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Ahmed MSK Pt.2 Sadness > Lecture 7: Connective Tissue Diseases > Flashcards

Lecture 7: Connective Tissue Diseases Flashcards

1
Q

Homogenous/diffuse ANA staining patterns may be associated with what 3 underlying conditions?

A
  • Drug-induced SLE**
  • Sjogren’s syndrome
  • SLE
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2
Q

What does the centromere pattern of ANA staining refer to and what condition is it seen in?

A
  • Presence of 30-60 uniform speckles distributed throughout the nucleus of resting cells
  • Limited systemic sclerosis (CREST)
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3
Q

A nucleolar pattern of ANA staining may be seen in which 2 conditions?

A
  • Diffuse systemic sclerosis
  • SLE
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4
Q

What are 3 positive serology findings seen in SLE; which is most specific and correlates with disease activity?

A
  • (+) ANA
  • (+) anti-ds DNA –> correlates w/ disease activity; most specific
  • (+) Sm (Smith)
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5
Q

What will be seen with complement activity during active SLE?

A

↓ C3 or C4 due to ↑ consumption

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6
Q

Anti-Phospholipid Antibody Syndrome (APS) arises in association with what?

A
  • 1/3 of SLE pt’s (secondary APS)
  • Can also be present w/o a dx of SLE (primary APS)
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7
Q

Of the 3 types of antiphospholipid antibodies, what is the significance of Type One?

A

Causes biologic false-(+) tests for syphilis

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8
Q

Of the 3 types of antiphospholipid antibodies, what is the significance of Type Two; how is it detected?

A
  • Lupus anticoagulant
  • Risk factor for venous and arterial thrombosis and miscarriage
  • Causes prolongation of aPTT
  • Presence is confrmed by an abnormal dilute Russell viper venom time (DRVVT)
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9
Q

Of the 3 types of antiphospholipid antibodies, what is the significance of Type Three?

A
  • Anti-cardiolipin antibodies
  • Directed at a serum co-factor Beta2GPI
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10
Q

Which 2 serology findings if positive correlates with lupus-like syndrome/drug-induced?

A
  • (+) ANA
  • (+) Anti-histone antibodies (95%)
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11
Q

Neonatal lupus affects children born of mothers with which serum antibodies?

A

Anti Ro (SSA) or La (SSB) Abs

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12
Q

Which complication may arise in children with neonatal lupus?

A

Permanent complete heart block

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13
Q

What are 4 treatment strategies/drugs used for SLE?

A
  • Avoid sun exposure, wear sunscreen
  • NSAIDs
  • Corticosteroids (topical or systemic)
  • Hydroxychloroquine
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14
Q

What is the hallmark finding of Scleroderma (systemic sclerosis)?

A
  • Thickening and hardening of the skin
  • Microangiopathy and fibrosis of the skin and visceral organs
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15
Q

Which antibodies are specific to diffuse cutaneous scleroderma?

A
  • (+) Anti-Scl 70 –> aka Anti-(DNA) topoisomerase I
  • Anti-RNA polymerase III
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16
Q

Which antibody is specific to the limited cutaneous type of scleroderma?

A

(+) Anti-centromere

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17
Q

Which type of imaging may be appropriate for working up Scleroderma?

A
  • CXR
  • Barium swallow if indicated
  • Hand x-rays may show distal tuft resorption and calcinosis
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18
Q

Which manifestations of disease are more pronounced in the limited cutaneous type of Scleroderma?

A
  • Vascular manifestations i.e., digital ischemia and progressive Pulmonary artery HTN
  • Think is limited to pulmonary a.; while diffuse is all over and caused interstitial lung disease
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19
Q

A patient with diffuse cutaneous systemic sclerosis (scleroderma) in renal crisis may have what lab finding?

A

Hemolytic anemia

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20
Q

What is localized scleroderma and most often affects whom?

A
  • Benign skin conditions; most often affects children
  • Discreet areas of discolored skin induration; patches = morphea
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21
Q

Deficiency of which nutrients is common in systemic sclerosis patients with GI involvement?

A

Fat + protein + B12 + vitamin D

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22
Q

Which esophageal manifestations may be seen with systemic sclerosis?

A

Strictures, Barret esophagus –> ↑ risk of esophagal adenocarcinoma

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23
Q

Which disease manifestations of the stomach may be seen in systemic sclerosis?

A
  • Gastric Antral Vascular Ectasia (GAVE) aka Watermelon stomach
  • Gastroparesis
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24
Q

Which biliary disease manifestation may be seen with systemic sclerosis; what is the associated antibody?

A

Primary biliary cirrhosis –> anti-Mitochondrial Ab

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25
Q

What is the primary cause of morbidity and mortality in patients with systemic sclerosis?

