Rheumatology
Childhood Rheumatic Diseases Quiz
Test your knowledge on rheumatology, particularly in pediatric care. This quiz covers various aspects of juvenile idiopathic arthritis and related disorders, making it ideal for healthcare professionals and students.
- Assess your understanding of common childhood rheumatic diseases.
- Learn about symptoms, diagnosis, and treatment approaches.
What is the most common rheumatic disease in childhood?
Juvenile dermatomyositis
Juvenile systemic lupus erythematodes
Transient postinfectious arthritis
The juvenile idiopathic arthritis
Juvenile dermatomyositis and transient post-infedctious arthritis
Why do pediatric rheumatologists believe the true prevalence of juvenile idiopathic arthritis may be higher than we know?
Population based studies resulted in much higher prevalence numbers than reported from pediatric rheumatology clinics
Patients may not recognize that they have a joint disease
Parents may not recognize their child has a joint disease
Family doctors may not be aware of the fact that children can get rheumatic disease
Patients may not recognize that they have a joint disease and their child has a joint disease
What is NOT true about distribution of rheumatic diseases around the world:
Children from Africa have more lupus than children from Europe
The children from America have more JIA than children from Europe
Children from Europe have more JIA than children from Asia
Children from Asia have more lupus than children from Europe
All answers are not correct
Which symptom is useful to distiguish between mechanical and inflammatory joint pain
Swelling
Pain when moving
The morning stiffness
Limping in the evening
Swelling and pain when moving
Which symptom is useful to distinguish between mechanical and inflammatory joint pain
Functional limitation
Night pain
Joint effusion
The joint blocade
Morning pain
Which information is NOT important in the family history for a suspected rheumatic disease
A history of inflammatory bowel disease in close relative
The exact birth dates of the parents
A history of psoriasis in a sibling
A history of spondylarthritis in the father
A history of psoriasis in a sibling and a history of spondyloarthritis in the father
Which of the following autoimmune diseases does NOT share a genetic susceptibility factor with juvenile idiopathic arthritis?
Rheumatoid arthritis
Coeliac disease
Autoimmune thyroiditis
The multiple sclerosis
Rheumatoid arthritis and coeliac disease
Which are the earliest changes of the joint structure in acute arthritis
Hypertrophy of the synovial lining
Swelling of the capsular ligament
Erosion of the ajacent bone
Subcutanous edema
All answers are not correct
According tot he ACR criteria arthritis is defined by
Joint tenderness
Painful limitation of the range of motion
Combination of tendernes and painful limitation of the motions
Limping
Joint tenderness and limping
The rheumatologic screening examination pGALS is
An easy and quick tool to detect musculoskeletal problems in the children
A diagnostic tool for juvenile idiopathic arthritis
A tool that can only be used by pediatric rheumatologists
An online questionnaire for children with rheumatic diseases
All answers are not correct
The following global tests are helpful to assess joint function in children, except
Squatting test
Head scratch tests
Stretching test
Gait
Watching test
Watching a child limping may be helpful
To distinguish between a traumatic and an inflammatory disease
To find out which joint is the affected joint causing the limps
To test whether the parents are telling truth
Because children do not like to sit still
All answers are not correct
Children with a hip problem will
Walk with their knees bend to avoid pain
Walk with foot on affected side rotated externally to avoid pain
Walk with foot on affected side rotated internally to avoid pain
Sway their upper body over the affected hip to avoid pains
Normal walk
What is a typical finding in a young child with chronic arthritis of one knee:
A shorter leg on the affected side
Muscle hypertrophy on the affected side
A longer leg with muscle atrophy on the affected sides
A shorter leg with muscle atrophy on affected side
A shorter hand on the affected side
What is enthesitis?
