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Rheum Flashcards

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1
Q

Reactive arthritis

A

Arthritis that occurs following enteropathic or urogential infections

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2
Q

Post infectious arthritis

A

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

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3
Q

Transient Synovitis

A
  • 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
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4
Q

Incomplete KD labs

A
  1. Anemia
  2. Hypoalbuminemia < 30
  3. Sterile pyuria (> 10 WBC)
  4. Plt > 450 after 7 days of fever
  5. ALT greater than normal
  6. WBC > 15
    OR positive echo with less than 3 otherwise need to have 3
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5
Q

KD Ddx

A
  • 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
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6
Q

FMF

A

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
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7
Q

Genetics for FMF

A

Autosomal recessive MEFV gene

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8
Q

Cyclic neutropenia

A
  • 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
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9
Q

Gene for cyclic neutropenia

A

ELANE

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10
Q

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

A

Growing pains

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11
Q

Intermittent pain in a child associated with hyperextensible joints on exam

A

Benign hyper mobility syndrome

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12
Q

Gelling phenomenon

A

Fatigue or stiffness after physical inactivity

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13
Q

Pulmonary vascular aneurysms

A

Bechets disease

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14
Q

anti smooth muscle

A

autoimmune hepatitis

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15
Q

anti SSA (Ro)

A

SLE, Sjogrens syndrome

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16
Q

SSB (La)

A

SLE, Sjogrens syndrome

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17
Q

Ribonuclease protein

A

SLE, MCTD

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18
Q

Histone

A

drug induced lupus

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19
Q

cANCAs

A

GPA, cystic fibrosis

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20
Q

pANCAs

A

microscopic polyangitis, polyarteritis nodosa, SLE, IBD, cystic fibrosis, PSC, HSP, KD, Chur Strauss syndrome

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21
Q

anti citrullinated protein (anti-CCP)

A

RF positive JIA

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22
Q

Methotrexate is used to treat?

A

JIA, uveitis

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23
Q

MTX side effects

A

GI intolerance (N/V), chemical hepatitis, myelosuppresion, mucositis, teratogenesis, lyphoma, interstitial pneumonitis

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24
Q

Hydroxychloroquine is used to treat?

A

SLE, JDMS, Anti phospholipid antibody syndrom

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25
Q

Hydroxychloroquine SE

A

retinal toxicity, GI intolerance, rash, skin discoloration, anemia, cytopenias, myopathy, CNS stimulation, death (overdose)

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26
Q

Infliximab treats

A

HIA, spondyloarthropatyh, uveitis, sarcoidosis

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27
Q

Cyclophosphamid used to treat

A

SLE, vasculitis, JDMS, pulmonary hemorrhage

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28
Q

cyclophosphamid SE

A

nausea, vomiting, myelosuppresion, mucositis, hyponatremia, alopecia, hemorrhagic cystitis, gonadal failure, teratgoenesis, secondary malignancy

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29
Q

What needs to me monitored on hydroxychloroquine

A

EYES for retinal toxicity every 6-12mo

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30
Q

Infliximab is used to treat

A

polyarticular JIA, Bechest syndrome, uveitis, sarcoidosis
And IBD

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31
Q

Adverse effects of infliximab

A

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

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32
Q

Rituximab is

A

chimeric antibody to the antigen CD20 which is a transmembrane protein on the surface of B cell precursors and mature B lymphocytes

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33
Q

Rituximab SE

A

serious infusion reactions cytopenias, hep B virus reactivation, hypogammaglobulinemia, infections, serum sickness, vasculitis and progressive multifocal luekoencephaloptahy

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34
Q

Anakinra is used to treat

A

sJIA and other autoinflammatory syndromes

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35
Q

Criteria for JIA

A

○ 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

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36
Q

Systemic JIA criteria

A

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

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37
Q

Psoriatic arthritis criteria

A

Psorisasis and arthritis or arthritis and 2 of the following: dactylitis, nal bed changes, psoriasis in first degree relative

