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