Inflammation at the site where a tendon or ligament is attached to the bones
Bone destruction at site where a tendon or ligament is attached to bone
Destruction of a tendon or a ligament during course of spondyloarthritis
A manifestation of spondyloarthritis primarily seen in adult patients
Inflamamtion of hip
The neutral-zero method:
Is a specific neutralisation method
Is a way to record the joint range of movement by adding the neutral position as 0
Is a method to find neutral position of a joint
Is a complicated method to measure the joint mobility
Is a method of mesuring of flexion of the movement
The Schober test is used:
To assess the lumbar spine mobilities
To assess cervical spine mobility
To assess general flexibility of an individual
To assess sacroiliac joint mobility
To assess genenral extension lower back
For the assessment of the ankle (tibio-talar) joint mobility it is important to:
Fixate heel with one hand
Stretch knee completely
The bend the knee to about 90°
Bend hip to about 90°
Movement dorsi extension of foot
To test the mobility of the subtalar (talo-calcaneal) joint
Move the heel into a pronation – supination positions
Hold heel firmly and move forefoot into a pronation - supination position
Hold malleoli and move foot into a pronation - supination position
Hold tibia above malleoli and move foot into a pronation-supination position
Movment plantarflexion of foot
Joint puncture is NOT useful for
Diagnosis of a septic arthritis
Pain relief in large effusions
Treatment with intraarticular steroid
The diagnosis of polyarthritis
Diagnosis of meningitis
Choose the best answer: What can cause inflammation?
Infection
Insect sting
Bone fracture
All answers are correct
Infection, insect sting
What is true about the innate immune system?
It develops during first 2-3 years of life
It provides protection against pathogens encountered earlier in life
It is an unspecific « first line » reaction to proteins of the bacterial cell walls
It has a memory and learning function
It can produce antibody
What is true about the adaptive immune system?
It provides specific protection against pathogens encountered earlier in the life
It unspecifically reacts to contact with bacterial cell wall protein
It is an inborn protective mechanism
It is a powerful, unspecific first line defense mechanism
All answers are not correct
Granulocytes
Can not produce cytokine
Produce antibody
Can die when they phagocyte the pathogen and the form pus
Have a memory function
Diried from macrophage
B-cells
Can not produce cytokine
The produce antibodies
Can die when they phagocyte pathogen and form pus
Are the cells controlling the immune response
Produced in thymus
The major histocompatibility complex (MHC)
Is displayed on surface of every cell of our body
Is an inherited pattern of the cell surface antibodies
Is not relevant for organ transplantation
Is part of bloodgroup antibody
Is killed by bacteria
Antigen presenting cells
Present bacterial antigens in their MHC complexes
Is not found in lymphocyte
Need granulocytes for activation
May develop from reticulocyte
Can kill the bacteria
Antibodies
Are produced by T lymphocyte
Are produced by macrophage
Are produced by granulocyte
Protect the body against pathogens encountered earlier in the life
Have perminant in blood
A specific antibody titre
Rises after every new encounter with the antigens
Is stable for decades after original activation
Depends on granulocyte function
Depends on CRP level
Depends on number of antigen
A superantigen
Is a highly protective protein of immune response
Can activate the T cells without co stimulations
Can downregulate immune response
Is an important player to keep immune system in balance
Can be actived by B cell
Acute Rheumatic Fever
Is most common autoimmune disease in childhood
Can cause severe joint destruction if untreated
Is caused by the cross-reactive antibodies against streptococcis
Is always accompanied by a typical rash
Is an acute febrile monoarthritis
Acute rheumatic fever
May occur after any streptococcal infection
Often occurs together with poststreptococcal glomerulonephritis
Occurs after the throat infection with certain strains of Group A hemolytic streptococcis
May occasionally occur after staphyloderma infection, too
May occur just 2 days after throat infection
Chorea
Is the most common extracardial manifestation of acute rheumatic fever
Usually manifests several months after the triggering throat infections
Causes permanent brain damage
Requires evidence of a recent throat infection
Is abnormal and involuntary movement happening during sleep
Which of the following is NOT a major criterium for acute rheumatic fever according tot he modified Jones criteria:
The fever
Chorea
Polyarthritis
Subcutanous nodules
High CRP
Together with evidence of a recent streptococcal infection, which combination of symptoms will fulfill the modified Jones criteria for acute rheumatic fever?