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38
Q

Enthesitis related arthritis criteria

A

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

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39
Q

Which joints are usually affected in oligoarthritis

A

Knees +++. ankles, fingers/wrist

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40
Q

Sex predominance in sJIA

A

EQUAL

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41
Q

ANA positive is correlated with

A

uveitis, younger age of disease onset, female sex, asymmetric arthritis and fewer involved joints over time

42
Q

Koebner phenomenon

A

cutaenous hypersensitivty where classic lesions are brough on by superficial trauma
seen in sJIA rash

43
Q

Is ESR high or low in MAS

A

low because of hypofibrinogemia and hepatic dysfunction

44
Q

Classification for sJIA associated MAS

A

hyperferritinemia and any 2 of thrombocytopenia, hypofibrinogemia, elevated liver enzymes, hypertriglyceridemia

45
Q

Lab features MAS

A

○ Cytopenia
○ Abnormal liver function tests
○ Hypofibrinogenemia
○ Decreased ESR
○ Hypertriglyceridemia
○ Hyponatremia
○ Hypoalbuinemia
○ Hyperferritinemia
Elevated CD25 and CD 163

46
Q

Path features in MAS

A

Macrophage hemophagocytosis in the bone marrow aspirate

47
Q

Marker for more aggressive disease in JIA

A

anti-CCP and RF

48
Q

Treatment for oligoarthritis JIA

A

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

49
Q

Enthesitis related JIA features

A

○ 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

50
Q

DIP joint involvement is highly suggestive of ___ arthritis

A

psoriatic

51
Q

Axial spondyloarthritis criteria

A

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)

52
Q

Erythema nodosum, proderma gangrenosum, oral ulcers, abdominal pain, diarrhea, weight loss, fever, anorexia

A

IBD

53
Q

Arthritis in IBD

A

Most commonly polyarthritis affecting large and small joints
Less frequently arthritis of the axial skeleton

54
Q

Organisms causing reactive arthritis

A

○ Probable: chlamydia, shigella, salmonella, yersinia, campylobacter
Possible: Neisseria gonorrhae, mycoplasma, e. coli, cryptosporidium, entamoeba Histolytica, giardia, brucella, c. diff, strep pyogenes, chlamydia

55
Q

Triad of reactive arthritis

A

Classic triad of arthritis, urethritis and conjunctivitis is relatively uncommon in children

56
Q

Skin findings in reactive arthritis

A

keratoderma blenorrhagicum (scaly psoriasis like eruption on the feet), circinate balanitis, erythema nodosum

57
Q

Type of arthritis in post-strep

A

Typically oligoarticular, affecting lower extremity joints and symptoms can persist for months

58
Q

Boys 3-10 years of age are most often affected and have acute onset of severe pain in the hip, lasting approximately one wee

A

Transietn synovitis

59
Q

Viruses causing viral myositis

A

Influenza A and B and enteroviruses are the most common viruses implicated

60
Q

Poor outcomes in KD

A

Poor outcomes- young age, male gender, persistent fever, poor response to IVIG, lab abnormalities

61
Q

KD labs

A

○ 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

62
Q

Genes associated with CVID

A

Genes associated: ICOS, SH2DIA, TAC1

63
Q

Hallmark finding in FMF

A

Hallmark finding is erysipeloid erythematous rash overlying the ankle or dorsum of the foot

64
Q

Gene for FMF

A

Caused by autosomal recessive mutations in MEFV a gene encoding amino acid protein pyrin

65
Q

Treatment for FMF

A

colchicine

66
Q

Colchicine SE

A

○ SE: GI, dose related transaminitis, bone marrow suppression rare

67
Q

Complication of FMF

A

Most serious complication of FMF is amyloidosis

68
Q

inheritance cyclic neutropneia

A

Autosomal dominant

69
Q

Cyclic neutorpneia presentation

A
  • 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
70
Q

How to diagnosis cyclic neutropneia

A

Diagnosed by obtaining blood counts 3 times/week for 6-8 weeks

71
Q

Serum Sickness

A

Systemic, immune complex mediated hypersensitivity vasculitis classically attributed to the therapeutic administration of foreign serum proteins or other medications

