CT Disorders and Vasculitides Flashcards by Billy Harrah

What is used to test for autoimmune disease?

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

1) ANA

2) Not specific

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Systemic lupus Erythematosus has autoantibodies to what antigens?

What type of hypersensitivity is it?

1) Nuclear

2) Type III

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What are notable cutaneous features of SLE?

1) Malar butterfly rash

2) Photosensitivity

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What is a notable hematologic feature of SLE?

Thrombosis

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What are notable cardiopulmonary features of SLE?

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

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What body position does pericarditis get better/worse?

What does it look like on ECG?

What can be heard on auscultation?

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

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Why are SLE patients at an increased risk of MI?

Accelerated atherosclerosis

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What is a notable renal feature of SLE?

Nephritis

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Where does the malar butterfly rash spare on the face?

Nasolabial folds

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What specific antigens do SLE patients have autoantibodies for?

Which one correlates with disease activity?

1) dsDNA (Correlates with disease activity)

2) Sm (Smith)

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

C3 or C4

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What causes Libman-Sacks endocarditis in SLE?

Immune deposits on the valves

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What are the treatment/prevention options for SLE?

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

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

In later years?

1) Opportunistic infections, kidney, or CNS disease

2) MI or thromboembolic events

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

Syphilis

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What is type two antiphospholipid antibodies?

What does it cause risk factors for?

1) Lupus anticoagulant

2) Venous/arterial thrombosis and miscarriage

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What is type three antiphospholipid antibodies?

The antibodies are directed towards what serum cofactor?

1) Anti-cardiolipin antibodies

2) Beta-2 glycoprotein I

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What is the treatment for APS?

Anticoagulation

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

Cotton wool spots

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

No renal or neuro symptoms

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

Anti-histone Abs

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What may happen to SLE patients when given Sulfa antibiotics?

Exacerbate lupus symptoms

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Neonatal lupus affects children born of mothers with what Abs?

What can this condition cause?

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

2) Permanent complete heart block

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

Where does it most commonly occur?

What is it characterized by?

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

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What is the hallmark of scleroderma?

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

What causes the dry itchy skin seen in scleroderma?

Obliteration of eccrine sweat and sebaceous glands

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

1) Secondary Raynaud phenomenon | 2) Changes in skin pigmentation

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

Localized scleroderma

How does raynaud's present in localized scleroderma? What are the patches seen on the skin called?

1) There is no Raynaud's | 2) Morphea

Which type of scleroderma is associated with the CREST syndrome?

Limited Cutaneous Systemic Sclerosis

What is the CREST syndrome?

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

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

1) Digital ischemia | 2) Pulmonary artery HTN

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

1) As shortness of breath | 2) GERD

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

Diffuse cutaneous systemic sclerosis

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

1) Renal crisis | 2) Interstitial lung disease

Which type of scleroderma causes interstitial lung disease? Which causes pulmonary artery HTN?

1) Diffuse | 2) Limited

What does interstitial lung disease sound like on auscultation? How is it diagnosed?

1) Fine “velcro” crackles (rales) | 2) Pulmonary function test

How is pulmonary artery HTN diagnosed? What does PAH increase the incidence of?

1) Right heart catheterization | 2) Bronchoalveolar carcinoma

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

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

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

Glucocorticoids because they induce renal crisis

What GI manifestations are seen with scleroderma?

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

What does Barrett esophagus increase the risk for?

Esophageal adenocarcinoma

What effect does scleroderma have on msk? On the thyroid?

1) Carpal tunnel | 2) Hypothyroid

What is found on serology for diffuse scleroderma?

1) Anti-Scl 70 aka Anti-(DNA) topoisomerase I | 2) Anti-RNA polymerase III

What is found on serology for limited scleroderma?

Anti-centromere

What are the Sicca symptoms seen in Sjӧgren syndrome?

1) Dry eyes, mouth, vagina | 2) Parotid enlargement

Xerostomia in Sjӧgren patients can lead to what complications?

1) Candida | 2) Dental caries

Keratoconjunctivitis sicca is due to? How is it diagnosed?

1) Inadequate tear production | 2) Schirmer test

What does Sjӧgren syndrome have a strong association with?

B cell non-Hodgkin lymphoma (MALT lymphoma)

What is found on serology for Sjӧgren syndrome?

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

What is essential for diagnosing Sjӧgren syndrome?

Lip biopsy

What treatments should Sjӧgren syndrome patients avoid?

Atropinic drugs and decongestants

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

Inflammtory myopathies

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

Creatine kinase and aldolase

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

Dermatomyositis

What are the characteristic skin lesions seen with dermatomyositis?

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

What is heliotrope rash?

Periorbital edema and purplish suffusion over eyelids

What does dermatomyositis increase the risk for?

Occult malignancy

What is found on serology for dermatomyositis and polymyositis? What is the main treatment for dermatomyositis and polymyositis?

1) Anti Jo-1 | 2) Glucocorticoids

While polymyositis has many similarities to dermatomyositis what seperates it?

No skin changes

What confirms diagnosis of polymyositis on biopsy?

Endomysial inflammation

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

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

What causes weakness in finger flexion or quadriceps weakness?

Inclusion body myositis

What confirms diagnosis of inclusion body myositis on biopsy? What labs do we find for IBM? What serology?

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

Henoch-Schönlein Purpura (HSP) affects what sized vessels? What population is it more common in?

1) Small vessel | 2) Kids

What is the most common symptom seen with Henoch-Schönlein Purpura? How is it diagnosed on biopsy? What is the Tx?

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

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?

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

Granulomatosis with Polyangiitis (GPA) aka Wegener’s Granulomatosis affects what sized vessels? How is it diagnosed on biopsy? On serology?

1) Small vessel 2) Granulomas 3) ANCA

What are the hallmarks of GPA?

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

Because of the renal involvement of GPA what may we see? How does it affect the respiratory tract?

1) Hematuria and proteinuria | 2) Nasal involvement usually saddle nose

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?

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

How is EGPA diagnosed on CBC? On Serology? On lung biopsy?

1) High eosinophil count 2) ANCA 3) Granulomas

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?

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

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?

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

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?

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

How is diagnosis of Polyarteritis Nodosa confirmed by biopsy? BY angiogram? By serology?

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

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?

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

How is Kawasaki disease treated?

1) IVIG within 10 days of sx | 2) High dose aspirin

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?

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

What causes the HTN in Takayasu Arteritis? What does the HTN lead to? What aortic complications are seen?

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

How is Takayasu Arteritis diagnosed on histology?

Granuloma with some giant cells

What biomarkers are elevated in Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR)? How are the two conditions related?

1) ESR and CRP | 2) They often coexist

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?

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

What are hallmarks of GCA?

1) Jaw claudication 2) Headache 3) Visual abnormalities

Polymyalgia Rheumatica (PMR) is associated with what symptoms? How is it diagnosed?

1) Stiffness, soreness, and pain in proximal muscles | 2) Everything normal except elevated ESR and CRP

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?

1) Primary | 2) Secondary

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.”?

1) Nailfold capillaroscopy | 2) Secondary

CT Disorders and Vasculitides Flashcards

(87 cards)