Myocarditis, prolonged PR interval and fever
Myalgia, fever and polyarthritis
Chorea, weight loss and elevated ESR
Carditis, subcutanous nodules and arthralgias
Carditis, dysuria
Carditis of acute rheumatic fever
Is always present at onset
Is a self limited disease
Can cause rapid onset acute cardiac failures
Will always heal without sequelae
Can casuse slow onset acute cardiac failure
Second attacks of rheumatic fever
Are very rare
Are especially unlikely in young children and those with cardiac involvement
Can be prevented with the prophylactic antibiotic treatments
Are nothing to worry about
Are life threatening disease
Juvenile idiopathic arthritis
Has its peak of onset in adolescence
Is an umbrella term for different subtypes of the arthritis
Will always disappear before adulthood
Does not cause joint damage
Often seen in old adult
Oligoarticular juvenile idiopathic arthritis
May have the persistant or the extended courses
Is not associated with extraarticular manifestation
Always causes severe joint damage
Is a rare subtype of JIA
Always associated with anterior uveitis
Polyarticular, Rheumatoid factor negative JIA
Onset form of Rheumatoid Arthritis
Never causes joint damage
May present with daily high spiking fever
It may be associated with the anterior uveitis
Never needs medical intervention
Arthritis is
A painful joint
A joint with painful limited motion
A joint with tenderness
A joint with tenderness and the painful limited motion
A joint with sensation of heat
Juvenile idiopathic arthritis is defined as
Arthritis of unknown origin in a child <15 years of age
Any arthritis in a child <5 years of age
Arthritis of > 6weeks duration, of unknown origin and with onset <16 years of age
Any arthritis with onset <1 years of age
Any arthritis in a child between 5 to 14 years of age
Systemic juvenile idiopathic arthritis
Requires daily high spiking fever for the diagnosis
Rarely comes into remission
Always has very severe joint disease
Is mildest subtype of JIA
Most commonly affects knee
Enthesitis
Is a special form of arthritis
Is an inflammation at the site of the attachement of a ligament or tendon unto the bones
Is a special form of tendinitis
Most commonly affects knee
Does usually not occur in JIA
The following diseases are HLA B27 related, EXCEPT
Ankylosing spondylitis
Psoriasis arthritis
Crohn’s disease
The Still’s diseases
Septic arthritis
Uveitis
Is most common in ANA positive children with young age at onset of the oligoarticular JIA
Is most common in schoolaged children with Rheumatoid factor positive polyarticular JIA
Is most common in schoolaged children with systemic onset JIA
Is uncommon in children with JIA
Rarely leads to joint destructions in JIA
Growth
Is not affected by juvenile idiopathic arthritis
May be locally accelerated in the patients with JIA
Is always disturbed in patients with JIA
Is delayed in patients with oligoarticular JIA
Is not a big concern in patients with JIA
Arthritis of the temporomandibular joint (jaw joint)
Does usually not occur in JIA
Rarely leads to joint destruction in JIA
Often causes significant growth delay of the mandible in JIA
Is not a big concern in patients with JIA
Is a special form of arthritis
The following are important differential diagnoses of juvenile idiopathic arthritis, EXCEPT
Leukemia
Septic arthritis
Tuberculosis
The myositis
Arthritis
Juvenile Dermatomyositis
May start with the only myositis or the only dermatitis
Most common connective tissue disease in children
Will always involve muscles and skin together
Is caused by vasculitis
All answers are not correct
Juvenile Dermatomyositis
Is a lifelong disease
Should be treated aggressively with steroids and methotrexates
Is a benign, self limited disease
Will not cause longterm damage
Is a short disease
Juvenile Dermatomyositis
May lead to severe calcifications of the muscles and skins
Never causes ulcerating skin disease
Easy to recognize because of the typical heliotrope rash
Is not associated with arthritis
All answers are not correct
Gottron’s papules
Is heliotrope rash typical for juvenile dermatomyositis
Typically are of a slightly violet colour
Are located over the extensor surfaces of the finger joints of patients with juvenile dermatomyositis
Are a very unspecific symptom
Typically are of a slighty white colour
Nailfold capillaroskopy
Is technically very difficult to perform
Is not helpful for disease management
Can give insight into the disease activity of juvenile dermatomyositis
Is a tool for diagnosis of juvenile dermatomyositis
Is easy for disease managment
The following items are part of the Bohan criteria for the diagnosis of juvenile dermatomyositis, EXCEPT
Heliotrope rash
Gottron’s papule
Muscle weakness
