RHEUMATOLOGY

Question 1

Anastasia, 8 years old female, came in for facial rashes crossing the nasolabial fold associated with periorbital edema. You are considering Juvenile dermatomyositis for this patient. What initial evaluation tests will you perform to further establish your impression?

CBC, CPK, ESR, ALT, AST, Aldolase, ANA, LDH
CBC, ALT, AST, ANA, LDH, Aldolase
CBC, CPK, ALT, AST, LDH, Aldolase, ANA
CBC, CPK, ALT, AST, Creatinine, Albumin, ANA, LDH

Answer 1

CBC, CPK, ALT, AST, LDH, Aldolase, ANA

Question 2

Behcet disease is an autoinflammatory disease with recurrent oral ulcerations, uveitis and skin abnormalities. Of the manifestations mentioned which is the most frequent initial symptom:

Uveitis
Painful oral ulcer
Genital scars
Erythema nodosum

Answer 2

Painful oral ulcer

Question 3

Desiree, 3 years old female complained of joint pains specifically on the knee with easy fatigability during preschool activities which was noted about 2 months ago. Her mother thought that this was initially due to her increased daily activities. However she later developed fever with rash which prompted them to eventually seek consult. When you saw her, physical examination further revealed involvement of 4 joints presenting with tenderness, swelling and limited range of motion. Rashes were non-pruritic, linear and are distributed over the trunk and proximal extremities. Patient also presented with hepatosplenomegaly and lymphadenopathy. Based on the history and physical examination of the patient what will be your initial assessment?

Systemic lupus erythematosus
Juvenile dermatomyositis
Acute rheumatic fever
Juvenile idiopathic arthritis

Answer 3

Juvenile idiopathic arthritis

Question 4

This is a stronger predictor of the presence of rheumatic diseases and a reason for referral to a Pediatric Rheumatologist.

Weakness
Arthralgia
Arthritis
Malar rash

Answer 4

Arthritis

Question 5

JP, 4 year old male was admitted for high grade fever (Tmax >39C) for 4 days, unresponsive to antipyretics, his mother described him to be flushed looking, with reddish eyes. Upon physical examination he had bilateral nonexudative conjunctival injection, unilateral lymphadenopathy, strawberry tongue and edematous hands. You are considering Kawasaki disease for this patient. 2d echo was requested to check for cardiac involvement; What condition occurs during acute Kawasaki disease?

Myocarditis
Myocardial infarction
Angina
Thrombosis

Answer 5

Myocarditis

Question 6

This NDMARD is associated with severe systemic hypersensitivity and Stevens-Johnson Syndrome:

Hydroxychloroquine
Methotrexate
Leflunomide
Sulfasalazine

Answer 6

Sulfasalazine

Question 7

Based on the 2017 AHA definition for giant coronary aneurysms, a z score of ≥10 or an absolute dimension of ≥8mm is associated with:

Angina and myocardial infarction
Pericardial effusion
Myocarditis
Diminished left ventricular systolic function

Answer 7

Angina and myocardial infarction

Question 8

A biologic agent that is a used as a treatment for B-cell non-Hodgkin lymphoma and is FDA approved for use in Adult RA and idiopathic thrombocytopenic purpura but does not have a pediatric indication.

Rituximab
Infliximab
Belimumab
Golimumab

Answer 8

Rituximab

Question 9

Non-steroidal anti-inflammatory drugs (NSAIDs) are prescribed to decrease both the pain and the acute and chronic inflammation associated with arthritis, pleuritis, pericarditis, uveitis and cutaneous vasculitis. The said mechanism of action of this drug is to:

a. Soluble, fully human fusion protein of the extracellular domain of CTLA-4. Acts as a co-stimulatory signal inhibitor by binding competitively to CD80 or CD86 where it selectively inhibits T-cell activation.
b. Chimeric human/mouse monoclonal antibody that binds to soluble TNF-α and its membrane bound precursor, neutralizing its action
c. Inhibition of folate dependent processes by MTX polyglutamates primarily their effect on the enzyme 5-aminoimidazole-4-carboxamide ribonucleotide (AICAR) transformylase
d. Inhibiting the enzyme cyclooxygenase (COX) which is critical in the production of prostaglandins.