A

Pulmonary involvement

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26
Q

What type of pulmonary involvement is seen in the diffuse type of systemic sclerosis and how is it diagnosed/confirmed?

A
  • Interstitial lung disease
  • Dx by pulmonary function test (PFT)
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27
Q

What type of pulmonary disease is seen in the limited type of systemic sclerosis and how is it diagnosed?

A
  • Pulmonary artery hypertension (PAH)
  • Right heart catheter to confirm dx: mean pulm. artery pressure of 25 mmHg or greater
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28
Q

There is an increased incidence of which lung malignancy in those with systemic sclerosis?

A

Bronchoalveolar carcinoma

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29
Q

Which MSK and endocrine are often seen with systemic sclerosis?

A
  • Carpal tunnel syndrome
  • Hypothyroid from thyroid fibrosis
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30
Q

Sjögren syndrome has strong association with what malignancy?

A

B-cell Non-Hodgkin lymphoma

31
Q

What are the sicca sx’s associated with Sjögren syndrome; increased risk for what complication?

A
  • Immune-mediated destruction of lacrimal and salivary glands
  • Dry eyes + mouth (xerostomia) + vaginal dryness + tracheobronchial dryness
  • ↑ incidence of oral infections (candida) + dental caries
  • Parotid or other major salivary gland enlargement
32
Q

What type of biopsy is essential for the diagnosis of Sjögren syndrome; what is seen?

A

Lip biopsy –> reveals characteristic lymphoid foci in accessory salivary glands

33
Q

What serological findings are characteristic of Sjögren syndrome?

A
  • Hypergammaglobulinemia
  • (+) Anti SSA/Ro
  • (+) Anti SSB/La = never present without Ro
34
Q

Which type of medicated drops may help the dry eye sx’s of Sjögren syndrome?

A

Cyclosporine drops

35
Q

Which 2 medications may help the sicca manifestations of Sjögren syndrome?

A

Pilocarpine and Cevimeline

36
Q

Inflammatory myopathies are associated with what muscle sx’s?

A
  • Myalgias
  • Symmetric bilateral proximal muscle weakness
  • Difficulty rising from a chair or bathtub or climbing stairs
37
Q

What is the characteristic biopsy finding of Dermatomyositis?

A

Perimysial and perivascular inflammation, perifascicular atrophy

38
Q

What are the most common malignancies associated with Dermatomyositis?

A
  • Ovarian: check transvaginal US, CT abd/pelvis, CA-125
  • Lung
  • Pancreatic
  • Stomach
  • Colorectal
  • NHL
39
Q

Which serology findings will be positive in Polymyositis?

A
  • Elevated serum CK
  • Anti-Jo-1
40
Q

What are the steps to tx of the inflammatory myopathies, DM and PM?

A
  • Step 1: Glucocorticoids (3-4 wks, then tapered very gradually)
  • Step 2: Approximately 75% of pt’s require additional therapy with other immunosuppressive drugs
  • Step 3: IV immunoglobulin
  • Step 4: a trial of one of the following: rituximab, cyclosporine, cyclophosphamide, or tacrolimus
41
Q

Which sex and ethnicity are most often affected by Inclusion Body Myositis (IBM)?

A

Males > F; more often Caucasians

42
Q

What are characteristic sign/sx’s of Inclusion Body Myositis (IBM); what are the serum CK levels like?

A
  • Finger flexion or quadriceps weakness
  • CK is mild** or **normal
43
Q

What is the characteristic finding on muscle biopsy in Inclusion Body Myositis (IBM); which autoantibodies may be present?

A
  • Endomysial inflammation, rimmed vacuoles, invasion of non-necrotic muscle fibers
  • Anti-cN1A autoantibodies
44
Q

What are the characteristic gross vascular lesions of Takayasu Arteritis; what is seen histologically?

A
  • Long smooth tapered stenosis
  • Granuloma w/ some giant cells, fibrosis in chronic stages
45
Q

What are some of the complications of Takayasu Arteritis?

A
  • 50% have pulmonary involvement
  • Retinopathy, Renal artery stenosis, Aortic dilations, Aortic regurgitation, Aneurysm, and Aortic rupture
46
Q

How is diagnosis of Takayasu Arteritis made; what is the tx?

A
  • Dx: MRI or CT angiography
  • Tx: Glucocorticoids
47
Q

What is a characteristic fundoscopic finding in pt with Takayasu Arteritis?

A

Copper-wiring

48
Q

What is the triad of Behcet Syndrome?

A
  • Recurrent mouth ulcers
  • Genital ulcers
  • Eye inflammation (uveitis)
49
Q

What is Pathergy seen in the setting of Behcet Syndrome?

A

Pustules at site of sterile needle pricks

50
Q

Which HLA is Behcet Syndrome associated with?

