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MSK II Final > McGowan: Connective Tissue Diseases > Flashcards

McGowan: Connective Tissue Diseases Flashcards

1
Q

What lab value will autoimmune disease have?

A
    • ANA
  • NOT SPECIFIC for individual AI disorders
  • Titer of <1:40 is normal
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2
Q

What is cANCA?

A

-PR3-ANCA

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3
Q

What is pANCA

A

-MPO-ANCA

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4
Q

What does a homogenous ANA staining pattern mean?

A
  • the entir nucleus is diffusely staidned
  • Antibody includes those directed against chromatin, histone, proteins, and DNA
  • Drug induced SLE
  • Sjogren’s
  • SLE
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5
Q

What does speckled staining pattern mean?

A
  • fine or caorse speckles are seen throughout the nucleus
  • many different antibodies, including those directed against UI RNP, Sm, and La antigens
  • -Mixed CT disease
  • Diffuse Systemic sclerosis
  • Sjogren’s syndrome
  • SLE
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6
Q

What does the Centremromere pattern mean?

A
  • refers to the presence of 30 to 60 uniform speckles distributed throughout the nucleus of resting cells
  • inmitotic cells, the speckles localize to the chromosomes at the metaphase plate
  • Limited systemic sclerosis (CREST)
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7
Q

Nucleolar pattern

A
  • refers to homogenous or speckled staining of the nucleoulus
  • Ab directed against RNA
  • Diffuse systemic sclerosis
  • SLE
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8
Q

What will we see in SLE?

A
  • Pleural effusions
  • Heart problems
  • Lupus nephritis
  • Arthritis
  • Raynaud’s phenomenon
  • butterfly rash
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9
Q

What are the antibodies directed against in SLE?

A
  • Auto antibodies to nuclear antigens

- immune complexes (Type 3 hypersensitivity

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10
Q

SLE serology

A
  • +ANA
  • +anti- ds DNA
  • +Sm
  • Complement activation promotes inflammation
  • C3 or C4 lowered, meaning increased consumption: suggests disease activity
  • returns towards normal when in remission
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11
Q

Anti phospholipid antibody syndrome (APS)

A
  • 1/3 of SLE patients
  • can also be present w/o a dx of SLE
  • 3 types of antibodies
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12
Q

Tx of APS

A
  • Warfarin to acheive INR of 2-3
  • Pregnancy= low molecular weight heparin + aspirin
  • ***Warfarin contraindicated in preggo!
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13
Q

Type one Antiphospholipi antibody

A

-causes biologic false-positive tests for syphilis!

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14
Q

Type 2

A
  • Lupus anticoagulant
  • risk factor for venous and arterial thrombosis and miscarriage
  • prolongation of the activated partial thromboplastin time (aPTT)
  • presence is confirmed by an abnormal Russell viper venom time (RVVT) that corrects with addition of phosphohlipid but not normal plasma
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15
Q

Type 3

A
  • Anti-cardiolipin antibodies
  • directed at a serum cofactor beta-2-glycoptn I
  • Beta2GPI***
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16
Q

What will we see in the retina with SLE/APS

A

-cotton wool spots

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17
Q

What is the lupus-like syndrome/drug induced?

A
  • Promote demethylation of DNA
  • no renal or neuro symptoms
    • ANA
    • anti histone antibodies (95%)
  • **Sulfa abx…. SLE flare
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18
Q

What drugs will in induce the lupus like syndrome

A
  • hydralazine
  • isoniazid
  • Minocycline
  • TNG inhibitors
  • Quinine
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19
Q

Lupus: Prgnancy and neonatal

A
  • gestaional HTN, fetal growth restriction, fetal distress….fetal losss or premature delivery
  • Neonatal lupus
  • affects children born of mothers with Anti Ro (SSA) or La (SSB) Abs
  • Permanent complete heart block
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20
Q

What is a sign of transient neonatal lupus?

A
  • rashes
  • thrombocytopenia
  • hemolytic anemia
  • arthritis
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21
Q

Tx for SLE

A
  • Avoid sun exposure, wear sunscreen
  • NSAIDS
  • Corticosteroids
  • Hydroxycholoquine
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22
Q

SLE Ddx

A
  • RA
  • Systemic vasculitis
  • scleroderma
  • inflammatory myopathies
  • viral hepatitis
  • sarcoidosis
  • acute drug rxn
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23
Q

What kind of endocarditis will we see with SLE?