Answer 9

Inhibiting the enzyme cyclooxygenase (COX) which is critical in the production of prostaglandins.

Question 10

For a criteria of Juvenile systemic sclerosis to be fulfilled it needs to have at least:

3 minor criteria
1 major and 2 minor criteria
2 major criteria
2 major and 1 minor criteria

Answer 10

1 major and 2 minor criteria

Question 11

It is the virus associated with post infectious arthritis that involves large joints especially the knees

Hepatitis A
Rubella
Mumps
Hepatitis B

Answer 11

Mumps

Question 12

This Non-biologic Disease Modifying Anti-rheumatic Drug (NDMARDs) used primarily for treatment of lupus, uveitis and autoimmune skin manifestations.

Leflunomide
Methotrexate
Mycophenolate mofetil
Sulfasalazine

Answer 12

Mycophenolate mofetil

Question 13

What is the current gold standard for diagnosis for Sjorgen syndrome?

Detection of SSA and SSB antibodies
Biopsy of parotid glands
(+) Rheumatoid factor
Hypergammaglobulinemia

Answer 13

Biopsy of parotid glands

Question 14

This drug is used as a secondary disease modifying agent to reduce rash and maintain remission for those with Juvenile dermatomyositis.

Intravenous gamma globulin
Methotrexate
Methylprednisolone
Hydroxychloroquine

Answer 14

Hydroxychloroquine

Question 15

This imaging tool helps localize areas of abnormality in the patient with diffuse pains caused by osteomyelitis, neuroblastoma, chronic multifocal osteomyelitis, systemic arthritis:

Plain radiographs
Radionucleotide bone scans
Magnetic resonance imaging
CT scan of the bone

Answer 15

Radionucleotide bone scans

Question 16

Which of the following differential diagnosis for Juvenile dermatomyositis that presents with muscle weakness but without a rash

Bartonella infection
Mixed connective tissue disease
Muscular dystrophies
Staphylococcal pyomyositis

Answer 16

Muscular dystrophies

Question 17

This condition is associated with genetic predisposition toward slow drug acetylation and manifestations typically resolve after withdrawal of the offending medication.

Enthesitis related arthritis
Juvenile idiopathic arthritis
Vasculitides
Drug-induced lupus

Answer 17

Drug-induced lupus

Question 18

Long term therapy for patients with coronary abnormalities include(s):

Aspirin (3 - 5 mg/kg/day) orally once daily + Clopidogrel (0.5 mg/kg/day)
Aspirin (30 - 50 mg/kg/day) orally once daily + Clopidogrel (0.5 mg/kg/day)
Aspirin (3 - 5 mg/kg/day) orally once daily + Clopidogrel (1 mg/kg/day)
Aspirin (30 - 50 mg/kg/day) orally once daily + Clopidogrel (1 mg/kg/day)

Answer 18

Aspirin (3 - 5 mg/kg/day) orally once daily + Clopidogrel (1 mg/kg/day)

Question 19

This sign when present can be seen in both rheumatic cause such as Vasculitis or in non- rheumatic conditions such as Meningococcemia:

Purpuric rash
Gottron papules
Heliotrope rash
Malar rash

Answer 19

Purpuric rash

Question 20

For a patient to be considered a Behcet disease based on the ISG criteria, it requires the presence of oral ulcers along with 2 major features such as:

Erythema nodosum and Sclerodactyly
Anterior uveitis and necrotic folliculitis
Venous thrombosis and sclerodactyly
Venous thrombosis and arterial aneurysm

Answer 20

Anterior uveitis and necrotic folliculitis

Question 21

What phase of Kawasaki disease presents with desquamation and thrombocytosis?