51
Q

What are 2 vessel complication which may arise in the setting of Behcet Syndrome?

A
  • Large vessel = aneurysms
  • Venous impairment —> DVT
52
Q

Polyarteritis nodosa may be associated with what infectious agent?

53
Q

Which neurological manifestations may be seen in the setting of Polyarterisitis Nodosa?

A
  • Peripheral nerve: 80% have vasculitis nephropathy
  • Mononeuritis multiplex: foot drop
54
Q

Which organ system is characteristically spared in Polyarteritis Nodosa?

55
Q

How is diagnosis of Polyarteritis made; what is seen?

A
  • Bx: infiltration and destruction of blood vessels by inflammatory cells –> fibrinoid necrosis, NO granulomas
  • Angiogram: microaneurysms
56
Q

If patient with Polyarteritis Nodosa is ANCA negative, what should you check for?

A

HBsAg and HBeAg

57
Q

Which serum marker is positive in granulomatosis with Polyangiitis (Wegener’s)?

A

C-ANCA aka PR3-ANCA

58
Q

What are the 3 hallmarks of granulomatosis with Polyangiitis (Wegener’s)?

A
  • Granulomatous inflammation
  • Necrotizing vasculitis
  • Segmental glomerulonephritis (hematuria/RBC/proteinuria)
59
Q

What are the upper and lower respiratory tract manifestations which may be seen in setting of granulomatosis with Polyangiitis (Wegener’s); what may be seen on CXR?

A
  • 90% have nasal involvement: saddle nose, crusting, bleeding, obstruction
  • Lung: Asx nodule –> alveolar hemorrhage
  • CXR: infiltrate/nodules/cavitary lesions
60
Q

Which serum marker is positive in some cases of eosinophilic granulomatosis with Polyangiitis (Churg-Strauss)?

A

MPO-ANCA aka P-ANCA

61
Q

What is the hallmark of eosinophilic granulomatosis with Polyangiitis (Churg-Strauss)?

A

Asthma + Eosinophilia –> Vasculitis

62
Q

What is the characteristic finding on angiography of Thromboangiitis Obliterans (aka Buerger Disease)?

A

“Corkscrew” appearance

63
Q

Which finding on nailfold capillaroscopy is characteristic of secondary raynaud’s?

A

Distorted with widened and irregular looop, dilated lumen and areas of vascular “dropout”

64
Q

Which arteries most often affected in Giant Cell Arteritis (aka Temporal arteritis)?

A
  • Cranial arteries (temporal/facial/opthalmic)
  • Aortic arch
65
Q

Which HLA is associated with Giant Cell Arteritis (aka Temporal arteritis)?

66
Q

What are the signs/sx’s and characteristic lab findings of Giant Cell Arteritis (aka Temporal arteritis)?

A
  • HA + jaw claudication + PMR + visual abnormalitis (amaurosis fugax or diplopia)
  • ↑↑ ESR (>50 mm/h)
67
Q

What is the gold standard for diagnosis of Giant Cell Arteritis (aka Temporal Arteritis); what is seen?

A
  • Temporal artery bx: need a 1.0 cm segment
  • Segmental granulomatous vasculitis w/ multinucleated giant cells
68
Q

What is an important thing to remember about the tx of Giant Cell Arteritis (aka Temporal Arteritis)?

A

Start corticosteroids (before biopsy!) –> dramatic improvement

69
Q

What are the signs/sx’s of Polymyalgia Rheumatica and what is it associated with?

A
  • Proximal severe symmetrical morning and daylong stiffness, soreness, and pain in shoulder, neck, and pelvic girdles
  • “Feelings” of weakness as a result of pain (as opposed to PM), no true weakness
  • Associated with Giant Cell Arteritis (aka Temporal Arteritis)
70
Q

What is seen on muscle biopsy and in the serum of patient with Polymyalgia Rheumatica?

A
  • NO inflammation on muscle bx
  • Muscle enzymes and EMG = normal
  • ↑↑↑ ESR and CRP
71
Q

What are the cardiopulmonary complications which may arise in SLE?

A
  • Pleuritis + pericarditis + myocarditis + endocarditis
  • Pt’s also at ↑ risk of MI usually due to accelerared atherosclerosis
72
Q

Which complication may arise in the eye associated with SLE/APS?

A

Retinopathy: cotton wool spots

73
Q

Which antibody is seen in cases of polymyositis w/ pneumonitis + arthritis?

74
Q

Henoch-Schonlein Purpura is associated with what underlying signs/sx’s?

A
  • Palpable purpura: NO thrombocytopenia
  • Arthritis
  • Abdominal pain
  • Glomerulonephritis

Ahmed MSK Pt.2 Sadness (10 decks)

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