MSK II Final > McGowan: Connective Tissue Diseases > Flashcards

McGowan: Connective Tissue Diseases Flashcards

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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
Scleroderma
- 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
26
tx for scleroderma
-controls sx and slows progression to improve quality of life and prolong survival
27
Diffuse scleroderma
- systemic - diffuse involvemtn - including proximal extremities and trunk - early and progressive internal organ involvement: especially kidney, cardiac, and interstitial lung disease - worst prognosis
28
Limited scleroderma
- fingers, toes, face, disatal extremities - raynaudes commonly precedes other sx - pulmonary htn - CREST syndrome - indolent course - good prognosis
29
What is CREST syndrome
- calcinosis cutis - Ranaud's (secondary): usually first sx - esophageal dysmotility - sclerodactyly - telangiectasia
30
Localized scleroderma
- 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
31
Scleroderma (SSc) serology
- +ANA - Diffuse cutaneous: + anti Scl 70 ( anti DNA topoisomerase 1, anti RNA polymerase 3 - Limited cutaneous: + anti centromere
32
Is there a tx for scleroderma?
no not yet | -so just manage organ system involvement
33
In limited cutaneous systemic sclerosis, what will we find?
- long standing h/o raynaud's | - vascular manifestations are more pronounced with digital ischemia and progressive pulmonary artery Htn
34
Which kind of cutaneous sytemic sclerosis do we find renal crisis?
- diffuse kind | - may see hemolytic anemia on labs during renal crisis
35
What things will we find with the skin in scleroderma?
- hyper/o pigmented - dry and itchy (glands obliterated b y fibrosis) - masklike facies/wrinkles - calcium deposits - raynaud's
36
GI things with scleroderma
- 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
37
What antibody will we find in primary biliary cirrhosis?
-anti mitochondrial ab
38
What is the primary cause of morbidity and mortality in scleroderma?
- Pulmonology - Interstitial lung disease: diffuse - Pulmonary artery htn (PAH): limited - use right heart cath to confirm dx - increased incidence of bronchoalveolar CA
39
Renal things with SSc
- CKD - renal crisis uncommon but life-threatening - abrupt onset of malignant htn, hemolytic anemia, and progressive renal insufficiency
40
MSK things with SSc
- carpal tunnel syndrome | - hypothyroid from thyroid fibrosis
41
Cardiac things with SSc
- Myocardial fibrosis - carditis - pericardial effusion
42
Sjogren syndrome
- females - Sicca sx: immune mediated dyfunction of lacrimal and salivary glands... dry everything - keratoconjuctivitis sicca - inflammatory destruction of exocrine glands
43
What cancer has a strong associated with Sjogren's?
-B cell non hodgkin lymphoma
44
Dx for sjogrens?
- Lip biopsy - reveals characteristic lymphoid foci in accessory salivary glands - essential for dx
45
Serology for sjogrens
- +ANA - +RF - + hypergammaglobulinemia - + anti SSA/Ro - + anti SSB/La (never present without Ro)
46
tx of sjogrens
- symptomatic - Artificial tears - no immunomodulatory drug has proved efficacious
47
Inflammatory myopathies
- 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
Typical drmal features of inflammatory myopathies
- Gottron's patches/papules: raise violaceous lesions overlying the dorsa of DIP, PIP, and MCP joint - heliotrope rash - periungual erythrema - V neck erythema
49
Dermatomyositis (DM)
-weakenss w/o sensory sxs****** -increased risk of occult malignancy -
50
What is the characteristic skin lesions of DM?
- heliotrope rash: periorbital edema, purplish suffusion over eyelids - Shawl sign: erythema over neck/shoulders, upper chest and back
51
Dx of DM
- bx: perimysial and pervascular inflammation - ****perifascicular atrophy***** - elevated CK, Aldolase - Anti Jo-1, anti Mi2, and anti-MDA5, anti-P155/P140
52
Malignancies associated with DM
- 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
Polymyositis
- subacute prox muscle weaness - NO SKIN CHANGES (there are skin changes with DM, but not this ) - Elevated serum CK - Anti-Jo-1
54
What will we see on muscle bx of Polymyositis
-ENDOmysial inflammation with invasion of non-necrotic muscle fibers without features suggestive of another dx
55
Management of DM/PM
- Corticosteroids (bolded) - MTX - azathioprine......
56
Inclusion body myositis (IBM)
- white males - finger flexion or quadriceps weakness - CK is mild elevation or normal
57
what will we see on muscle bx for IBM?