Convalescent phase
Subacute phase
Acute febrile phase
Acute clinical phase

Answer 21

Subacute phase

Question 22

Which of the following is the etiology of Juvenile dermatomyositis?

a. Complex and includes genetic predisposition and possibly an infectious trigger.
b. Patients often have family members, especially mothers and sisters, with same condition or other autoimmune diseases.
c. History of infection 3 months before onset is noted (Upper respiratory or Gastrointestinal infection)
d. Infectious agent may be responsible for inducing the aberrant innate immune system attacks such as your Herpes simplex virus type I and Parvovirus B19.

Answer 22

c. History of infection 3 months before onset is noted (Upper respiratory or Gastrointestinal infection)

Question 23

This treatment for Takayasu arteritis is reserved for severe or refractory disease

Cyclophosphamide
Methylprednisolone
Methotrexate
Azathioprine

Answer 23

Cyclophosphamide

Question 24

This clinical feature is described as thickened erythematous and scaly rash that develops over the palms and soles along the flexor tendons.

Shawl sign
Mechanic’s hands
Gottron papules
Coup de sabre lesion

Answer 24

Mechanic’s hands

Question 25

Most common cause of mortality in Systemic lupus erythematosus: Neuropsychiatric disease Infection Atherosclerosis Complications of glomerulonephritis

Answer 25

Atherosclerosis

Question 26

The classic triphase sequence of Raynaud phenomenon is: a. Reflex vasodilation caused by the factors released from the ischemic phase; Initial arterial vasoconstriction resulting in hypoperfusion and pallor; Venous stasis b. Venous stasis; Reflex vasodilation caused by the factors released from the ischemic phase Initial arterial vasoconstriction resulting in hypoperfusion and pallor c. Initial arterial vasoconstriction resulting in hypoperfusion and pallor; Venous stasis; Reflex vasodilation caused by the factors released from the ischemic phase d. Venous stasis; Initial arterial vasoconstriction resulting in hypoperfusion and pallor; Reflex vasodilation caused by the factors released from the ischemic phase

Answer 26

c. Initial arterial vasoconstriction resulting in hypoperfusion and pallor; Venous stasis; Reflex vasodilation caused by the factors released from the ischemic phase

Question 27

What will be your treatment regimen for a patient diagnosed for Systemic lupus erythematosus? a. Use of sunscreen and avoidance of prolonged direct sun exposure, give corticosteroids, check for cardiovascular risk factors (lipid profile, BMI, blood pressure monitoring), intake of Vitamin D b. Use of sunscreen and avoidance of prolonged direct sun exposure, give hydroxychloroquine, check for cardiovascular risk factors, routine immunization c. Use of sunscreen and avoidance of prolonged direct sun exposure, give hydroxychloroquine, check lipid profile d. Give methotrexate, advised avoidance of prolonged direct sun exposure

Answer 27

a. Use of sunscreen and avoidance of prolonged direct sun exposure, give corticosteroids, check for cardiovascular risk factors (lipid profile, BMI, blood pressure monitoring), intake of Vitamin D

Question 28

Most commonly involved branch for Takayasu arteritis includes: Femoral artery Renal artery Pulmonary artery Abdominal artery

Answer 28

Renal artery

Question 29

Which is a complication of uveitis? Retinal vaculitis Cataract Vitreitis Retinal detachment

Answer 29

Retinal detachment

Question 30

Poorer prognosis of Juvenile idiopathic arthritis is related to: Young age of onset with RF seropositivity Presence of anti-cyclic citrullinated peptide antibodies Fever lasting >3 months and increased inflammatory marker Leg length discrepancy

Answer 30

Fever lasting >3 months and increased inflammatory marker

Question 31

It is defined as joint inflammation following urogenital infections: Post-infectious arthritis Ankylosing spondylitis Reactive arthritis Juvenile dermatomyositis