72
Q

Drugs causing serum sickness

A

anti-tetanus toxoid, infliximab, rituximab, etanercept, anti HIV antibodies, antibiotics (penicillin, septra, minocycline, meopnemen), neuro (carbamazepine, phenytoin, sulfonamides, barbiturates)

73
Q

What type of reaction is serum sickness

A

class 3

74
Q

Inv in serum sickness

A

Inv: often low plt, ESR and CRP elevated, mild proteinuria, complement studies are usually decreased

75
Q

Clinical ft serum sickness

A
  • 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
76
Q

Treatment for serum sickness

A
  • Treatment is supportive, removal of offending agent, antihistamines for pruritus, NSAIDs and analgesics
    Can use short course of steroids if very severe symptoms
77
Q

Do ANA titers correlate with disease severity in SLE

A

No

78
Q

Anti-ds DNA

A
  • Specific for diagnosis of SLE
  • Correlates with disease activity, esp nephritis in some with SLE
    84-100%
79
Q

Anti-Smith antibody

A

Specific for the diagnosis of SLE
Does not correlate with disease severity
Less common 23-48%

80
Q

Anti-ribonucleoprotein antibody

A

Increased risk for Raynaud phenomenon, ILD and pulmonary hypertension 31-62%

81
Q

Anti-RO antibody (anti-SSA antibody)

A

Associated with Sicca syndrome 38-54%

82
Q

Anti-La antibody (Anti-SSB antibody)May suggest diagnosis of Sjogrens syndrome

A

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%

83
Q

Antiphospholipid antibodies

A

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

84
Q

Anti-histone antibodies

A

Present in majority of patients with drug induced lupus (00%)
May be present in SLE

85
Q

Anti Ribosomal P antibodies

A

Found more frequently in children and are associated with neuropsychiatric, renal and hepatic involvement

86
Q

RPR can be false positive in

A

Can be false positive in: SLE, other autoimmune disorders, HIV, OVDU, chronic liver disease

87
Q

Positive p-ANCA

A
  • Specific but not sensitive for UC
    • Eosinophilic granulomatosis with polyangiitis
    • Primary sclerosing cholangitis
    • Microscopic polyangiitis
    • Focal necrotizing and crescentering GN
      Rheumatoid arthritis
88
Q

Positive anti-LKM antibody

A

AIH type 2

89
Q

ARF criteria

A

Need 2 major or 1 major and 2 minor
Major= JONES
MINOR= fever, arthralgias, prolonged PR, elevated ESR/CRP

90
Q

Valves affected in ARF carditis

A

Mitral valve is most commonly affected, followed by aortic

91
Q

Rash in ARF

A

erythema marginatum

92
Q

Sydenham’s chorea

A

Facial grimace, protruding tongue (wormian tongue), irregular contractures of hand muscles on active grip (milkmaids grip)

93
Q

Drugs for drug induced lupus

A

hydralazine, minocycline, anticonvulsants, sulfonamides, antiarrhythmic agents

94
Q

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

A

Periodic Fever, Apthous Stomatitis, pharyngitis and adenitis (PFAPA)

95
Q

Treatment for PFAPA

A

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

96
Q

Criteria for PFAPA

A

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

97
Q

HLA alleles for JDM

A

HLA alleles such as B8, DRB1, DQA1 are associated with increased risk for JDM

98
Q

Diagnosis for JDM

A

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

99
Q

EMG in JDM shows

A

EMG shows signs of myopathy (increased insertional activity, fibrillations and sharp waves) as well as muscle fiber necrosis (decreased action potential amplitude and duration)

100
Q

Biopsy in JDM shows

A

Biopsy shows focal necrosis, phagocytosis of muscle fibers, fiber regeneration, endomysial proliferation, inflammatory cell infiltrates or vasculitis, tubulovesicular bodies within endothelial cells

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