Rheum Flashcards
Reactive arthritis
Arthritis that occurs following enteropathic or urogential infections
Post infectious arthritis
Occurs after infectious illnesses not classically considered in the reactive arthritis (enteropathic or urogential) group such as post GAS or viruses
Pain or joint swelling is usually transient, lasting < 6 weeks and does not neccessarily share the typicaly spondyloarthrthirits pattern of joint involvement
Transient Synovitis
- Typically affects the hip
- Often after uRTI
- Boys 3-10 years of age are most often affected and have acute onset of severe pain in the hip, lasting approximately one week
- ESR and CBC are usually normal
- Radiologic or ultrasound exam may confirm widening of the joint space secondary to an effusion
- Aspiration of the joint is often necessary to exclude septic arthritis
Trigger is presumed to be viral
Incomplete KD labs
- Anemia
- Hypoalbuminemia < 30
- Sterile pyuria (> 10 WBC)
- Plt > 450 after 7 days of fever
- ALT greater than normal
- WBC > 15
OR positive echo with less than 3 otherwise need to have 3
KD Ddx
- Viral
○ Adenovirus
○ Enterovirus
○ Measles
○ EBV
○ EMV- Bacterial infections
○ Scarlet fever
○ Rocky Mountain Spotted fever
○ Leptospirosis
○ Meningococcemia
○ UTI - Rheum
○ JIA
○ Bechet disease
○ Rheumatic fever - Other
○ TSS
○ Serum Sickness
○ Staph scalded skin syndrome
○ MAS
○ Drug hypersensitivity reactions
○ Steven Johnson syndrome
Aseptic meningitis
- Bacterial infections
FMF
Major
1. Typical attacks with peritonitis
2. Typical attacks with pleuritis or pericarditis
3. Typical attacks with Monoarthritis
4. Typical attacks with fever alone
5. Incomplete abdominal attack
Minor 1. Incomplete attacks involving chest pain 2. Incomplete attacks involving Monoarthritis 3. Exertional leg pain 4. Favorable response to colchicine
Genetics for FMF
Autosomal recessive MEFV gene
Cyclic neutropenia
- Autosomal dominant congenital granulopoiesis disorder
- Regular, periodic oscillations with ANC ranging from normal to <2, mirrored by reciprocal cycling of monocytes
- During the neutropenic nadir, many patients develop malaise, fever, oral and genital ulcers, gingivitis, periodontitis, or pharyngitis and occas. lymph node enlargement
- Mean period of the cycle is 21 days
- More serious infections occur occas. including pneumonia, mastoiditis and intestinal perforation
- Association with mutations in Neutrophil elastase gene (ELANE) cycles are less noticeable with age
- Diagnosed by obtaining blood counts 3 times/week for 6-8 weeks
- Can identify patients are risk for progression to myelodysplastic syndrome/AML associated with severe congenital neutropenia
- Affected patients are treated with GCSF and their cycle of profound neutropenia changes from a 21 day period to 9-11 day cycle with 1 day of less profound neutropenia
Gene for cyclic neutropenia
ELANE
Age 3-10 child with episodic pain that occurs at night after increased daytime physical activity that is relieved by rubbing with no complain of limp or pain in the AM
Growing pains
Intermittent pain in a child associated with hyperextensible joints on exam
Benign hyper mobility syndrome
Gelling phenomenon
Fatigue or stiffness after physical inactivity
Pulmonary vascular aneurysms
Bechets disease
anti smooth muscle
autoimmune hepatitis
anti SSA (Ro)
SLE, Sjogrens syndrome
SSB (La)
SLE, Sjogrens syndrome
Ribonuclease protein
SLE, MCTD
Histone
drug induced lupus
cANCAs
GPA, cystic fibrosis
pANCAs
microscopic polyangitis, polyarteritis nodosa, SLE, IBD, cystic fibrosis, PSC, HSP, KD, Chur Strauss syndrome
anti citrullinated protein (anti-CCP)
RF positive JIA
Methotrexate is used to treat?
JIA, uveitis
MTX side effects
GI intolerance (N/V), chemical hepatitis, myelosuppresion, mucositis, teratogenesis, lyphoma, interstitial pneumonitis
Hydroxychloroquine is used to treat?
