Rheum Flashcards
What condition mainly affects the SI joints causing eventual fusion of the spine?
Ankylosing spondylitis
What antigen is positive over 90% of the time with ankylosing spondylitis?
HLA-B27
True or False: Ankylosing spondylitis more often affects males?
True (3:1)
What two main things besides ortho problems is ankylosing spondylitis associated with?
- Eye problems (iritis. uveitis)
2. IBD
Bamboo spine on plain film, night pain, morning stiffness relieved by exercise, pain of large joints (knee), leg/back pain (increased when bending over), low grade fever, weight loss?
Ankylosing spondylitis
How can you differentiate between pain due to ankylosing spondylitis versus low back pain?
AS: Improves with activity and worse with rest
Mechanical: Worse with activity and improves with rest
What happens to ANA, RF, and ESR in ankylosing spondylitis?
ANA/RF: Normal
ESR: Normal or mildly elevated
Kid younger than 10, heliotrope rash on face (similar to lupus), tight/shiny/scaly skin on extensor surfaces of extremities or over interphalangeal joints, periungual lesions?
Dermatomyositis
What is Gottron’s sign?
Tight, shiny, or scaly skin on extensor surfaces of the extremities or over the itnerphalangeal joints (seen in dermatomyositis)
What symptoms does the rash associated with dermatomyositis cause?
- Pruritus
2. Hair loss (if on scalp)
How might the periungual lesions seem in dermatomyositis be described?
Nail fold telangectasias
Describe the heliotrope rash seen in dermatomyositis?
Violaceous “butterfly” discoloration of the malar region and/or eyelids
What are the findings that constitute the “myositis” part of dermatomyositis?
Proximal muscle weakness: Difficulty getting dressed or climbing steps, clumsy, voice change, difficulty swallowing
What is the most appropriate initial step to evaluate a patient with clinical dermatomyositis?
Cretine kinase level (often elevated)
*You would do this before EMG or MRI
What is the most common systemic vasculitis in kids?
HSP
What systems does HSP involve?
Skin, GI, joints, kidneys
What causes HSP?
Etiology unknown, but often with antecedent bacterial or viral infection
Who is more often affected with HSP, boys or girls?
Boys
Which symptom is present in all cases of HSP, but may not be the first sign?
Palpable purpura (usually on lower extremities and buttocks)
What are the main symptoms of HSP?
- Palpable purpura
- Colicky abdominal pain
- Heme positive stools
- Renal disease
- Asthritis/arthralgia
What is often the cause of abdominal pain in HSP?
Intussuception
How does renal disease in HSP present?
- Hematuria
- Proteinuria
- Azotemia
- HTN
What lab tests diagnose HSP?
None
What labs are suggestive of HSP in the right clinical scenario?
- Elevated BUN and creatinine
- Heme positive stool
- UA with hematuria
What happens to platelet count and coags in HSP?
- No thrombocytopenia (normal platelet count)
2. Normal PT/PTT
What is HSP often misdiagnosed as?
Abuse (Rash appears like bruises)
What is the treatment for HSP?
No specific treatment- Care is supportive only and most cases resolve without issue
What can the rash seen with HSP be confused with?
Erythema marginatum (rheumatic fever)
Besides skin findings, what other feature is common between HSP and erythema marginatum?
Joint pains (but, the joint pain seen in rheumatic fever is more severe and a more prominent feature)
What is the rash often seen in JIA?
Salmon-colored evanescent rash
True or false: JIA is not typically associated with abdominal pain and the rash isn’t purpuric
True
What is another name for IgA vasculitis?
HSP (this is an autoimmune vasculitis associated with IgA deposition)
What test needs to be done in all patients with HSP?
UA: To evaluate for hematuria and proteinuria
What do the findings on renal biopsy show for HSP?
Exact same as IgA nephropathy
What is the natural course for hematuria and proteinuria in HSP?
Transient and resolve in a few months without permanent kidney damage
Which patients have an increased risk of long-term renal disease in HSP?
Presenting with:
- Nephrotic-range proteinuria
- Elevated creatinine
- HTN
- Females
What did JIA used to be called?
Juvenile rheumatoid arthritis
What is the most common rheumatologic disease of childhood?
JIA
How is JIA diagnosed?
Basically a diagnosis of exclusion
How does JIA typically present?
- Morning stiffness
- Gradual loss of motion
- Rash
What age and time requirements must be met for JIA diagnosis?
- Under 16 years of age
2. Symptoms present for at least 6 weeks in at least one joint
What joints are more commonly involved in JIA?
Large joints (versus small joints)
What lab is often positive in JIA?
ANA
Who is more affected by JIA, boys or girls?