MRI confirmation of muscular inflammations
Muscle biopsy
Skin disease in juvenile dermatomyositis includes the following features, EXCEPT
Gottron’s papule
The butterfly rash
Periungual edema
Skin ulcers
Vasculopathic ulcer
Muscle disease in juvenile dermatomyositis is characterised by the following features, EXCEPT
Distal muscle weakness
Elevated muscle enzymes
Pathologic electromyography
Signs of muscle inflammation on MRI
Muscle pain
A sign of early active juvenile dermatomyositis is
Calcifications in the skin and muscle
Lypoatrophy
The periungual edema
Normal nailfold capillaroscopy
Muscle weakness
Localized scleroderma
May evolve into systemic sclerosis
Is caused by transplacental autoantibodies transmitted from mother
May develop pulmonary fibrosis
Is also called the Morphea
Cause by infection
Treatment of morphea en coup de sabre
Depends on location
Consists of the high dose steroids and methotrexates
Consists in local skin treatment (cream)
Is rarely necessary
High dose antibiotic
Systemic sclerosis
Is more common in Asian ethnicity
Is most common connective tissue disease in children
May be complicated by the severe pulmonary fibrosis
Typically affects young boy
Injurie to small veins
Morphea en bande
Can cause growth disturbance and functional limitation of the affected limb
Is a circular morphea lesion on a limb
Is always limited to skin
Is usually associated with arthritis
Affect face only
What is NOT true about Morphea en plaque
May involve superficial and deep tissue layers
Usually has a self limited course
Can be treated with local creams unless it is located over a joint
Usually completely resolves
Is all so called localized scleroderma
Mixed connective tissue disease
Is commonly seen in adolescent boy
Typically has secondary Raynaud’s phenomenon as the prominent symptoms
Is the underlying disease of primary Raynaud’s phenomenon
Causes genital ulcer
Is not an autoimmune disease
The most feared complication of systemic sclerosis is
Muscle calcinosis
Raynaud phenomenon
The pulmonary fibrosis
Skin thickening
Encephalitis
Mixed connective tissue disease is characterized by
High titre anti-RNP antibodies
Oral and genital ulcer
Butterfly rash
Severe musckle weakness
Multiple arthritis
Untreated Morphea en coup de sabre may lead to
Permanent disfiguration of a limb
Functional impairment of affected extremity
Total alopecia
Facial hemiatrophies
Skin artrophy
Secondary Raynaud phenomenon
Is a benign condition
Usually affects young, otherwise healthy women
May cause necrosis of the finger tips (rat bite necrosis)
Is not connected to an underlying disease
Is a medical condition in which spasm of vein
Juvenile systemic lupus
Is a disease of young men
Often has a butterfly rash as skin manifestations
Has typical clinical features that can be found in a patient
Has a self limited course
Is an acute disease
Juvenile systemic lupus
Always presents with the typical malar rash (butterfly rash)
Has a heliotrop rash and edematous swelling of the eyelid
Per definition involves several organ systems
May have Gottron’s papules as a typical manifestation
Can affect only one organ
Juvenile systemic lupus
Typically has high titre of ANA and the anti-DNA antibodies
Typically has increased complement levels as a sign of disease activity
Typically has leukocytosis as a sign of disease activity
Typically has thrombocytosis as a sign of disease activity
Typically has low titre of ANA and anti DNA antibody
Juvenile systemic lupus
Is a genetic disorder
Commonly affects young preschool-aged girl
Can cause uveitis in about 15% of patient
Is characterized by multiple auto-antibodies
Can cause uveitis in about 5%
Patients with juvenile systemic lupus
Will usually outgrow their disease once they have reached adult age
Are the typically adolescent girls
Always have polyarthritis
Commonly have leukocytosis
Is typically boy
Patients with juvenile systemic lupus
Are susceptible to infections because they often have low complement levels
Rarely have kidney involvement
Rarely have neurological manifestation
Often have genital ulcer
Rarely have arthritis
Juvenile systemic lupus
Is a disease with decreased antibody production
Is a disease with the high diversity of clinical symptoms
Usually has high CRP
Is a disease that « licks the joints but bites the heart »
Usually have thrombocytopenia
Patients with juvenile systemic lupus
Often have asymptomatic oral ulcers
Often have asymptomatic genital ulcer
Often have very tight and thick skin
Often have very high complement level
Often have affected to one part of the body
Common neurological manifestations of lupus are the following, EXCEPT
Seizure
Organic brain syndrome (hallucinations, cognitive defect)
The bacterial meningitis
Stroke
