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Clin Med MSKII > McGowan > Flashcards

McGowan Flashcards

1
Q

what titer level of ANA is normal/negative

A

-<1:40 . .. so higher ration like 1:160 is +

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2
Q

Homogenous ANA staining

A

Drug induced SLE

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3
Q

Speckled staining pattern of ANA

A

Sjogren’s

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4
Q

Centromere ANA staining pattern

A

-Limited systemic sclerosis (CREST)

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5
Q

Nucleolar ANA staining pattern

A

-Diffuse Systemic sclerosis

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6
Q

multisystem involvement of SLE

A
  • butterfly rash
  • Pleural effusions
  • Heart problems
  • Lupus nephritis
  • arthritis
  • Raynaud’s
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7
Q

Serology of SLE?

which correlates with disease activity

A
  • ANA
  • Anti-ds DNA (DISEASE ACTIVITY)
  • Sm
  • low C3 and C4: increased consumptions
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8
Q

SLE and anti-phospholipid antibody syndrome (APS)

A
  • 1/3 of SLE pts have APS

- treat with anticoagulation

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9
Q

what are the 3 types of antiphospholipid antibodies

A
  • type 1: causes biologic false + syphilis test
  • type 2: Lupus anticoagulant- risk factor for venous and arterial thrombosis and miscarriage, prolongs aPTT, presence confirmed by an abnormal Russel viper venom time (RVVT)
  • Type 3: anti cardiolipin antibodies (beta2GPI)
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10
Q

Cotton wool spots

A

SLE/APS retinopathy

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11
Q

serology for drug induced lupus like syndrome

A
  • ANA

- Anti-histone antibodies

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12
Q

Neonatal lupus affects children born of mothers with what Abs

A
  • Ro (SSA)

- La (SSB)

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13
Q

major complication of Neonatal Lupus

A

-permanent complete heart block

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14
Q

Treatment of SLE

A
  • Avoid sun exposure, wear sunscreen
  • NSAIDs
  • Corticosterioids
  • Hydroxychloroquine
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15
Q

what type of hypersensivity is SLE

A
  • type 3

- immune complex mediated

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16
Q

hallmark of Scleroderma (systemic sclerosis)

A
  • thickening and hardening of the skin

- microangiopathy and fibrosis of the skin and visceral organs

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17
Q

3 types of scleroderma

A
  • Diffuse
  • Limited (CREST: calcinosis cutis, Raynauds secondary, esophageal dysmotility, sclerodactyly, telangiectasia)
  • Localized
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18
Q

Serology for Diffuse scleroderma

A
  • Anti-Scl 70 aka anti DNA topoisomerase I

- Anti-RNA polymerase III

19
Q

Serology for Limited cutaneous scleroderma

A

-Anti-centromere

20
Q

GI effects of scleroderma

A
  • malnutrition: fat, protein, B12, and vitamin D deficiency
  • GERD –>Barrett Esophagus –> increased risk of esophageal adenocarcinoma
  • GAVE aka watermelon stomach
21
Q

pulmonary effects of scleroderma

A
  • primary cause of morbifity and mortality
  • interstitial lung disease: diffuse . .dry Velcro crackles
  • Pulmonary artery HTN: limited
  • right heart cath to confirm dx
  • increased incidence of bronchoalveolar carcinoma
22
Q

Renal effects of scleroderma

A
  • Chronic kidney disease
  • Renal crisis uncommon but life threatening: abrupt onset of malignant HTN, hemolytic anemia, and progressive renal insufficiency
  • more common in DIFFUSE scleroderma
  • Treatment: avoid high dose corticosteroids
23
Q

MSK and other effects of scleroderma

A
  • Carpal tunnel syndrome

- Hypothyroid from thyroid fibrosis

24
Q

highlights for Sjogren

A
  • Sicca symptoms (immune mediated dysfunction of lacrimal and salivary glands
  • Keratoconjunctivitis sicca (foreign body sensation-inadequate tear production)
  • Strone association with B cell non Hogkin lymphoma
25
Q

Lip biopsy in Sjogren

A
  • characteristic lymphoid foci in accessory salivary glands

- essential for diagnosis

26
Q

Serology for Sjogren-

A
  • ANA
  • RF
  • Hypergammaglobulinemia
  • anti SSA/Ro: may lead to newborn complete heart block
  • Anti SSB/La: never present without Ro
27
Q

Highlights for inflammatory myopathies

A
  • Symmetrical bilateral proximal muscle weakness: difficulty rising from chair or bathtub or climbing stairs
  • elevated CK and aldolase
  • characteristic muscle biopsy
28
Q