- endomysial inflammation - rimmed vacuoles - invasion of non-necrotic muscle fibers - anti-cN1A autoantibodies
58
Tx of IBM
-refractory to tx, so tx is just supportive
59
Takayasu Arteritis
- 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
Dx of takaysu
MRI or CT angiography | -histology: granuloma with some giant cells, fibrosis in chronic stages
61
Tx of takayasu
-Glucocorticoids
62
What will we see on fundoscopic exam for takayasu arteritis
-Copper -wiring fundoscopic
63
IgAV aka Henoch schonlein purpura
- palpable purpura - no thrombocytopenia - arthritis - abdominal pain - glomerulonephritis - any age, more common in kids - Bx:IgA deposits - Tx: supportive/ glucocorticoids
64
Anti GBM
- glomerular capillaries - pulmonary capillaries - deposition of anti-basement membrane autoantibodies in basement membrane
65
BEehcet syndrome
- 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
What is the triad wtih BEhcet syndrome-
- recurrent mouth ulcers - genital ulcers - Eye inflammation (uveits)
67
In Behcet syndrome, what is pathergy?
-pustules at site of sterile needle pricks
68
Polarteritis nodosa
- medium vessel/segmental (may be ANCA+) - associated with HBV - males - fever, malaise, weight loss - foot drop - *****lungs are spared****
69
What will we find on bx with PAN
-infiltration and destruction of blood vessels by inflammatory cells.... fibrinoid necrosis, NO GRANULOMAS
70
What will angiogram show us for PAN?
- micro-aneurysm | - ANCA negative, (check HBsAg and HBeAg)
71
tx for PAN
-corticosteroids
72
Kawasaki disease
- 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
Tx of kawasaki disease
-IVIG within 10 days of sxs and high dose ASA.... yes, aspirin in a pediatric patient
74
GPA (granulomatosis with polyangiitis: aka Wegener's Granulomatosis)
- small vessel - ANCA+ (cANCA aka PR3-ANCA) - respiratory tract (90% nasal involvement) - alveolar hemorrhage - CSR: inflitrate/nodules/cavitary lesions - kidney involvement *****
75
What nose do they have with GPA?
-saddle nose deformity
76
What are the hallmarks of GPA?
- Granulomatous inflammation - necrotizing vasculitis - segmental glomerulonephritis
77
Tx of GPA
- cyclophosphamide and high dose glucocorticoids or rituximab - MTX can be considered if renal function is normal
78
Eosinophilic granulomatosis with polyangiitis (aka EGPA or Churg strauss syndrome)
- 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
Hallmarks of EGPA?
-asthma+eosinophilia.... vasculitis
80
Thromboangiitis obliterans (aka Buerger disease)
- medium vessel - young males (<35 yo) - *only occurs in smokers***** - distal vessels.... then proximal - thrombosis.... loss of digits... hands/feet - segmental disease
81
Dx of thromboangiitis obliterans
-angiography "corkscrew" appearance
82
Tx of thromboangiitis obliterans
-Stop smoking (glucocorticoids and anticoagulation doesn't work)
83
Primary raynaud
- benign, symmetric - exaggerated physiologic response to cold or emotion - women - younger ppl - thumbs rarely affected - Naifold capillaraoscopy normal in primary
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secondary raynaud
- 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
tx for raynaud
-wear freaking gloves -lotions -stop smoking -limit use or stop sympathomimetic drugs -calcium channel blockers -surgery -
86
Polymyalgia rheumatica (PMR) and Giant cell arteritis (GCA)
- women - >40 - white - fequently co exist - contituition sx: fever, malaise, weight loss, normal WBC count
87
GCA (aka temporal arteritis)
- Crainal arteries, aortic arch (large vessels) - headache, jaw claudication, PMR, visual abnormalities, and raised ESR - northern europena ancestry - associated with PMR
88
What genetic thing goes with GCA?-
-HLA-DR4
89
What is the gold standard for dx of GCA?
- temporal artery bx | - segmental granulomatous vasculitis with multinucleated giant cells
90
What do we do right away if someone has GCA?
-sart corticosteroids (before biopsy)... dramatic improvement
91
What happens if GCA goes on untreated?
-blindness
92
Polymylagia Rhuematica
- 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
What is the most likely cause of mortality in a lupus patient?
-Atherosclerosis
94
IN tachyasu arteritis, what is the patient at highest risk of developing?
-Aortic rupture
MSK II Final
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