SLE, JDMS, Anti phospholipid antibody syndrom
Hydroxychloroquine SE
retinal toxicity, GI intolerance, rash, skin discoloration, anemia, cytopenias, myopathy, CNS stimulation, death (overdose)
Infliximab treats
HIA, spondyloarthropatyh, uveitis, sarcoidosis
Cyclophosphamid used to treat
SLE, vasculitis, JDMS, pulmonary hemorrhage
cyclophosphamid SE
nausea, vomiting, myelosuppresion, mucositis, hyponatremia, alopecia, hemorrhagic cystitis, gonadal failure, teratgoenesis, secondary malignancy
What needs to me monitored on hydroxychloroquine
EYES for retinal toxicity every 6-12mo
Infliximab is used to treat
polyarticular JIA, Bechest syndrome, uveitis, sarcoidosis
And IBD
Adverse effects of infliximab
Injection site reactions, increased frequency of severe systemic infections including spsis, latent Tb and invasive fungal infections
Tb testing should be done before infliximab treatmetn
Rituximab is
chimeric antibody to the antigen CD20 which is a transmembrane protein on the surface of B cell precursors and mature B lymphocytes
Rituximab SE
serious infusion reactions cytopenias, hep B virus reactivation, hypogammaglobulinemia, infections, serum sickness, vasculitis and progressive multifocal luekoencephaloptahy
Anakinra is used to treat
sJIA and other autoinflammatory syndromes
Criteria for JIA
○ Age of onset < 16 years
○ Arthritis (swelling or effusion, or the presence of two or more of: limitation of ROM, tenderness or pain on motion, increased in heat in more than one joint)
○ Duration of disease > 6 weeks
○ Onset type defined by type of articular involvement in the 1st 6mo after onset (polyarthritis >5 involved joints, oligo < 4 involved joints, systemic onset disease: arthritis with rash and a characteristic quotidian fever)
○ Exclusion of other forms of juvenile arthritis
Systemic JIA criteria
arthritis in over 1 joint with or that is preceded by fever of at least 2 week in duration that is documented to be daily (quotidian) for at least 3 days and is accompanied by ONE of erytheamous rash, generalized lymph node enlargement, HSM, serositis
Psoriatic arthritis criteria
Psorisasis and arthritis or arthritis and 2 of the following: dactylitis, nal bed changes, psoriasis in first degree relative
Enthesitis related arthritis criteria
Arthritis and entehesitis with at least 2 of the following: presence or history of SI joint tenderness or inflammatory lumbosacral back pain, presence of HLA B 27 antigen, onset of arthritis in a male over 6 years old, acute symptomatic anterior uveitis, history of AS, ethesitis relted arthrtitis, sacroilitis with IBD, reiter syndrome or acute anteiror uvveitis in a first degree relative
Which joints are usually affected in oligoarthritis
Knees +++. ankles, fingers/wrist
Sex predominance in sJIA
EQUAL
ANA positive is correlated with
uveitis, younger age of disease onset, female sex, asymmetric arthritis and fewer involved joints over time
Koebner phenomenon
cutaenous hypersensitivty where classic lesions are brough on by superficial trauma
seen in sJIA rash
Is ESR high or low in MAS
low because of hypofibrinogemia and hepatic dysfunction
Classification for sJIA associated MAS
hyperferritinemia and any 2 of thrombocytopenia, hypofibrinogemia, elevated liver enzymes, hypertriglyceridemia
Lab features MAS
○ Cytopenia
○ Abnormal liver function tests
○ Hypofibrinogenemia
○ Decreased ESR
○ Hypertriglyceridemia
○ Hyponatremia
○ Hypoalbuinemia
○ Hyperferritinemia
Elevated CD25 and CD 163
Path features in MAS
Macrophage hemophagocytosis in the bone marrow aspirate
Marker for more aggressive disease in JIA
anti-CCP and RF
Treatment for oligoarthritis JIA
NSAIDs, then intra-articular steroids if no improvement after 4-6 weeks. Then may need to consider DMARDs such as MTX and TNF-alpha inhibitors
Enthesitis related JIA features
○ Frequent HLA B 27 positive
○ Most common category of JIA in India
○ Mean age of onset is 12
○ Peripheral arthritis (younger patients), enthesitis or axial involvement (teens)
○ Difficulty with forward flexion and flattening of natural lumbar lordosis