Highlights for Dermatomyositis

A
  • Heliotrope rash
  • “shawl sign”: erythema over neck/shoulders, upper chest and back
  • increased risk of occult MALIGNANCY
  • Dx: biopsy-perimysial and perivascular inflammation . . Perifascicular atrophy
29
Q

serology for Dermatomyositis

A
  • Elevated CK, aldolase

- Anti Jo-1, anti-Mi2, and anti-MDA5, anti-P155/140

30
Q

Malignancies and Dermatomyositis

A
  • Ovarian most common

- check transvaginal US, CT abd/pelvis, CA-125

31
Q

highlights for polymyositis

A
  • NO skin changes like Dermatomyositis
  • elevated CK
  • Anti-Jo-1
  • muscle bx: endomysial inflammation
32
Q

management of dermatomyositis and polymyositis

A

Corticosteroids

33
Q

highlights for inclusion body myositis

A
  • Males more
  • Finger flexion and quad weakness
  • CK is mild elevation or normal
  • Muscle bx: endomysial inflammation, RIMMED VACUOLES, invasion of non-necrotic muscle fibers, ANTI-cN1A autoantibodies
34
Q

highlights for Takayasu Arteritis

A
  • Asian less than 40
  • Large vessel
  • long smooth tapered stenosis
  • “pulseless disease”: obliterate UE peripheral pulses, collaterals so limb loss from ischemia is rare
  • Diagnosis: MRI or CT angiography
  • Histology: granuloma with some giant cells
  • Tx: Glucocorticoids
35
Q

highlights for Behcet Syndrome

A
  • Silk route: Turkey, Asia, Mid East
  • HLA-B51
  • Triad: recurrent mouth ulcers, genital ulcers, eye inflammation (uveitis)
  • Large vessel=aneurysm
  • Venous involvement: DVT
  • Pathergy: pustules at site of sterile needle pricks
  • 50% pulmonary involvement
  • Retinopathies
36
Q

highlights for Polyarteritis Nodosa

A
  • associated with HBV
  • males more
  • 80% have vasculitis neuropathy . .foot drop
  • LUNGS SPARED
  • Bx: infiltration and destruction of blood vessels by inflammatory cells–>fibrinoid necrosis, NO granulomas
  • Angiogram: micro aneurysm
  • ANCA negative, check HBsAg and HBeAg
37
Q

highlights for Kawasaki

A
  • <5yr
  • Asian
  • “mucocutaneous lymph node syndrome”
  • “strawberry tongue”
  • Death: from coronary involvement (aneurysm or MI-can occur years later)
  • Tx: IVIG w/in 10 days of sxs and high dose ASA . . YES ASPIRIN in a pediatric pts
38
Q

highlights for Granulomatosis with polyangiitis: aka Wegener’s Granulomatosis

A
  • C-ANCA aka PR3-ANCA
  • respiratory tract (Saddle nose)
  • Chest XR: cavitary lesions
  • kidney involvement
  • Hallmarks: Granulomatous inflammation, Necrotizing vasculitis, Segmental Glomerulonephritis
39
Q

highlights for Eosinophilic Granulomatosis with polyangiitis aka Churg-Strauss Syndrome

A
  • ANCA + in 50% . .typically MPO-ANCA
  • Granulomas with eosinophilia
  • Hallmarks: asthma + eosinophilia
40
Q

Highlights for Thromboangiitis Obliterans aka Buerger Disease

A
  • young males <35
  • only occurs in smokers
  • Dx: angiography-“corkscrew” appearance
  • Tx: STOP SMOKING
41
Q

Secondary Raynaud

A
  • more severe than primary . .ischemia
  • unilateral (primary is bilateral)
  • Nailfold Capillaroscopy: distorted with widened and irregular loops, dilated lumen and areas of vascular “dropout” . . normal in primary
42
Q

what frequently occurs with polymyalgia Rheumatica

A

Giant cell arteritis aka Temporal Arteritis

43
Q

highlight for Giant Cell arteritis aka Temporal Arteritis

A
  • Cranial arteries (temporal/facial/ophthalmic)
  • Aortic arch
  • Headache, Jaw claudication, visual abnormalities
  • super high ESR . . >50 mm/h
  • HLA-DR4
  • associated with polymyalgia rheumatic
  • Temporal artery bx (gold standard for dx: need a 1 cm segment)
  • multinucleated giant cells
  • Start corticosterioids before biopsy .. if no treatment then blindness
44
Q

highlights for polymyalgia Rheumatica

A
  • Associated with giant cell arteritis
  • Stiffness, soreness and pain
  • weakness as result of pain
  • NO inflammation on muscle bx
  • muscle enzymes and EMG normal
  • elevated ESR and CRP

Clin Med MSKII (16 decks)

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