CT Disorders and Vasculitidies Flashcards

1
Q

Diagnosis?

Multisystem autoimmune disorder (predominantly F)
Immune complex deposition damages tissues (Type III HSR)
Fever, malar butterfly rash, photosensitivity
Inflammatory, symmetric, non-erosive arthritis
Cytopenias, thrombosis risk
*Pericarditis (pleuritic chest pain), improves with sitting upright and leaning forward, diffuse ST elevation on ECG
Libman-Sacks endocarditis
Nephritis
Neurologic disorders

A

SLE

*Serology will show anti-dsDNA Ab and anti-Smith Ab

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2
Q

How will chest pain in SLE present?

A

-Pleuritic chest pain that improves with sitting upright and leaning forward
-Friction rub on auscultation
Diffuse ST elevations on ECG

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3
Q

Cause of cardiovascular related death in SLE?

A

Accelerated atherosclerosis increases risk for MI

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4
Q

SLE serology

A

Anti-dsDNA Ab; Anti-Smith Ab

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5
Q

Prevention of cardiovascular death in SLE

A

Minimize risk factors for atherosclerosis

*Smoking cessation, tx for HTN, cholesterol, obesity, sedentary

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6
Q

Diagnosis?

Pt with venous thrombosis/miscarriage
Serology + for anti-cardiolipin ab. lupus anticoagulant, beta 2 glycoprotein

A

Anti-phospholipid antibody syndrome (APS)

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7
Q

Anti-phospholipid antibody that can produce false positive tests for syphilis

A

Anti-cardiolipin

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8
Q

Anti-phospholipid antibody that shows prolonged PTT

A

Lupus anticoagulant

  • pro-coagulant in vivo
  • anticoagulant in vitro (i.e. during PTT testing)
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9
Q

Retinal cotton wool spots can appear in

A

SLE and APS

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10
Q

Diagnosis?

SLE-like symptoms
+ anti-histone antibodies***
Precipitated by drugs

A

Drug induced Lupus (aka Lupus like syndrome)

*Tx = stop the med, will resolve DIL

Procainamide
Hydralazine
Isoniazid (INH)
Methyldopa
Lithium
Phenytoin
Nitrofurantoin
Sulfasalazine
Hydrochlorothiazide (HCTZ)
Simvastatin
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11
Q

Drug induced Lupus serology

A

+ anti-histone antibodies

+ ANA

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12
Q

Diagnosis?

Fetus with Lupus symptoms
Fetus may have congenital heart block
Mom has Anti-Ro (SSA) Abs

A

Neonatal Lupus

  • Tx is to give pregnant mom dexamethasone
  • Deliver fetus if it is distressed
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13
Q

Diagnosis?

May or may not have manifestations of SLE
Well-defines inflammatory plaques that evolve into atrophic disfiguring scars

A

Discoid Lupus

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14
Q

Diagnosis? Scleroderma type?

Discreet areas of discolored skin induration (patches = morphea)
May affect joints or muscles in the area
Not systemic
Histologically indistinguishable from other forms of scleroderma

A

Localized Scleroderma

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15
Q

Diagnosis? Scleroderma type?

Calcinosis cutis
Raynauds (presents early)
Esophageal dysmotility
Sclerodactly
Telangectasia
\+Anti-centromere Ab, +ANA
Pulmonary artery hypertension (PAH) (pt SOB)
A

Limited Scleroderma (CREST Syndrome)

*RT heart cath to diagnose PAH

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16
Q

Diagnosis? Scleroderma type?

Interstitial lung disease (SOB, dry crackles on auscultation)
Renal crisis (caused by HTN)
Reynauds (presents late)
\+Anti-Scl 70/Anti-DNA topoisomerase Abs
\+Anti-RNA polymerase III Abs
\+ANA
CXR and PFTs
A

Diffuse Scleroderma

*Dx with PFTs, velcro crackles (rales) on auscultation

17
Q

Treatment for CREST Syndrome (Limited Scleroderma)

A

No therapy significantly alters disease state
Manage organ system involvement
Glucocorticoids my lead to renal crisis

Renal crisis = malignant HTN, hemolytic anemia, progressive renal insufficiency

18
Q

Treatment for Diffuse Scleroderma

A

No therapy significantly alters disease state
Manage organ system involvement
Glucocorticoids my lead to renal crisis
*O2 and pulmonary rehab

Renal crisis = malignant HTN, hemolytic anemia, progressive renal insufficiency

19
Q

Sjogren syndrome serology

A

Polyclonal hypergammaglobulinemia
+Anti-SSA/Ro
+Anti-SSB/La

*dx with labial salivary gland biopsy

20
Q

Diagnosis?