Answer 31

Reactive arthritis

Question 32

In a case of Juvenile idiopathic arthritis, what early radiologic findings will you expect to find? Peripheral joints may exhibit periarticular osteoporosis Soft tissue swelling with periarticular osteopenia Loss of cartilage and degrees of bony destruction and fusio Indistinct margins and erosions that can result in joint space widening

Answer 32

Soft tissue swelling with periarticular osteopenia

Question 33

2d-echo is the most useful test to monitor for coronary artery abnormalities, based on the AHA z-score Classification system for aneurysms, a small aneurysm is considered if it has ≥3.5 to < 5 mm >1.5 to 5 mm >3 to < 5 mm ≥2.5 to < 5 mm

Answer 33

≥2.5 to < 5 mm

Question 34

Lisa, 15 years old female complained of joint pains (hands, knees) usually with morning stiffness, she also noted easy fatigability during her extracurricular activities in school. Condition was eventually associated with fever and the occurrence of facial rash most noted after prolonged outdoor daytime activities. She was eventually admitted and further evaluation with laboratory tests revealed anemia, thrombocytopenia by CBC, Proteinuria on urinalysis, and a positive ANA test since you were already considering a rheumatic condition for this case. With the above history, clinical manifestations and laboratory results, what will be your working diagnosis? Juvenile idiopathic arthritis Systemic lupus erythematosus Acute rheumatic fever Post streptococcal glomerulonephritis

Answer 34

Systemic lupus erythematosus

Question 35

This manifestation of Pediatric scleroderma is confined to the dermis, occasionally superficial panniculus; well-circumscribed, circular area of induration, often a central waxy, ivory-colored area surrounded by a violaceous halo. Bullous morphea Linear scleroderma Plaque morphea Deep morphea

Answer 35

Plaque morphea

Question 36

This is considered as a Major criteria for Familial Mediterranean Fever (FMF): Incomplete attacks involving chest pain Incomplete attacks involving monoarthritis Favorable response to colchicine Incomplete abdominal attack

Answer 36

Incomplete abdominal attack

Question 37

This drug has been used for Juvenile dermatomyositis, Lupus-associated thrombocytopenia, Polyarticular JIA, with a dose of 1 - 2 g/kg/dose administered once a month: Intravenous immunoglobulin anakinra cyclophosphamides canakinumab

Answer 37

Intravenous immunoglobulin

Question 38

Andrew a 10 year old male came in for consult due to swelling of the parotid area for almost a week. Other associated signs and symptoms include dry eyes, fever and lower extremity weakness. You were entertaining Sjorgen syndrome. Laboratory tests revealed elevated ESR, leukopenia on CBC and elevated serum amylase. Based on the Proposed criteria for Sjorgen syndrome; How many needed criterion must be fulfilled for a condition to be considered as such? ≤4 criteria ≥4 criteria 4 - 5 criteria < 4 criteria

Answer 38

≥4 criteria

Question 39

Juvenile scleroderma encompasses a range of conditions unified by the presence of fibrosis of the skin. This is believed to be a key process in the pathogenesis of both localized and systemic scleroderma. fibrosis Subclinical graft vs host reaction autoimmunity vasculopathy

Answer 39

autoimmunity

Question 40

What is the most feared complication in Neonatal lupus? Congenital heart block Hepatitis Hydrops fetalis Valvular dysfunction

Answer 40

Congenital heart block

Question 41

Common but nonspecific laboratory findings for HSP includes: Leukocytosis, anemia, normal platelet count Leukopenia, normal hemoglobin, thrombocytosis Leukocytosis, anemia, thrombocytopenia Normal WBC, anemia, thrombocytopenia

Answer 41

Leukocytosis, anemia, normal platelet count

Question 42

It is the complication noted in children with reactive arthritis: Arthritis-dermatitis syndrome cardiomyopathy encephalomyelitis uveitis

