CT Disorders and Vasculitides Flashcards Preview

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Flashcards in CT Disorders and Vasculitides Deck (87)
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1
Q

What is used to test for autoimmune disease?

What feature of this test makes it important to gather more information about the patient?

A

1) ANA

2) Not specific

2
Q

Systemic lupus Erythematosus has autoantibodies to what antigens?

What type of hypersensitivity is it?

A

1) Nuclear

2) Type III

3
Q

What are notable cutaneous features of SLE?

A

1) Malar butterfly rash

2) Photosensitivity

4
Q

What is a notable hematologic feature of SLE?

A

Thrombosis

5
Q

What are notable cardiopulmonary features of SLE?

A

1) Pericarditis
2) Libman-Sacks endocarditis
3) MI

6
Q

What body position does pericarditis get better/worse?

What does it look like on ECG?

What can be heard on auscultation?

A

1) Worse in supine, better with sitting upright
2) ST elevation
3) Pericardial friction rub

7
Q

Why are SLE patients at an increased risk of MI?

A

Accelerated atherosclerosis

8
Q

What is a notable renal feature of SLE?

A

Nephritis

9
Q

Where does the malar butterfly rash spare on the face?

A

Nasolabial folds

10
Q

What specific antigens do SLE patients have autoantibodies for?

Which one correlates with disease activity?

A

1) dsDNA (Correlates with disease activity)

2) Sm (Smith)

11
Q

What complement proteins will be decreased on SLE serology because of their increased consumption with this condition?

A

C3 or C4

12
Q

What causes Libman-Sacks endocarditis in SLE?

A

Immune deposits on the valves

13
Q

What are the treatment/prevention options for SLE?

A

1) Avoid sun exposure/wear sunscreen
2) NSAIDs
3) Glucocorticosteroids
4) Hydroxychloroquine

14
Q

In the early years after diagnosis what are causes of SLE mortality?

In later years?

A

1) Opportunistic infections, kidney, or CNS disease

2) MI or thromboembolic events

15
Q

What does type one antiphospholipid antibodies cause biologic false-positive tests for?

A

Syphilis

16
Q

What is type two antiphospholipid antibodies?

What does it cause risk factors for?

A

1) Lupus anticoagulant

2) Venous/arterial thrombosis and miscarriage

17
Q

What is type three antiphospholipid antibodies?

The antibodies are directed towards what serum cofactor?

A

1) Anti-cardiolipin antibodies

2) Beta-2 glycoprotein I

18
Q

What is the treatment for APS?

A

Anticoagulation

19
Q

Both SLE/APS cause retinopathy, what diagnostic feature is seen in the eye?

A

Cotton wool spots

20
Q

What symptoms are not seen in Lupus-like syndrome/Drug-induced?

A

No renal or neuro symptoms

21
Q

Lupus-like syndrome/Drug-induced are positive for what antibodies?

A

Anti-histone Abs

22
Q

What may happen to SLE patients when given Sulfa antibiotics?

A

Exacerbate lupus symptoms

23
Q

Neonatal lupus affects children born of mothers with what Abs?

What can this condition cause?

A

1) Anti-Ro (SSA) or La (SSB) Abs

2) Permanent complete heart block

24
Q

Discoid lupus erythematosus (DLE) is a variant of which lupus?

Where does it most commonly occur?

What is it characterized by?

A

1) Cutaneous lupus
2) Head
3) Inflammatory plaques that evolve into atrophy, disfiguring scars

25
Q

What is the hallmark of scleroderma?

A

Thickening and hardening of the skin and visceral organs (fibrosis)

26
Q

What causes the dry itchy skin seen in scleroderma?

A

Obliteration of eccrine sweat and sebaceous glands

27
Q

What is usually the first symptom seen in caucasian scleroderma patients?

In African American patients?

A

1) Secondary Raynaud phenomenon

2) Changes in skin pigmentation

28
Q

Which type of scleroderma is seen in children and causes discrete areas of discolored skin induration?

A

Localized scleroderma

29
Q

How does raynaud’s present in localized scleroderma?

What are the patches seen on the skin called?

A

1) There is no Raynaud’s

2) Morphea

30
Q

Which type of scleroderma is associated with the CREST syndrome?

A

Limited Cutaneous Systemic Sclerosis

31
Q

What is the CREST syndrome?

A

1) Cutaneous calcinosis
2) Raynaud’s (2⁰)
3) Esophageal dysmotility
4) Sclerodactyly
5) Telangiectasia

32
Q

How are the vascular manifestations more pronounced in limited cutaneous systemic sclerosis?

A

1) Digital ischemia

2) Pulmonary artery HTN

33
Q

How does pulmonary artery HTN present in limited cutaneous systemic sclerosis?

How does esophageal dysmotility present?

A

1) As shortness of breath

2) GERD

34
Q

Which type of scleroderma causes fibrosis, loss of body hair, and impaired sweating?