○ Tenderness over SI joints
○ Male predominance
Strong family predominance
DIP joint involvement is highly suggestive of ___ arthritis
psoriatic
Axial spondyloarthritis criteria
3 mo of back pain and sacroilitis on imaging PLUS 1 feature of SpA (inflam back pain, arthritis, enthesitis, uveitis, dactylitis, psoriasis, chronic disease/UC, good response to NSAIDs, fam hx, HLA-B27, elevated CRP)
Erythema nodosum, proderma gangrenosum, oral ulcers, abdominal pain, diarrhea, weight loss, fever, anorexia
IBD
Arthritis in IBD
Most commonly polyarthritis affecting large and small joints
Less frequently arthritis of the axial skeleton
Organisms causing reactive arthritis
○ Probable: chlamydia, shigella, salmonella, yersinia, campylobacter
Possible: Neisseria gonorrhae, mycoplasma, e. coli, cryptosporidium, entamoeba Histolytica, giardia, brucella, c. diff, strep pyogenes, chlamydia
Triad of reactive arthritis
Classic triad of arthritis, urethritis and conjunctivitis is relatively uncommon in children
Skin findings in reactive arthritis
keratoderma blenorrhagicum (scaly psoriasis like eruption on the feet), circinate balanitis, erythema nodosum
Type of arthritis in post-strep
Typically oligoarticular, affecting lower extremity joints and symptoms can persist for months
Boys 3-10 years of age are most often affected and have acute onset of severe pain in the hip, lasting approximately one wee
Transietn synovitis
Viruses causing viral myositis
Influenza A and B and enteroviruses are the most common viruses implicated
Poor outcomes in KD
Poor outcomes- young age, male gender, persistent fever, poor response to IVIG, lab abnormalities
KD labs
○ Elevated leukocyte count, predominance of neutrophils and immature forms
○ Normocytic, normochromic anemia
○ Platelet count is generally normal in 1st week and increases rapidly in 2-3rd week
○ Elevated CRP or ESR
○ Sterile pyuria
○ Mild elevation of hepatic enzymes
○ Hyperbilirubinemia
○ CSF pleocytosis
Coronary size echo measurement
Genes associated with CVID
Genes associated: ICOS, SH2DIA, TAC1
Hallmark finding in FMF
Hallmark finding is erysipeloid erythematous rash overlying the ankle or dorsum of the foot
Gene for FMF
Caused by autosomal recessive mutations in MEFV a gene encoding amino acid protein pyrin
Treatment for FMF
colchicine
Colchicine SE
○ SE: GI, dose related transaminitis, bone marrow suppression rare
Complication of FMF
Most serious complication of FMF is amyloidosis
inheritance cyclic neutropneia
Autosomal dominant
Cyclic neutorpneia presentation
- During the neutropenic nadir, many patients develop malaise, fever, oral and genital ulcers, gingivitis, periodontitis, or pharyngitis and occas. lymph node enlargement
Mean period of the cycle is 21 days +/- 4 days
How to diagnosis cyclic neutropneia
Diagnosed by obtaining blood counts 3 times/week for 6-8 weeks
Serum Sickness
Systemic, immune complex mediated hypersensitivity vasculitis classically attributed to the therapeutic administration of foreign serum proteins or other medications
Drugs causing serum sickness
anti-tetanus toxoid, infliximab, rituximab, etanercept, anti HIV antibodies, antibiotics (penicillin, septra, minocycline, meopnemen), neuro (carbamazepine, phenytoin, sulfonamides, barbiturates)
What type of reaction is serum sickness
class 3
Inv in serum sickness
Inv: often low plt, ESR and CRP elevated, mild proteinuria, complement studies are usually decreased
Clinical ft serum sickness
- Symptoms begin 7-12 days after injection of the foreign material but may be up to 3 weeks afterward
- Symptoms: fever, malaise, rashes, urticaria and morbilliform rashes
- Symptoms typically resolve within 2 weeks of removal of the offending agent
- Carditis, GN, GBS, peripheral neuritis are rare complications
Diagnosis is clinical based on characteristic pattern of rash, fever, arthralgia and myalgia disproportionate to the degree of swelling after exposure to potential culprit
Treatment for serum sickness