Myalgias
Bilateral proximal muscle weakness without sensory sxs
Gottron's patches
Heliotrope rash
V-neck erythema
Elevated CK and aldolase
ESR and CRP normal
Anti-Jo-1 Abs
Perimysial and perivascular inflammation, 
*perifasicular atrophy*
A

Dermatomyositis

  • check for malignancy!!!
  • Tx with glucocorticoids
21
Q

Diagnosis?

Bilateral proximal muscle weakness

Elevated CK and aldolase
ESR and CRP normal
Anti-Jo-1 Abs
Endomysial inflammation

A

Polymyositis

  • Tx with glucocorticoids
  • polymyositis has objective muscle weakness, polymyalgia rheumatica does not
22
Q

Diagnosis?

Myalgias
Weakness in finger flexion or quads
Mildly elevated CK
Endomysial inflammation
*rimmed vacuoles*
A

Inclusion body myositis

*refractory to treatment, supportive

23
Q

Vasculitis diagnosis?

More common in kid
Palpable purpura with no thrombocytopenia
Arthralgias
Abdominal pain
Renal disease (glomerulonephritis)
IgA deposits on biopsy
A

IgA Vasculitis aka Henoch-Schonlein Purpura

Tx glucocorticoids

24
Q

Vasculitis diagnosis?

Hematuria due to glomerular capillary damage
Hemoptysis
***Deposition of anti-basement membrane abs on renal biopsy

A

Anti-GBM Glomerulonephritis aka Goodpasture syndrome

Tx plasmapheresis (remove abs) and glucocorticoids

25
Q

Vasculitis diagnosis?

More common in males*
Granulomatous inflammation (necrotizing vasculitis)
Affects the nose and upper respiratory tract (cavitary lung lesions)
+ c-ANCA

A

Granulomatosis with Polyangitis aka Wegner’s Granulomatosis

*Tx high dose glucocorticoids

26
Q

Vasculitis diagnosis?

Systemic necrotizing vasculitis and palpable purpura that follows years after asthma/allergic rhinitis
Hallmark of Asthma + Eosinophilia

A

Eosinophilic Granulomatosis with Polyangitis aka Churg-Strauss Syndrome

Tx glucocorticoids and no smoking

27
Q

Vasculitis diagnosis?

Small vessels: Recurrent apthous ulcers, Genital ulcers, Uveitis
Large vessels: aneurysms
Venous involvement: DVT

Serology: HLA-B51
Pathergy (pustules at site of sterile needle pricks)

A

Behcet Syndrome

Tx low dose glucocorticoids

28
Q

Vasculitis diagnosis?

Males <45
Only occurs in those who smoke tobacco
Thrombosis leads to loss of digits
Corkscrew on angiography

A

Thromboangitis Obliterans aka Bueger Disease

Tx STOP SMOKING (glucocorticoids and anticoag dont work)

29
Q

Vasculitis diagnosis?

More common in males
Associated with HBV (+ serology)
Livedo reticularis
Ulcers, digital gangrene
Peripheral neuropathy (foot drop)
Renal infarct leading to newly acquired HTN
Lungs are spared
Fibrinoid necrosis, no granulomas
A

Polyarteritis nodosa

30
Q

Vasculitis diagnosis?

<5yo more likely male (high incidence in Japan)
Fever, LAD, rash, strawberry tongue

A

Kawasaki disease aka Mucocutaneous Lymph Node Syndrome

  • Tx with IVIG within 10 days and high dose ASA
  • Morbidity from coronary artery involvement (aneurysm or MI can occur years later)
31
Q

Vasculitis diagnosis?

Affects aorta and branches
Loss of peripheral pulses, although ischemic limb loss is rare
Copper wiring retinopathy on funduscopic exam
Long smooth tapered stenosis on angiography
Granuloma w/ some giant cells on histo

A

Takayasu Arteritis

*Aortic complications include dilations, regurg, aneurysm, rupture

32
Q

Vasculitis diagnosis?

Affects cranial arteries (temporal, facial, opthalmic)
Headache
Pain while chewing
Visual abnormalities
Associated with polumyalgia rheumatica
Elevated ESR
Temporal biopsy shows multinucleated giant cells

A

Giant Cell Arteritis (GCA) aka Temporal Arteritis

Tx with glucocorticoids BEFORE biopsy!!! Pt will go blind without treatment

33
Q

Diagnosis?

Stiffness, soreness and muscle pain
Feelings of weakness due to pain (subjective)
No true objective weakness
Elevated ESR and CRP
Normal electromyography (EMG)
Associated with giant cell arteritis
A

Polymyalgia Rheumatica (PMR)

Tx glucocorticoids

*polymyositis has objective muscle weakness, PMR does not

34
Q

Polymyalgia rheumatica commonly occurs alongside what vasculidity?

A

Giant Cell Arteritis aka Temporal Arteritis