A

Diffuse cutaneous systemic sclerosis

35
Q

In what ways do diffuse cutaneous systemic sclerosis involve internal organs?

A

1) Renal crisis

2) Interstitial lung disease

36
Q

Which type of scleroderma causes interstitial lung disease?

Which causes pulmonary artery HTN?

A

1) Diffuse

2) Limited

37
Q

What does interstitial lung disease sound like on auscultation?

How is it diagnosed?

A

1) Fine “velcro” crackles (rales)

2) Pulmonary function test

38
Q

How is pulmonary artery HTN diagnosed?

What does PAH increase the incidence of?

A

1) Right heart catheterization

2) Bronchoalveolar carcinoma

39
Q

Renal crisis due to diffuse cutaneous systemic sclerosis can cause abrupt onset of?

A

1) Malignant hypertension
2) Hemolytic anemia
3) Progressive renal insufficiency

40
Q

High doses of what treatment should be avoided in diffuse scleroderma?

Why?

A

Glucocorticoids because they induce renal crisis

41
Q

What GI manifestations are seen with scleroderma?

A

1) Barrett esophagus
2) Gastric antral vascular ectasia (GAVE Syndrome) aka watermelon stomach
3) Primary biliary cirrhosis

42
Q

What does Barrett esophagus increase the risk for?

A

Esophageal adenocarcinoma

43
Q

What effect does scleroderma have on msk?

On the thyroid?

A

1) Carpal tunnel

2) Hypothyroid

44
Q

What is found on serology for diffuse scleroderma?

A

1) Anti-Scl 70 aka Anti-(DNA) topoisomerase I

2) Anti-RNA polymerase III

45
Q

What is found on serology for limited scleroderma?

A

Anti-centromere

46
Q

What are the Sicca symptoms seen in Sjӧgren syndrome?

A

1) Dry eyes, mouth, vagina

2) Parotid enlargement

47
Q

Xerostomia in Sjӧgren patients can lead to what complications?

A

1) Candida

2) Dental caries

48
Q

Keratoconjunctivitis sicca is due to?

How is it diagnosed?

A

1) Inadequate tear production

2) Schirmer test

49
Q

What does Sjӧgren syndrome have a strong association with?

A

B cell non-Hodgkin lymphoma (MALT lymphoma)

50
Q

What is found on serology for Sjӧgren syndrome?

A

1) Polyclonal Hypergammaglobulinemia
2) Anti SSA/Ro
3) Anti SSB/La

51
Q

What is essential for diagnosing Sjӧgren syndrome?

A

Lip biopsy

52
Q

What treatments should Sjӧgren syndrome patients avoid?

A

Atropinic drugs and decongestants

53
Q

What causes symmetrical proximal muscle weakness making it difficulty rising from a chair or bathtub?

A

Inflammtory myopathies

54
Q

What enzymes do we see elevated in serum with inflammatory myopathies?

A

Creatine kinase and aldolase

55
Q

What causes proximal muscle weakness early with distal muscle weakness in later disease course while having no sensory symptoms?

A

Dermatomyositis

56
Q

What are the characteristic skin lesions seen with dermatomyositis?

A

1) Gottron’s patches/papules
2) Heliotrope rash
3) Periungual and V-neck (poikiloderma shawl sign) erythema

57
Q

What is heliotrope rash?

A

Periorbital edema and purplish suffusion over eyelids

58
Q

What does dermatomyositis increase the risk for?

A

Occult malignancy

59
Q

What is found on serology for dermatomyositis and polymyositis?

What is the main treatment for dermatomyositis and polymyositis?

A

1) Anti Jo-1

2) Glucocorticoids

60
Q

While polymyositis has many similarities to dermatomyositis what seperates it?

A

No skin changes

61
Q

What confirms diagnosis of polymyositis on biopsy?

A

Endomysial inflammation

62
Q

Unlike most autoimmune diseases that tend to affect females more, which ones are more prevalent in males?

A

1) Inclusion body myositis
2) Granulomatosis with Polyangiitis (GPA) aka Wegener’s Granulomatosis
3) Polyarteritis Nodosa
4) Kawasaki Disease
5) Thromboangiitis Obliterans

63
Q

What causes weakness in finger flexion or quadriceps weakness?

A

Inclusion body myositis

64
Q

What confirms diagnosis of inclusion body myositis on biopsy?

What labs do we find for IBM?

What serology?

A

1) Rimmed vacuoles
2) Mild elevation or normal CK
3) Anti-cN1A autoAbs

65
Q

Henoch-Schönlein Purpura (HSP) affects what sized vessels?

What population is it more common in?

A

1) Small vessel

2) Kids

66
Q

What is the most common symptom seen with Henoch-Schönlein Purpura?

How is it diagnosed on biopsy?

What is the Tx?

A

1) Palpable purpura without thrombocytopenia
2) IgA deposits
3) Glucocorticoids

67
Q

Goodpasture syndrome affects what sized vessels?