Answer 42

uveitis

Question 43

This is the characteristic finding of advanced ankylosing spondylitis and is rare in early disease particularly in childhood. osteoporosis sclerosis Bamboo spine Subchondral erosions

Answer 43

Bamboo spine

Question 44

This TNF - α antagonist is given especially to those patients who failed to respond to methotrexate golimumab adalimumab etanercept infliximab

Answer 44

etanercept

Question 45

This virus associated arthropathy occurs in young women that presents with arthralgia of the knees and hands usually begins within 7 days of onset of the rash or 10-28 days after immunization: Rubella associated arthropathy Hepatitis B arthritis-dermatitis syndrome Varicella associated arthropathy Parvovirus B19 arthropathy

Answer 45

Rubella associated arthropathy

Question 46

Gottron papules when present plus the associated heliotrope rash is suggestive for this rheumatic condition: Juvenile dermatomyositis Psoriatic arthritis Systemic lupus erythematosus Juvenile idiopathic arthritis

Answer 46

Juvenile dermatomyositis

Question 47

This specific spondyloarthritis presents with disease the arthritis is typically asymmetric and involves ≤4 joints most frequently the knees, ankles and hips in the first 6 months with inflammation of the small joints of the foot or tarsitis. Enthesitis-related Arthritis (ERA) Juvenile idiopathic arthritis Psoriatic arthritis Ankylosing spondylitis

Answer 47

Enthesitis-related Arthritis (ERA)

Question 48

This is the most common recurrent fever in children with fever episodes that last 4-6 days regardless of antipyretic or antibiotic treatment, associated with mild leukocytosis and elevated acute phase reactants: Familial Mediterranean Fever (FMF) Periodic Fever, Aphtous Stomatitis, Pharyngitis and Adenitis (PFAPA) Hyperimmunoglobulinemia D with Periodic Fever Syndrome (HIDS) Tumor Necrosis Factor Receptor-Associated Periodic Syndrome

Answer 48

Periodic Fever, Aphtous Stomatitis, Pharyngitis and Adenitis (PFAPA)

Question 49

The Hallmark of HSP is the presence of a palpable purpuric rash, with symmetric skin lesions and occurs in this area: On the trunk area Shoulder portion of the upper extremities Lesions on the periorbital area Extensor aspect of the upper extremities

Answer 49

Extensor aspect of the upper extremities

Question 50

Which of the following describes Henoch - Schonlein Purpura (HSP): It is the most common medium vessel vasculitis It is characterized by leukocytoclastic vasculitis Many cases follows a documented gastrointestinal infection Affects females more than males

Answer 50

It is characterized by leukocytoclastic vasculitis

Question 51

One of the following is associated with increased risk of developing chronic uveitis among children with JRA? a. ANA titer b. Rheumatoid factor c. Elevated ESR, CRP d. Hypergammaglobulinemia

Answer 51

a. ANA titer

Question 52

One of the following laboratory parameters is specific for SLE in children: a. ANA b. Anti-dsDNA c. ANti-Smith antibody d. C-reactive proteins

Answer 52

c. ANti-Smith antibody

Question 53

Shawl sign is seen in which of the following rheumatic diseases of childhood? a. Juvenile dermatomyositis b. Henoch-Schِnlein purpura c. Systemic lupus erythematosus d. Systemic-onset juvenile idiopathic arthritis

Answer 53

a. Juvenile dermatomyositis

Question 54

Rita, an 11-year-old female with history of fatigue, malaise, and progressive muscle weakness, accompanied by low-grade fevers and scaly, red plaques are found across the knuckles, she is not able to sit up from a supine position. Laboratory exams showed elevated muscle enzymes (ALT, AST, LDH, CPK, and aldolase), EMG abnormalities: fasciculations, needle insertion irritability, and high-frequency discharges. What do you think is the complication for this disease? a. calcinosis b. infections c. avascular necrosis d. myocardial infarction