Girls…except:
- Systemic onset: Equal
- Enthesitis-related JIA: Boys
True or False: RF is a helpful lab to diagnose JIA?
False- RF is seldom positive and there are also a lot of false positives
Name 3 specific subtypes of JIA
- Polyarthritis
- Oligoarthritis/Pauciarthritis
- Systemic onset JIA
What are the requiresments for polyarthritis?
5 or more joints affected in first 6 months of disease
Which gender does polyarthritis JIA occur more commonly in?
Females
True or False: Systemic disease is common in polyarticular JIA
False
What are the requirements for oligoarthritis/pauciarthritis?
4 or fewer joints affected during first 6 months of disease
Which age group with oligoarthritis (JIA) is ANA-positive?
Young females (2-4 years of age)
What is the main morbidity of oligoarthritis?
Chronic uveitis
True or False: RF is usually negative in oligoarthritis?
True (but when it is positive the disease is often worse)
What are boys with oligoarthritis usually positive for?
HLA-B27 (their prognosis is good)
What is Still’s disease?
Systemic Onset JIA
Which gender does systemic JIA affect more frequently?
Males and females equally
True or False: Extraarticular involvement is common in systemic JIA
True
Most JIA deaths involve which type?
Systemic
What is an important consideration in evaluation of fever of unknown origin from a rheum perpective?
Systemic onset JIA
What is the timeframe for systemic JIA?
The systemic manifestations may precede joint manifestations by several months
Name 6 presenting symptoms of systemic onset JIA
- High fever with shaking chills
- Leukocytosis (as high as 30-50,000)
- Rash
- Hepatosplenomegaly
- Lymphadenopathy
- Pleuritis/pericarditis/serositis
What are the time requirements for fever/chills with systemic onset JIA?
At least 2 weeks duration that is daily for at least 3 days
Describe the rash seen in systemic onset JIA?
Small, salmon-colored macules with central clearings which coalesce and are vanescent “come and go”.
True or False: Uveitis is rare in systemic JIA
True
What are ANA and RF in systemic onset JIA?
Usually negative (ANA is only positive about 10% of the time)
True or False: Most of the systemic manifestations of systemic JIA are self-limited
True
Name 3 more minor forms of JIA
- Psoriatic
- Enthesitis-related
- Undifferentiated
What type of JIA is seen with family history of psoriasis and may have nail pitting and dactylitis?
Psoriatic arthritis
Which gender is psoriatic arthritis more common in?
Girls (despite association with HLA B-27)
What type of JIA involves sacro-iliac tenderness and HLA B-27?
Enthesitis-related arthritis
What is the only arthritis more common in boys?
Enthesitis-related arthritis
True or False: You can see anterior uveitis in enthesitis-related arthriris?
True
What is it called when you have an arthritis that doesn’t fit criteria of another chronic arthritis?
Undifferentiated arthrtitis
What condition can JIA be difficult to distinguish from?
Leukoemia (especially when they have MSK findings in addition to the hematologic ones)
How are the MSK symptoms of JIA and leukemia different?
JIA: Morning stiffness, spiking fevers, rashes
Leukemia: MSK pain awakens child at night, bone pain that doesn’t involve a joint
How are the timing of symptoms different in JIA and leukemia?
JIA: Periodic, waxing and waning
Leukemia: Persistent and worsening
How are the hematologic abnormalities different in JIA and leukemia?
JIA: More mild
Leukemia: More severe
Name 5 findings that are typical of both JIA and leukemia and won’t help you differentiate between the two
- Lymphadenopathy
- Leukocytosis
- Anemia
- Fatgiue
- Hepatosplenomegaly
Name the 3 mainstays of treatment for JIA
- PT/OT
- Anti-inflammatory meds
- Psychosocial support
What is the 1st line anti-inflammatory medication for JIA?
NSAIDs:
- Indomethacin
- Ibuprofen
- Naproxen
In kids with JIA on NSAIDs, what are they at risk for?
- GI bleeds
- Elevated LFTs
(known side effects of NSAIDs)
If NSAIDs don’t work in JIA, what is your next step (2nd line treatment)?
- Steroids
2. Immunosuppressive meds
What immunosuppresive medication may be necessary in kids with aggressive JIA?
Methotrexate
How does methotrexate work?
Folate antagonist
What are the 2 main side effects of methotrexate?
- GI distress
2. Pulmonary toxicity
Name 4 long term consequences of JIA
- Leg-length discrepancy
- Joint contractures
- Permanent join destruction
- Blindness from chronic uveitis
What % of kids with JIA continue to have active disease into adulthood?
50%
What do you have to remember with vaccine schedules for children with JIA taking methotrexate?
They are immunosuppressed- Avoid live vaccines (risk for infection)