Encephalitis
Common constitutional symptoms in lupus patients are the following, EXCEPT
Fever
Weight gains
Fatigue
School failure
Pericarditis
Juvenile systemic lupus commonly affects the following organ systems, EXCEPT
Lymphoid tissue
Kidney
Joint
Lungs
Heart
Discoid lupus skin lesions
Are common in children
Will heal with the leaving a scars
Are a photosensitivity reaction
Will heal without any scar
Uncontrole inflammation by drug
Headache in patients with lupus
Are the very frequent symptoms
Is a rare manifestation
Is a manifestation of organic brain disease
Is usually a psychosomatic symptom
Happened in the morning
Common hematological manifestations of juvenile systemic lupus are
Leukocytosis
Thrombocytosis
Acute hemolytic crisis
Mild Coombs positiv hemolytic anemias
Hemoconcentration
Lupus nephritis
Is uncommon in children
Has no prognostic value
Presents with hematuria and the proteinuria
Presents with dysuria
Presents with polakiuria
Lupus nephritis
Is self limited and benign
Are the major reason for morbidity and mortality in lupus patients
Is always associated with hypertension
Does not respond to steroid treatment
Response to any medication
Lung involvement in juvenile systemic lupus
Is a common feature
Is the uncommon except for pleuritis
Usually manifests as pulmonary hemorrhage
Is a benign manifestation
Is common with pneumonia
Antiphospholipid syndrome:
Is a bleeding disorder caused by auto antibody
Is only known in association with systemic lupus
Are the thrombophilia caused by the auto antibody
Never causes severe symptoms
Thrombopenia
The longterm course of juvenile systemic lupus
Is limited because of disease and the treatment related complications
Is excellent, independent of organ involvement
Is characterised by a longterm remission
Has not changed during the last 50 years
No complication
Hydroxychloroquine in systemic lupus
Is a drug with a well known way of action
Should be limited to situations with very severe disease activity
Does not have a beneficial effect
Should be given to every patient as a longterm treatments
Only treated with lupus patient
Neonatal lupus
Is a neonatal onset autoimmune disease
Is a disease caused by transplacentally transfered antibodies from the mother
May cause severe skin disease
Increases the child’s risk for the development of lupus later in life
Transferred antibody from the mother during delivery
The following is true for neonatal lupus, EXCEPT
May cause skin manifestation
May cause heart block
The heart block is usually reversible
Skin disease will resolve spontaneously
Nephropathy
Childhood Vasculitis
Is very rare disease
Always involve the kidney
Have a very poor prognosis
Are classified according to the size of affected vessels
Most frequency in white skin
Takayasu arteritis
Predominantly affects small and medium sized arteries
Is accompanied by a purpuric rash
Is the only large vessel vasculitis in the childhood
Never causes hypertension
Onset in childhood
Childhood vasculitis
Have the wide variety of possible symptoms
Usually manifests with purpura
Usually affects the kidney
Has always a poor progsnosis
Usally associated with fever
Henoch Schonlein purpura
Is a very common vasculitis in the childhood
Predominantly involves the coronary artery
Is caused by an infection
Never has renal involvement
Purpura non palpable
Henoch Schonlein purpura
Commonly affects muscles, skin and lung
Commonly affects skin, intestine and brain
Commonly affects joints, skin and intestine
Commonly affects joints, skin, intestine and the kidney
Only manifest on skin
Henoch Schonlein purpura
Commonly lasts 10-15 days
May have bursts of the purpura during weeks and months
Is a leukocytoclastic vasculitis with IgE deposits
Always needs immunosuppressive treatment to prevent damage
Antibiotic is the first choice of treament
Henoch Schonlein purpura
Is unrelated to infection
Will rarely affect the kidney
Are characterized by palpable purpuras
Is also called “pulseless disease”
Affected all organs except SNC
Henoch Schonlein purpura
Only occurs in young children
Is usually a self limited and the benign diseases
Renal involvement will not affect the prognosis
Early steroid treatment can prevent kidney disease
Hypertension is not involve
Henoch Schonlein purpura
May affect persons of all ages
Is less severe in older people
Complicated by myocarditis
Is a disease of short duration
Affective age is under 1 year
Kawasaki syndrom
Is the only large vessel vasculitis in childhood
Is also called “pulseless disease”
In addition to at least 5 days of the fever 4 of 5 criteria are required for the diagnosis
Kidney involvement is the most feared complication
Is a small vaculitis
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