What is the cause of death if not treated?

How is it diagnosed on renal biopsy?

How is it treated?

A

1) Small vessel
2) Pulmonary hemorrhage
3) Deposition of anti-basement membrane antibodies
4) Plasmapheresis

68
Q

Granulomatosis with Polyangiitis (GPA) aka Wegener’s Granulomatosis affects what sized vessels?

How is it diagnosed on biopsy?

On serology?

A

1) Small vessel
2) Granulomas
3) ANCA

69
Q

What are the hallmarks of GPA?

A

1) Granulomatous inflammation
2) Necrotizing vasculitis
3) Segmental glomerulonephritis

70
Q

Because of the renal involvement of GPA what may we see?

How does it affect the respiratory tract?

A

1) Hematuria and proteinuria

2) Nasal involvement usually saddle nose

71
Q

Eosinophilic Granulomatosis with Polyangiitis (EGPA) aka Churg-Strauss Syndrome involves what sized vessels?

What area of the body does it mostly affect?

What are the hallmarks?

A

1) Small and medium
2) Respiratory tract
3) Asthma and Eosinophilia leading to vasculitis with granulomas

72
Q

How is EGPA diagnosed on CBC?

On Serology?

On lung biopsy?

A

1) High eosinophil count
2) ANCA
3) Granulomas

73
Q

When Behcet Syndrome affects the large vessels what does it cause?

When there is venous involvement?

When it affects small vessels what does it cause?

What is found on serology?

A

1) Aneurysms
2) DVT
3) Recurrent mouth ulcers, genital ulcers, and eye inflammation (uveitis)
4) HLA-B51

74
Q

Thromboangiitis Obliterans aka Buerger Disease affects what sized vessels?

What population does it only occur in?

How is it diagnosed on angiography?

What is the treatment?

A

1) Medium
2) Smokers
3) Corkscrew appearance
4) Stop smoking

75
Q

Polyarteritis Nodosa affects what sized vessels?

What virus is it associated with?

What skin symptom is common to see?

Majority of patients have what peripheral nerve issue?

What renal issue?

What respiratory issue?

A

1) Medium
2) HBV virus
3) Digital gangrene
4) Foot drop
5) Renin mediated HTN
6) None, lungs spared

76
Q

How is diagnosis of Polyarteritis Nodosa confirmed by biopsy?

BY angiogram?

By serology?

A

1) Fibrinoid necrosis and NO granulomas
2) Microaneurysm
3) HBsAg, HBsAb, and HBeAg (Hep B)

77
Q

Kawasaki Disease AKA Mucocutaneous lymph node syndrome affects what sized vessels?

What age population does it affect?

Where is incidence the highest?

What is the hallmark?

What is morbidity due to?

A

1) Medium
2) Less than 5yo
3) Japan
4) Strawberry tongue
5) Aneurysm or MI

78
Q

How is Kawasaki disease treated?

A

1) IVIG within 10 days of sx

2) High dose aspirin

79
Q

Takayasu Arteritis affects what sized vessels?

What vessel in particular?

What age population does it affect?

What is seen on angiography?

Why is it known as the pulseless disease?

A

1) Large
2) Aorta
3) Less than 40
4) Long smooth tapered stenosis
5) Obliterates UE peripheral pulses

80
Q

What causes the HTN in Takayasu Arteritis?

What does the HTN lead to?

What aortic complications are seen?

A

1) Renal artery stenosis
2) Copper wiring retinopathy
3) Dilations, regurgitation, aneurysm, and rutgers

81
Q

How is Takayasu Arteritis diagnosed on histology?

A

Granuloma with some giant cells

82
Q

What biomarkers are elevated in Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR)?

How are the two conditions related?

A

1) ESR and CRP

2) They often coexist

83
Q

Giant Cell Arteritis (GCA) aka Temporal Arteritis affects what sized vessels?

Which vessels in particular?

What is the gold standard for biopsy?

What is seen on biopsy?

How is it treated?

What happens if there’s no treatment?

A

1) Large vessels
2) Cranial arteries and aortic arch
3) Temporal artery
4) Multinucleated giant cells
5) Glucocorticoids before biopsy
6) Blindness

84
Q

What are hallmarks of GCA?

A

1) Jaw claudication
2) Headache
3) Visual abnormalities

85
Q

Polymyalgia Rheumatica (PMR) is associated with what symptoms?

How is it diagnosed?

A

1) Stiffness, soreness, and pain in proximal muscles

2) Everything normal except elevated ESR and CRP

86
Q

Which type of Raynaud is benign symmetric and is due to exaggerated physiologic response to cold?

Which one is unilateral is is more severe as it can lead to ischemia?

A

1) Primary

2) Secondary

87
Q

What test is used to differentiate between primary and secondary raynaud?

Which is suspected if results show distortion with widened and irregular loops, dilated lumen and areas of vascular “dropout.”?

A

1) Nailfold capillaroscopy

2) Secondary