A
  • Libman sacks endocarditis
  • non infectious
  • causes detachment of the chordae tendonae it looks like
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24
Q

If someone has discoid lupus, what other diseases should we think about?

A
  • Tinea infeaction (ring worm)
  • Psoriasis
  • morphea (limited scleroderma)
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25
Q

Scleroderma

A
  • thickening and hardening of skin
  • microangiopathy and fibrosis of the skin and visceral organs
  • secondary raynaud phenomenon
  • Obliteration of eccrine sweat and sebaceous glands….dry itchy skin
  • no approved isease modifying therapy
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26
Q

tx for scleroderma

A

-controls sx and slows progression to improve quality of life and prolong survival

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27
Q

Diffuse scleroderma

A
  • systemic
  • diffuse involvemtn
  • including proximal extremities and trunk
  • early and progressive internal organ involvement: especially kidney, cardiac, and interstitial lung disease
  • worst prognosis
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28
Q

Limited scleroderma

A
  • fingers, toes, face, disatal extremities
  • raynaudes commonly precedes other sx
  • pulmonary htn
  • CREST syndrome
  • indolent course
  • good prognosis
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29
Q

What is CREST syndrome

A
  • calcinosis cutis
  • Ranaud’s (secondary): usually first sx
  • esophageal dysmotility
  • sclerodactyly
  • telangiectasia
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30
Q

Localized scleroderma

A
  • benign skin conditions
  • affects children
  • discrete areas of discolored skin induration
  • NO raynaud’s
  • NOT systemic
  • histologically indistinguishable form SSc
  • pathces= morphea
  • coalesced patches= generalized morphea
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31
Q

Scleroderma (SSc) serology

A
  • +ANA
  • Diffuse cutaneous: + anti Scl 70 ( anti DNA topoisomerase 1, anti RNA polymerase 3
  • Limited cutaneous: + anti centromere
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32
Q

Is there a tx for scleroderma?

A

no not yet

-so just manage organ system involvement

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33
Q

In limited cutaneous systemic sclerosis, what will we find?

A
  • long standing h/o raynaud’s

- vascular manifestations are more pronounced with digital ischemia and progressive pulmonary artery Htn

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34
Q

Which kind of cutaneous sytemic sclerosis do we find renal crisis?

A
  • diffuse kind

- may see hemolytic anemia on labs during renal crisis

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35
Q

What things will we find with the skin in scleroderma?

A
  • hyper/o pigmented
  • dry and itchy (glands obliterated b y fibrosis)
  • masklike facies/wrinkles
  • calcium deposits
  • raynaud’s
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36
Q

GI things with scleroderma

A
  • Malnutrition: fat, protein, B12 and vitamin D deficiency
  • Barret esophagus (high risk for esophageal adenoCA)
  • Gastric antral vascular ectrasia (GAVE): watermelon stomach
  • Primary biliary cirrhosis
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37
Q

What antibody will we find in primary biliary cirrhosis?

A

-anti mitochondrial ab

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38
Q

What is the primary cause of morbidity and mortality in scleroderma?

A
  • Pulmonology
  • Interstitial lung disease: diffuse
  • Pulmonary artery htn (PAH): limited
  • use right heart cath to confirm dx
  • increased incidence of bronchoalveolar CA
39
Q

Renal things with SSc

A
  • CKD
  • renal crisis uncommon but life-threatening
  • abrupt onset of malignant htn, hemolytic anemia, and progressive renal insufficiency
40
Q

MSK things with SSc

A
  • carpal tunnel syndrome

- hypothyroid from thyroid fibrosis

41
Q

Cardiac things with SSc

A
  • Myocardial fibrosis
  • carditis
  • pericardial effusion
42
Q

Sjogren syndrome

A
  • females
  • Sicca sx: immune mediated dyfunction of lacrimal and salivary glands… dry everything
  • keratoconjuctivitis sicca
  • inflammatory destruction of exocrine glands
43
Q

What cancer has a strong associated with Sjogren’s?

A

-B cell non hodgkin lymphoma

44
Q

Dx for sjogrens?

