RHEUMATOLOGY Flashcards
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
CBC, CPK, ALT, AST, LDH, Aldolase, ANA
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
Painful oral ulcer
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
Juvenile idiopathic arthritis
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
Arthritis
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
Myocarditis
This NDMARD is associated with severe systemic hypersensitivity and Stevens-Johnson Syndrome:
Hydroxychloroquine
Methotrexate
Leflunomide
Sulfasalazine
Sulfasalazine
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
Angina and myocardial infarction
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
Rituximab
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.
Inhibiting the enzyme cyclooxygenase (COX) which is critical in the production of prostaglandins.
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
1 major and 2 minor criteria
It is the virus associated with post infectious arthritis that involves large joints especially the knees
Hepatitis A
Rubella
Mumps
Hepatitis B
Mumps
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
Mycophenolate mofetil
What is the current gold standard for diagnosis for Sjorgen syndrome?
Detection of SSA and SSB antibodies
Biopsy of parotid glands
(+) Rheumatoid factor
Hypergammaglobulinemia
Biopsy of parotid glands
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
Hydroxychloroquine
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
Radionucleotide bone scans
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
Muscular dystrophies
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
Drug-induced lupus
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)
Aspirin (3 - 5 mg/kg/day) orally once daily + Clopidogrel (1 mg/kg/day)
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
Purpuric rash
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
Anterior uveitis and necrotic folliculitis
What phase of Kawasaki disease presents with desquamation and thrombocytosis?
Convalescent phase
Subacute phase
Acute febrile phase
Acute clinical phase
Subacute phase
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.
c. History of infection 3 months before onset is noted (Upper respiratory or Gastrointestinal infection)
This treatment for Takayasu arteritis is reserved for severe or refractory disease
Cyclophosphamide
Methylprednisolone
Methotrexate
Azathioprine
Cyclophosphamide
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
Mechanic’s hands
Most common cause of mortality in Systemic lupus erythematosus:
Neuropsychiatric disease
Infection
Atherosclerosis
Complications of glomerulonephritis
Atherosclerosis
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
c. Initial arterial vasoconstriction resulting in hypoperfusion and pallor; Venous stasis; Reflex vasodilation caused by the factors released from the ischemic phase
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
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
Most commonly involved branch for Takayasu arteritis includes:
Femoral artery
Renal artery
Pulmonary artery
Abdominal artery
Renal artery
Which is a complication of uveitis?
Retinal vaculitis
Cataract
Vitreitis
Retinal detachment
Retinal detachment
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
Fever lasting >3 months and increased inflammatory marker
It is defined as joint inflammation following urogenital infections:
Post-infectious arthritis
Ankylosing spondylitis
Reactive arthritis
Juvenile dermatomyositis
Reactive arthritis
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
Soft tissue swelling with periarticular osteopenia
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
≥2.5 to < 5 mm
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
Systemic lupus erythematosus
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
Plaque morphea
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
Incomplete abdominal attack
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
Intravenous immunoglobulin
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
≥4 criteria
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
autoimmunity
What is the most feared complication in Neonatal lupus?
Congenital heart block
Hepatitis
Hydrops fetalis
Valvular dysfunction
Congenital heart block
Common but nonspecific laboratory findings for HSP includes:
Leukocytosis, anemia, normal platelet count
Leukopenia, normal hemoglobin, thrombocytosis
Leukocytosis, anemia, thrombocytopenia
Normal WBC, anemia, thrombocytopenia
Leukocytosis, anemia, normal platelet count
It is the complication noted in children with reactive arthritis:
Arthritis-dermatitis syndrome
cardiomyopathy
encephalomyelitis
uveitis
uveitis
This is the characteristic finding of advanced ankylosing spondylitis and is rare in early disease particularly in childhood.
osteoporosis
sclerosis
Bamboo spine
Subchondral erosions
Bamboo spine
This TNF - α antagonist is given especially to those patients who failed to respond to methotrexate
golimumab
adalimumab
etanercept
infliximab
etanercept
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
Rubella associated arthropathy
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
Juvenile dermatomyositis
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
Enthesitis-related Arthritis (ERA)
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
Periodic Fever, Aphtous Stomatitis, Pharyngitis and Adenitis (PFAPA)
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
Extensor aspect of the upper extremities
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
It is characterized by leukocytoclastic vasculitis
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
a. ANA titer
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
c. ANti-Smith antibody
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
a. Juvenile dermatomyositis
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
a. calcinosis
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
a. Hepatomegaly
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
c. Juvenile rheumatoid arthritis
What is the common visceral manifestation of patients with
scleroderma?
a. Dyspnea
b. Dysphagia
c. Failure to thrive
d. Raynaud phenomenon
a. Dyspnea
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
e. periarticular osteopenia
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
a. hip
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
b. Antinuclear Antibodies
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
c. Juvenile idiopathic arthritis
chatgpt
This is the most common serious
cardiac complication of neonatal
lupus?
a. heart block
b. Cardiac tamponade
c. Coronary aneurysm
d. Myocardial Infarction
a. heart block
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
b. Polyarteritis nodosa
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
d. nonsteroidal antiinflammatory drug
The characteristic skin lesion of Juvenile dermatomyositis on the periorbital area.
a. Malar rash
b. Gottron’s rash
c. Heliotrope rash
d. Livedo reticularis
c. Heliotrope rash
An initial presentation of a primary variable vessel vasculitis
is :
a. oral ulcer
b. genital scar
c. anterior uveitis
d. erythema nodosum
a. oral ulcer
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
c. Systemic-onset juvenile rheumatoid arthritis
Based on the ACR criteria for JRA,
the duration of arthritis should
be >/= ______ weeks?
a. 2
b. 4
c. 6
d. 8
c. 6
