Pediatric Rheuma From www.rheumatology.com Flashcards
A four year-old white female is brought to your office because her mother says, for the last two months, she has limped every morning for about an hour, which eventually improves. She has had no fevers or rash. On your exam, she is happy and playful but walks with a limp. She has swelling of her left knee and left ankle. Her CBC, ESR, and CRP are normal. Her rheumatoid factor, anti-CCP antibodies, and Lyme studies are negative. She has an ANA that is positive at 1:160
Diagnosis?
JIA
Differential Diagnosis of Patient with JIA:
Patient with significant joint swelling, especially of the knee
Lyme Arthritis
Differential Diagnosis of Patient with JIA:
not associated with a morning preponderance of symptoms
Trauma
Differential Diagnosis of patient with JIA:
With more acute in presentation and be associated with more systemic symptoms;
Septic Arthritis and Osteomyelitis
Differential Diagnosis of patient with JIA:
________________ , such as pediatric lupus, juvenile dermatomyositis or systemic vasculitis, which would have more extraarticular features
Systemic Autoimmune Disease
Differential Diagnosis of patient with JIA:
Non-_________ causes of joint pain, including slipped capital femoral epiphysis and idiopathic avascular necrosis, which would be associated with activity-related pain, rather than morning stiffness.
inflammatory
Significance of :
- Negative RF
- Negative anti-CCp Ab
- Normal inflammatory markers
More aggressive course
JIA patients with just a few joints affected often have normal inflammatory markers
Major commorbidity with JIA
Uveitis
Significant Risk Factors for UVEITIS:
_____ gender
_____ age
RF ____
recent development of disease
Significant Risk Factors for UVEITIS:
Female gender
Young age
RF Positive
recent development of disease
Patients with ENTHESITIS related arthritis get acute uveitis, characterized as:
Red, Painful, Photophobic eye
Patient with Systemic JIA, with fever altered mental status, Transaminitis and Pancytopenia
Macrophage activation syndrome
most sever complication of sJIA
Laboratory hallmarks of MAS:
Hyperferritinemia Cytopenia Transaminase \_\_\_\_\_\_\_\_ Paradoxically \_\_\_\_ or \_\_\_\_\_ ESR \_\_\_\_\_\_ Triglycerides \_\_\_\_\_\_\_\_\_\_\_ PT and PTT
Hyperferritinemia Cytopenia Transaminase elevation Paradoxically low or normal ESR Elevated Triglycerides Prolonged PT and PTT
You get called to a consult in the ED because there is a nine year-old Latino male who has a red raised rash on his legs and buttocks. When you see him, you also notice that his knee is swollen, but he seems to be walking without difficulty. Otherwise he looks happy and well. His CBC, ESR, and coagulation studies are normal.
HSP
IgA mediated vasculitis
Histologic Features of HSP
Leukocytoclasis
-vascular damage caused by nuclear debris form infiltrating neutrophils
IgA deposition
Major clinical manifestion of HSP:
Tetrad
- Purpura
- Arthritis
- GI involvement
- Renal involvement
A five year-old boy has had seven days of fever up to 104F. Over the last two days, he has developed a large right-sided cervical lymph node, a maculopapular rash, and injected sclera. His lips are red and cracked.
Kawasaki Disease
Medium vessel vasculitis: esp coronary arteries
Clinical criteria for Kawasaki disease:
Fever + at least 4 of
1) Bilateral conjunctival injection (occurs in 80-90% of patients; typically limbic-sparing); (2) Oropharyngeal mucous membrane changes (80-90%; injected and / or fissured lips, strawberry tongue, injected pharynx);
(3) Peripheral extremity changes (80%; Erythema and / or edema of the hands and feet or periungual desquamation);
(4) Polymorphous rash (>90%);
(5) Cervical lymphadenopathy with node >1.5 cm (50%)
Major complication of Kawasaki Disease
Coronary aneurysm
In clinic, a 14 year-old female is seen for erythematous plaques over her knuckles,elbows, and knees. She also has a purplish rash on her eyelids and some erythema on her cheeks. When you ask her to stand up so you can examine her back, she has trouble getting up out of the chair.
Juvenile Dermatomyositis
Children with JDM or Adult DM?
Calcinosis, lipodystrophy, and gastrointestinal and cutaneous ulcerations.
Children with JDM
Children with JDM or Adult DM?
(+) Anti-synthetase antibodies
Children 0-5%
Adult 20-25%
A patient with JDM presents with acute onset of abdominal pain.
Diagnosis?
Visceral Vasculopathy which may lead to death.
Severe abdominal pain, with or without melena or hematemesis, may signify vasculopathy of the gastrointestinal tract and resulting tissue ischemia or mesenteric infarction.
The presence of ___________ signifies a patient who is at higher risk for visceral ulceration and may warrant more aggressive therapy with cytotoxic agents.
Cutaneous ulceration
Type of JIA:
Four or less joints affected in the first 6 months of disease.
Oligoarticular
Typically younger (2-5 year) females. Associated with the best prognosis but highest risk of uveitis.