- Treatment is supportive, removal of offending agent, antihistamines for pruritus, NSAIDs and analgesics
Can use short course of steroids if very severe symptoms
Do ANA titers correlate with disease severity in SLE
No
Anti-ds DNA
- Specific for diagnosis of SLE
- Correlates with disease activity, esp nephritis in some with SLE
84-100%
Anti-Smith antibody
Specific for the diagnosis of SLE
Does not correlate with disease severity
Less common 23-48%
Anti-ribonucleoprotein antibody
Increased risk for Raynaud phenomenon, ILD and pulmonary hypertension 31-62%
Anti-RO antibody (anti-SSA antibody)
Associated with Sicca syndrome 38-54%
Anti-La antibody (Anti-SSB antibody)May suggest diagnosis of Sjogrens syndrome
Increased risk of neonatal lupus in offspring
May be associated with cutaneous and pulmonary manifestations of SL
May be associated with isolated discoid lupus
16-32%
Antiphospholipid antibodies
Increased risk for venous and arterial thrombotic events
Can be found in 66% of children and adolescent with SLE
Results in false positive RPR, prolonged aPTT and abnormal DRVVT
Anti-histone antibodies
Present in majority of patients with drug induced lupus (00%)
May be present in SLE
Anti Ribosomal P antibodies
Found more frequently in children and are associated with neuropsychiatric, renal and hepatic involvement
RPR can be false positive in
Can be false positive in: SLE, other autoimmune disorders, HIV, OVDU, chronic liver disease
Positive p-ANCA
- Specific but not sensitive for UC
- Eosinophilic granulomatosis with polyangiitis
- Primary sclerosing cholangitis
- Microscopic polyangiitis
- Focal necrotizing and crescentering GN
Rheumatoid arthritis
Positive anti-LKM antibody
AIH type 2
ARF criteria
Need 2 major or 1 major and 2 minor
Major= JONES
MINOR= fever, arthralgias, prolonged PR, elevated ESR/CRP
Valves affected in ARF carditis
Mitral valve is most commonly affected, followed by aortic
Rash in ARF
erythema marginatum
Sydenham’s chorea
Facial grimace, protruding tongue (wormian tongue), irregular contractures of hand muscles on active grip (milkmaids grip)
Drugs for drug induced lupus
hydralazine, minocycline, anticonvulsants, sulfonamides, antiarrhythmic agents
Usually presents between ages 2-5 with recurring episodes of fever, malaise, exudative-appearing tonsillitis with negative throat cultures, cervical lymphadenopathy, oral apthae and less often headache, abdominal pain and arthralgia
Periodic Fever, Apthous Stomatitis, pharyngitis and adenitis (PFAPA)
Treatment for PFAPA
Most patients show a dramatic response to a single dose of oral prednisone although this approach does not prevent recurrence and may actually shorten the interval between flares
- Cimetidine is effective at preventing recurrences in 1/3 of cases
- Anakinra may be effective during a flare
- Colchicine may extend the time between flares
Complete resolution has been shown with tonsillectomy
Criteria for PFAPA
Onset of disease in early childhood
●Regularly recurring episodes of fever
●Presence of at least one of these associated features during flares: aphthous stomatitis, pharyngitis, and/or cervical adenitis
●Asymptomatic intervals between flares with normal growth
●Absence of signs of respiratory tract infection during flares and exclusion of cyclic neutropenia, other known periodic fever syndromes, immunodeficiency, or autoimmune diseases
HLA alleles for JDM
HLA alleles such as B8, DRB1, DQA1 are associated with increased risk for JDM
Diagnosis for JDM
To diagnose, you need to have rash as well as 3 signs of muscle inflammation/weakness
This can be clinical, lab, EMG or muscle biopsy
Now MRI used instead of muscle biopsy
EMG in JDM shows
EMG shows signs of myopathy (increased insertional activity, fibrillations and sharp waves) as well as muscle fiber necrosis (decreased action potential amplitude and duration)
Biopsy in JDM shows
Biopsy shows focal necrosis, phagocytosis of muscle fibers, fiber regeneration, endomysial proliferation, inflammatory cell infiltrates or vasculitis, tubulovesicular bodies within endothelial cells