A
  • Lip biopsy
  • reveals characteristic lymphoid foci in accessory salivary glands
  • essential for dx
45
Q

Serology for sjogrens

A
  • +ANA
  • +RF
    • hypergammaglobulinemia
    • anti SSA/Ro
    • anti SSB/La (never present without Ro)
46
Q

tx of sjogrens

A
  • symptomatic
  • Artificial tears
  • no immunomodulatory drug has proved efficacious
47
Q

Inflammatory myopathies

A
  • myalgias
  • symmetrical bilateral proximal muscle weakness
  • difficulty rising form a chair or bathtub or climbing stairs
  • elevation of serum muscle enzymes
  • ESR and CRP often normal
  • Characteristic Muscle bx!!!!**
48
Q

Typical drmal features of inflammatory myopathies

A
  • Gottron’s patches/papules: raise violaceous lesions overlying the dorsa of DIP, PIP, and MCP joint
  • heliotrope rash
  • periungual erythrema
  • V neck erythema
49
Q

Dermatomyositis (DM)

A

-weakenss w/o sensory sxs****
-increased risk of occult malignancy
-

50
Q

What is the characteristic skin lesions of DM?

A
  • heliotrope rash: periorbital edema, purplish suffusion over eyelids
  • Shawl sign: erythema over neck/shoulders, upper chest and back
51
Q

Dx of DM

A
  • bx: perimysial and pervascular inflammation
  • perifascicular atrophy*
  • elevated CK, Aldolase
  • Anti Jo-1, anti Mi2, and anti-MDA5, anti-P155/P140
52
Q

Malignancies associated with DM

A
  • Ovarian is most commone
  • so make sure you check transvaginal US, CT, abd/pelvis, CA-125
  • Some malignancies may not be evident for months after the initial presentation of DM and initial screenings
53
Q

Polymyositis

A
  • subacute prox muscle weaness
  • NO SKIN CHANGES (there are skin changes with DM, but not this )
  • Elevated serum CK
  • Anti-Jo-1
54
Q

What will we see on muscle bx of Polymyositis

A

-ENDOmysial inflammation with invasion of non-necrotic muscle fibers without features suggestive of another dx

55
Q

Management of DM/PM

A
  • Corticosteroids (bolded)
  • MTX
  • azathioprine……
56
Q

Inclusion body myositis (IBM)

A
  • white males
  • finger flexion or quadriceps weakness
  • CK is mild elevation or normal
57
Q

what will we see on muscle bx for IBM?

A
  • endomysial inflammation
  • rimmed vacuoles
  • invasion of non-necrotic muscle fibers
  • anti-cN1A autoantibodies
58
Q

Tx of IBM

A

-refractory to tx, so tx is just supportive

59
Q

Takayasu Arteritis

A
  • large vessel: subclavian and innominate most common
  • long smooth tapered stenosis
  • pulseless disease
  • 50% pulmonary involvement
  • Retinopathy, renal artery stenosis, aortic dilation, aortic regurg, aneurysm, aortic rupture
60
Q

Dx of takaysu

A

MRI or CT angiography

-histology: granuloma with some giant cells, fibrosis in chronic stages

61
Q

Tx of takayasu

A

-Glucocorticoids

62
Q

What will we see on fundoscopic exam for takayasu arteritis

A

-Copper -wiring fundoscopic

63
Q

IgAV aka Henoch schonlein purpura

A
  • palpable purpura
  • no thrombocytopenia
  • arthritis
  • abdominal pain
  • glomerulonephritis
  • any age, more common in kids
  • Bx:IgA deposits
  • Tx: supportive/ glucocorticoids
64
Q

Anti GBM

A
  • glomerular capillaries
  • pulmonary capillaries
  • deposition of anti-basement membrane autoantibodies in basement membrane
65
Q

BEehcet syndrome

A
  • silk rout (turkey, asia, mid east
  • HLA -B51
  • large vessel=aneurysms
  • venous involvement…. DVT
  • large joint arthralgia
  • neuro involvement
  • ulcers in distal ileum or cecum
  • Tx: low dose glucocorticoids
66
Q

What is the triad wtih BEhcet syndrome-

A
  • recurrent mouth ulcers
  • genital ulcers
  • Eye inflammation (uveits)
67
Q

In Behcet syndrome, what is pathergy?

A

-pustules at site of sterile needle pricks

68
Q

Polarteritis nodosa

A
  • medium vessel/segmental (may be ANCA+)
  • associated with HBV
  • males
  • fever, malaise, weight loss
  • foot drop
  • **lungs are spared*
69
Q

What will we find on bx with PAN

A

-infiltration and destruction of blood vessels by inflammatory cells…. fibrinoid necrosis, NO GRANULOMAS

70
Q

What will angiogram show us for PAN?