Answer 54

a. calcinosis

Question 55

One of the following is **not** a minor criteria in the diagnosis of Juvenile Systemic Sclerosis: a. Hepatomegaly b. Right sided heart failure c. Gastroesophageal reflux d. Pulmonary hypertension

Answer 55

a. Hepatomegaly

Question 56

The rheumatic diseases of childhood are characterized by autoimmunity and inflammation, which may be localized or generalized. Which of the following is the classical rheumatic disease of childhood : a. Scleroderma b. Bechet syndrome c. Juvenile rheumatoid arthritis d. Sjogren syndrome

Answer 56

c. Juvenile rheumatoid arthritis

Question 57

What is the common visceral manifestation of patients with scleroderma? a. Dyspnea b. Dysphagia c. Failure to thrive d. Raynaud phenomenon

Answer 57

a. Dyspnea

Question 58

This is the most common radiologic finding in the early stages of juvenile idiopathic arthritis (JIA): a. fusion of C1-4 b. erosions of bony articular surfaces c. normal bone x-ray d. evidence of bony proliferation e. periarticular osteopenia

Answer 58

e. periarticular osteopenia

Question 59

Liz, an 8-year-old-girl complains from morning stiffness and pain of 4 joints in the last 6 months with uveitis. On physical examination, signs of inflammation are present, including joint tenderness, erythema, and effusion, but no fever or weight loss. Laboratory result was: ANA positive, rheumatoid factor negative. Of the following, the MOST commonly involved joint is a. hip b. ankle c. knee d. wrist

Answer 59

a. hip

Question 60

Most of the children with spondyloarthritis will have the following laboratory findings EXCEPT: a. C-reactive protein b. Antinuclear Antibodies c. Human Luekocyte Antigen d. Erythrocyte sedimentation rate

Answer 60

b. Antinuclear Antibodies

Question 61

Erosive arthritis in the following pediatric arthritis syndromes, is mostly seen in which of the following? a. Kawasaki disease b. Juvenile dermatomyositis c. Juvenile idiopathic arthritis d. Systemic lupus erythematosus

Answer 61

c. Juvenile idiopathic arthritis chatgpt

Question 62

This is the most common serious cardiac complication of neonatal lupus? a. heart block b. Cardiac tamponade c. Coronary aneurysm d. Myocardial Infarction

Answer 62

a. heart block

Question 63

Anti-neutrophil cytoplasmic antibody-associated vasculitis is seen in all the following EXCEPT… a. Wegener granulomatosis b. Polyarteritis nodosa c. Churg-Strauss syndrome d. Henoch-Schnِlein purpura

Answer 63

b. Polyarteritis nodosa

Question 64

The treatment of juvenile idiopathic arthritis (JIA) focuses on suppressing inflammation, preserving and maximizing function, preventing deformity, and preventing blindness. Of the following, what is the initial option in the treatment of JIA? a. infliximab b. hydroxychloroquine c. systemic corticosteroid d. nonsteroidal antiinflammatory drug

Answer 64

d. nonsteroidal antiinflammatory drug

Question 65

The characteristic skin lesion of Juvenile dermatomyositis on the periorbital area. a. Malar rash b. Gottron's rash c. Heliotrope rash d. Livedo reticularis

Answer 65

c. Heliotrope rash

Question 66

An initial presentation of a primary variable vessel vasculitis is : a. oral ulcer b. genital scar c. anterior uveitis d. erythema nodosum

Answer 66

a. oral ulcer

Question 67

Fever in rheumatic diseases is caused by cytokine release and can take many forms. Regular temperature spikes, once or twice a day is seen in patients with: a. Scleroderma b. Behçet syndrome c. Systemic-onset juvenile rheumatoid arthritis d. Sjogren syndrome

Answer 67

c. Systemic-onset juvenile rheumatoid arthritis

Question 68

Based on the ACR criteria for JRA, the duration of arthritis should be >/= ______ weeks? a. 2 b. 4 c. 6 d. 8

Answer 68

c. 6