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)
What type of malignancies are children on methotrexate at risk for?
Lymphoproliferative
What is the gold standard therapy for kids with JIA?
Methotrexate
Name 5 causes of arthritis in children
GLOVE Gonorrhea, Genetic Syndromes Lyme disease Osteomyelitis Viral (Toxic Synovitis) Evasive infection (Septic Arthritis)
What STD can manifest as arthritis?
GC
What are 3 things than can manifest as monoartricular arthritis?
L’s
Lyme (and other infections)
Legg-Calve-Perthes disease
Leukemia and bone tumors
What are 7 things that can cause polyarticular arthritis?
FIRE CAM
Fabry/Farber disease
Infections
Reactive arthritis (post strep, rheumatic fever, serum sickness)
slE (lupus)
Connective tissue disease: SLE, sarcoidoisis, vasculitis IBD
Malignancy (leukemia)
What is something you need to consider in any child with fever of unknown origin?
Kawasaki disease
What is the formal name of Kawasaki Disease?
Mucocutaneous Lymph Node Syndrome
What age group typically gets Kawasaki disease?
Children younger than 4 (most commonly around 2)
What months do you typically see more Kawasaki disease in?
Winter and spring
What gender and ethnicity are more likely to get Kawasaki?
Males, Asian (Japanese)
What are the diagnostic criteria for Kawasaki?
Acute high fever of at least 5 days plus 4 of the following:
- Cervical lymphadenopathy
- Dry/Fissured lips or swollen tongue
- Conjunctivitis
- Polymorphous exanthem concentrated on trunk
- Changes in extremities- Erythema/induration leading to desquamation on hands and feet
What are additional features that can be seen in Kawasaki, but aren’t part of the formal diagnostic criteria?
- Sterile pyuria (WBCs in urine)
- CNS symptoms (seizures and aseptic meningitis)
- Polyarthritis (migratory)
- Hydrops of the gallbladder
- Thrombocytosis
What is absolutely required for a diagnosis of Kawasaki?
Fever for at least 5 days
What day of illness do you see thrombocytosis in KawasakI?
By the 5th day
What day of illness do you see leukocytosis in Kawasaki?
By the 12th day
What type of anemia is seen in Kawasaki?
Normocytic anemia (consistent with chronic illness)
What acute phase reactants are elevated in Kawasaki and for how long?
CRP, ESR, A1AT
Remain high for 4-6 weeks
What % of cases of Kawasaki have sterile pyuria?
80%
What is seen on lipid panel for Kawasaki?
Decreased levels of total cholesterol and HDL
What are 3 common tests done from a rheum perspective that are not elevated in Kawasaki?
- ANA
- RF
- Circulating anticoagulant
What is the primary concern in Kawasaki?
Coronary artery disease
When should treatment begin in Kawasaki?
When the diagnosis is suspected
Even if diagnosis is clear, start treatment BEFORE getting a cardiac ECHO
What test must be done in all cases of suspected Kawasaki?
2-D cardiac ECHO (to look for coronary artery aneurysms)
True or False: Even children with no coronary findings in suspected Kawasaki disease need a follow-up ECHO one year after illness?
True
What are the 3 main things used to treat Kawasaki?
- IVIG, 2g/kg
- ASA, 80mg/kg/day for 24-48 hours… followed by
- ASA, 5mg/kg/day for 2 months
What is the leading cause of acquired heart disease in developed countries?
Kawasaki disease
What is the leading cause of acquired heart disease in developing countries?
Rheumatic heart disease
What changes to vaccine schedules must happen in kids who have had Kawasaki and got IVIG?
Measles and Varicella containing vaccines must not be given for 11 months after IVIG
Name 10 things which could be confused with Kawasaki disease
- Hypersensitivity reactions (drug reactions and SJS)
- JIA: Systemic type
- Measles
- Scarlet Fever
- EBV
- Enteroviruses
- RMSF
- Leptospirosis
- SSSS
- TSS
Child on antibiotics who hasn’t responded to treatment and developed a macular papular rash?
