the height of the fever, what is the most likely diagnosis
A. systemic onset JRA
B. pauciarticular JRA
C. polyarticular JRA
D. rheumatic fever

Answer 17

A. systemic onset JRA

Question 18

Study Classification of Childhood Chronic Arthritis ^_^

TABLE!

Answer 18

Topnotch p. 61 :)

Question 19

An 18 year-old female complains of fatigue, muscle and joint aches, and fever that have lasted for 2 months. On PE, she had rashes over her cheeks and nose as well as friction rub on cardiac auscultation. Lab findings: Hgb 10 g/dL, Hct 33%, platelet count of 145,00/mm3, WBC 4,300/mm3, urinalysis 3+ proteinuria. The following is expected in her disease except?
A. Responds to steroids and other immunosuppressant
B. More common in females than males
C. ANA is positive
D. Anti-dsdna reflects disease activity

Answer 19

C. ANA is positive

Question 20

Criteria for Diagnosis of SLE

“SOAP BRAIN MD”

Answer 20

4/11:

Serositis (Pleuritis, Pericarditis)
Oral ulcer (painless)
Arthritis (2 or more joints)
Photosensitivity
Blood changes (anemia, leukopenia, low platelet)
Renal disorders (persistent proteinuria, cellular cast)
ANA abnormal titer
Immunological changes
Neurological signs
Malar rash
Discoid rash

*(+) ANA result is not required for diagnosis, but its absence is rare
ANA: excellent screening tool but can be (+) in other rheumatic conditions.

*anti Smith Ab found only in SLE (specific for dx)

Question 21

Have you read your reading assignment?

Hala sya. :O

Answer 21

Ankylosing spondylosis
ILAR vs ACR Classification of JIA
Juvenile Scleroderma