A
  • micro-aneurysm

- ANCA negative, (check HBsAg and HBeAg)

71
Q

tx for PAN

A

-corticosteroids

72
Q

Kawasaki disease

A
  • Medium vessel
  • Mucocutaneous lympho noe syndrome
  • strawberry tongue***
  • DDx: TSS and scarlet fever
  • death from coronary involvement (aneurysm or MI- can occur years later)
73
Q

Tx of kawasaki disease

A

-IVIG within 10 days of sxs and high dose ASA…. yes, aspirin in a pediatric patient

74
Q

GPA (granulomatosis with polyangiitis: aka Wegener’s Granulomatosis)

A
  • small vessel
  • ANCA+ (cANCA aka PR3-ANCA)
  • respiratory tract (90% nasal involvement)
  • alveolar hemorrhage
  • CSR: inflitrate/nodules/cavitary lesions
  • kidney involvement *****
75
Q

What nose do they have with GPA?

A

-saddle nose deformity

76
Q

What are the hallmarks of GPA?

A
  • Granulomatous inflammation
  • necrotizing vasculitis
  • segmental glomerulonephritis
77
Q

Tx of GPA

A
  • cyclophosphamide and high dose glucocorticoids or rituximab
  • MTX can be considered if renal function is normal
78
Q

Eosinophilic granulomatosis with polyangiitis (aka EGPA or Churg strauss syndrome)

A
  • ANCA +: typically MP-ANCA
  • Granulomas (with eosinophilia)
  • prodromal phase: allergic disease… months to years
  • Eosinophilia-tissue infiltration phase
  • Vasculitis phase: palpable purpura.. ddx HSP
  • Resp tract and other organs
  • DDx: GPA
  • Tx: glucocorticoids
79
Q

Hallmarks of EGPA?

A

-asthma+eosinophilia…. vasculitis

80
Q

Thromboangiitis obliterans (aka Buerger disease)

A
  • medium vessel
  • young males (<35 yo)
  • only occurs in smokers**
  • distal vessels…. then proximal
  • thrombosis…. loss of digits… hands/feet
  • segmental disease
81
Q

Dx of thromboangiitis obliterans

A

-angiography “corkscrew” appearance

82
Q

Tx of thromboangiitis obliterans

A

-Stop smoking (glucocorticoids and anticoagulation doesn’t work)

83
Q

Primary raynaud

A
  • benign, symmetric
  • exaggerated physiologic response to cold or emotion
  • women
  • younger ppl
  • thumbs rarely affected
  • Naifold capillaraoscopy normal in primary
84
Q

secondary raynaud

A
  • occurs in CTD , hematologic and endocrine condtions, occupational disorders, use of beta blockers or cancer drugs cisplatin and bleomycin
  • > 30 yo typically
  • Unilateral
  • More severe: ischemia
  • Nailfold capillaroscopy: distorted with widened and irregular loops, dilated lumen and areas of vascular “dropout”
85
Q

tx for raynaud

A

-wear freaking gloves
-lotions
-stop smoking
-limit use or stop sympathomimetic drugs
-calcium channel blockers
-surgery
-

86
Q

Polymyalgia rheumatica (PMR) and Giant cell arteritis (GCA)

A
  • women
  • > 40
  • white
  • fequently co exist
  • contituition sx: fever, malaise, weight loss, normal WBC count
87
Q

GCA (aka temporal arteritis)

A
  • Crainal arteries, aortic arch (large vessels)
  • headache, jaw claudication, PMR, visual abnormalities, and raised ESR
  • northern europena ancestry
  • associated with PMR
88
Q

What genetic thing goes with GCA?-

A

-HLA-DR4

89
Q

What is the gold standard for dx of GCA?

A
  • temporal artery bx

- segmental granulomatous vasculitis with multinucleated giant cells

90
Q

What do we do right away if someone has GCA?

A

-sart corticosteroids (before biopsy)… dramatic improvement

91
Q

What happens if GCA goes on untreated?

A

-blindness

92
Q

Polymylagia Rhuematica

A
  • Associated with GCA
  • proximal severem symmetrical morning and daylong stiffness, soreness, and pain in shoulder, neck, and pelvic girdles
  • feelings of weakness a result of pain, no true weakness
  • no inflammation on muscle bx
  • muscle enzymes and EMG normal
  • elevated ESR and CRP
  • Tx: corticosteroids
93
Q

What is the most likely cause of mortality in a lupus patient?

A

-Atherosclerosis

94
Q

IN tachyasu arteritis, what is the patient at highest risk of developing?

A

-Aortic rupture

MSK II Final (13 decks)

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