Kawasaki v. Hypersensitivity reaction (Drug reaction or SJS)
What are some features to distinguish between Kawasaki and Hypersensitivity reaction?
In drug reaction there shouldn’t be a high fever or other clinical criteria associated with Kawasaki
What are some common features between Kawasaki and Systemic JIA?
Fever, rash, adenopathy
How is the fever different in Kawasaki and JIA?
Fever is not acute in JIA
Rash is an evanescent, reticular rash that appears when fever peaks in JIA
What are some clues that would point to systemic JIA versus Kawasaki?
Hepatosplenomegaly, pleural effusions, cardiomegaly
True or False: Cardiomegaly can be seen in Kawasaki?
FALSE
What two things do Kawasaki and Measles both present with?
Fever and conjunctivitis
How is the conjunctivitis seen in Kawasaki different from that in Measles?
Measles is an exudative conjunctivitis
Describe the rash in measles
Starts at hairline and progresses downward to extremities (different from Kawasaki)
What are 2 ways to distinguish scarlet fever from Kawasaki?
Rash in scarlet fever is usually in flexural areas of extremtieis
Scarlet fever should have a positive strep test
What causes Lyme disease?
Infection with Borrelia burgdorferi (spirochete) and the body’s immunologic response to the infection
What carries Borrelia Burgdorferi to the the body?
Ixodes deer tick
What is the formal name for the bullseye rash seen in Lyme disease?
Erythema chronicum migrans (ECM)
What % of cases doesn’t have the bullseye rash in Lyme disease?
25%
How are cases of Lyme disease most often presented on boards?
Patient with Lyme arthritis without a distinct history of ECM rash or deer tick bite
What happens in the first 2 weeks of Lyme disease?
- ECM rash at site of tick bite
2. Vague flu-like symptoms with severe arthralgia and extreme fatigue
What happens in the first several months of Lyme disease?
CNS/Cardiac/Arthritic
- Carditis
- Pauciarticular arthritis
- Neurological signs (Bell’s Palsy)
What happens years after infection with Lyme disease?
Progression of CNS, Cardiac, and arthritic symptoms- Especially the arthritis
How long to deer ticks need to feed to transmit Lyme disease?
At least 36 hours
What is the best way to prevent Lyme disease?
Frequent tick checks
Describe Lyme arthritis
Pauciarticular involving large joints (especially the knee)
What is different between Lyme arthritis affecting a knee versus a septic arthritis?
Lyme the knee can be tender and swollen, but the pain isn’t unbearable like in septic arthritis- The child will probably be able to walk around even with a big knee
Why are false negatives so common in Lyme disease (especially initially)?
Detectable levels of serum antibodies don’t build up until 4-6 weeks (false negatives)
How does early treatment affect lab testing for Lyme disease?
If treatment is given early, the immunologic response is blunted and the lab results become unreliable
What conditions can give false positive testing for Lyme?
- Autoimmune diseases: SLE, dermatomyositis
- Other rickettsial diaseases
What tests should be done for Lyme disease and in what order?
- Lyme antibody titer (EIA)
2. Western blot to confirm
What is treatment for Lyme disease?
Doxycycline (8 and older)
Amoxicillin (or cefuroxime if PCN allergic) (under 8)
How long should treatment for Lyme disease be given?
14-21 days (maximum duration of therapy 4 weeks)
Child with confirmed Lyme disease who gets treated then develops chills, fevers hypotension, sepsis-like picture…?
Jarisch-Herxheimer reaction- Result of lysis of the organism and release of endotoxin
What category does reactive arthritis fall under?
Seronegative spondyloarthropathy
What does seronegative spndyloarthroapthy mean?
ANA and RF should be normal
What HLA marker can reactive arthritis be assocaited with?
B-27
What are the 3 features of reactive arthritis?
- Urethritis
- Iritis
- Arthritis
“Can’t see, can’t pee, can’t climb a tree”
When do you usually see reactive arthritis?
After infection with
- Enteric (Yersinia, Shigella, Campylobacter, or Salmonella)
- Venereal (Chlamydia, Gonorrhea)
What is the treatment for reactive arthritis?
Supportive: NSAIDs and antibiotics
What GI disease should you always consider if presented with a child with arthritis?
IBD
What is the most common cause of reactive arthritis in US?
Chlamydia
What was the old name for reactive arthritis?
Reiter’s syndrome
How does sarcoidosis present?
History of weight loss and fatigue
Hilar adenopathy
Chronic cough
What race is sarcoidosis seen more frequently in?
African Americans
Otherwise healthy, afebrile child who fatigues easily during sports (sometimes to the point of quitting the sport) and has a chronic cough?
Sarcoidosis
What should you thinkg of with noncaseating granulomas and bilateral peribronchial cuffing on CXR?
Sarcoidosis
What effect do the noncaseating granulomas have in sarcoidosis?
Secrete form of vitamin D leading to hypercalcemia and hypercalciuria
What causes renal disease and eye disease seen in sarcoidosis?
Hypercalcemia and hypercalciuria from excess vitamin D secreted from noncaseating granulomas
True or False: Sarcoidosis can involve the heart?
True
How can you distinguish between Sarcoidosis and TB?
- Sarcoidosis should have a negative TB test
2. Sarcoidosis may have heart involvement- EKG with a rhythm disturbance
What is the triad of sarcoidosis?
- Arthritis
- Rash
- Uveitis
What results in a thickening and tightening of the skin (often with induration)?
Scleroderma
Which gender if more often affected by scleroderma?
Females
What are the 2 main forms of scleroderma?
- Localized linear
2. Systemic
How does the localized form of scleroderma being?
Linear hyperpigmented patch that becomes more and more fibrotic (only involves skin and adjacent subcutaneous tissue). Patches of skin are painful and tender.
Shiny hypopigmented skin with a brown border?
Localized linear scleroderma
Which form of scleroderma is more common and has a better outcome?
Localized- Requires minimal treatment and is self-limited
What is the treatment for localized scleroderma?
Topical lubricants
Photochemotherapy (occasionally)
If localized scleroderma was more widespread and progressive, what other treatment modalities (besides topical lubricants and photochemotherapy) could you use?
- Steroids
- Antimalarias
- Immunosuppressives (methotrexate, penicillamine)
True or False: With localized scleroderma, it is important to treat to prevent the progression to the systemic form
FALSE
True or False: The systemic form of scleroderma is rarely seen in children
True
What can the systemic form of scleroderma present with (in addition to findings seen in localized)?
- Sclerodactyly (localized scleroderma of digits)
- Pulmonary fibrosis
- Reflux/dysphagia (from LES incompetence)
- Raynaud’s
How do people with systemic scleroderma often present?
With Raynaud’s (almost everyone has this at some point in their course)
True or False: ANA is almost always positive in systemic scleroderma?
True
What causes SLE?
Formation of antigen-antibody complexes in a variety of tissues
Which gender gets SLE more often?
Women (but gender ratio is more equal in younger children)
Which race’s get SLE more often?
African Americans, Hispanics, Asians (versus Caucasians)
What 3 symptoms do most patients present with?
- Rash
- Fever
- Arthritis
Besides rash, fever, and arthritis, what are 4 other common symptoms at presentation?
- Fatigue
- Weight loss
- Lymphadenopathy
- Hepatosplenomegaly
A diagnosis of SLE is suggested when 4 or more of what features present over time?
- Malar rash
- Discoid lesions
- Photosensitivity
- Oral ulcerations
- Arhtritis and serositis
- Hematological abnormalities
- Renal abnormalities
- Presence of anti-ds-DNA, anti-DNA, and anti-Smith antibodies
- Psychosis or other neurologic abnormalities
- Positive ANA
Which antibodies are very specific for SLE?
Anti-ds-DNA (positive results are rarely seen in other patients- including those with other rheumatological disorders)
How can the severity of disease flareups be tracked in SLE?
- Anti-ds-DNA levels (fluctuate accordingly)
2. C3/C4 levels (inversely proportional- go down in more active or acute disease states)
Which antibody is specific to SLE, but remains elevated regardless of disease activity (useful for identifying disease, but not tracking disease)?
Anti-smith
Name 3 main components to clinical disease in SLE
- Polyserositis
- Renal disease (nephritis)
- Cerebritis
What does polyserositis affect?
Any organ with a pleural covering… includes primarily the heart (pericarditis) and lungs (pleurisy)
What is cerebritis a result of in SLE?
Areas of microischemia and vascular disease
What can cerebritis result in in SLE?
Seizures
What type of test is ANA (sensitive or specific)?
Sensitive (negative rules it out)
-Positive in almost all SLE, but many other inflammatory disorders can have a positive ANA
What type of test is dsDNA (sensitive or specific)?
Specific (positive rules it in)
What test do you want to screen for a disease?
Sensitive
What test to you want to secure a diagnosis?
Specific
What is the course of Raynaud?
White: Ischemia
Blue: Cyanosis
Red: Coldness
What do you often see Raynaud’s in?
Lupus
Name 3 signs that can be seen with neonatal lupus
- Rashes on trunk
- 3rd degree heart block
- Bradycardia
What can neonatal lupus be assocaited with?
Hydrops fetalis
What can heart block lead to in neonatal lupus?
Heart failure (baby may need a pacemaker)
What is the most likely explanation for death in an infant born to a mother with SLE?
Heart block
True or False: Most newborns born to women with SLE don’t develop neonatal lupus?
True
True or False: Many newborns with neonatal lupus, Mom didn’t know she had SLE?
True- If presented with neonate with bradycardia or heart block, they will likely not mention a history of lupus in Mom
What antibody is closely associated with neonaal lupus?
Anti-SSA
Most infants with congenital heart block have Mom’s who test positive for what antibodies?
Anti-SSA or Anti-SSB
What is used for treatment of mild lupus?
- NSAIDs
- Hydroxychloroquine
- Dapsone (skin)
- Prednisone (low dose)
What is a rare side effect of hydroxychloroquine?
Ototoxicity
What is a common side effect of hydroxychloroquine?
Opthalmological side effects (temporary blurring to permanent retinal damage)
What is used for treatment of severe lupus?
- Pulses of high dose steroids
2. Immunosuppressants (cyclophosphamide/azathioprine)
What is more severe lupus characterized by?
Unremitting arthralgias, kidney involvement, serositis, or CNS effects (seizures and cerebritis)
What medication is used for particular serious organ involvement in SLE?
Cyclophosphamide
What are lupus patients undergoing treatment with immunosuppresive agents at risk for?
Serious infections (varicella)
What are patient with lupus on steroids at risk for?
Complications associated with chronic steroid use: Cataracts, glaucoma, osteoporosis, high blood pressures, glucose intolerance, cushingoig features
What is the color and viscosity of normal synovial fluid?
Yellow/clear
Normal or slightly increased viscosity (WBC <200)
What is the color and viscosity of synovial fluid in arthritis secondary to trauma?
Bloody/clear Increased viscosity (WBC <2000)
What is the color and viscosity of synovial fluid in lupus?
Yellow/clear Normal viscosity (WBC 5,000 and LE cells)
What is the color and viscosity of synovial fluid in rheumatic fever?
Yellow/cloudy Decreased viscosity (WBC 5,000)
What is the color and viscosity of synovial fluid in JIA?
Yellow/cloudy Decreased viscosity (WBC 15,000-20,000)
What is the color and viscosity of synovial fluid in reactive arthritis?
Yellow/Opaque Decreased viscosity (WBC 20,000)
What is the color and viscosity of synovial fluid in septic arthritis?
Yellow Variable viscosity (WBC 50,000-300,000 and low glucose and bacteria)