CT Disorders and Vasculitidies Flashcards by Brock Wiley

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

SLE

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

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How will chest pain in SLE present?

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

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Cause of cardiovascular related death in SLE?

Accelerated atherosclerosis increases risk for MI

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SLE serology

Anti-dsDNA Ab; Anti-Smith Ab

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Prevention of cardiovascular death in SLE

Minimize risk factors for atherosclerosis

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

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Diagnosis?

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

Anti-phospholipid antibody syndrome (APS)

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

Anti-cardiolipin

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Anti-phospholipid antibody that shows prolonged PTT

Lupus anticoagulant

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

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Retinal cotton wool spots can appear in

SLE and APS

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Diagnosis?

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

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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Drug induced Lupus serology

+ anti-histone antibodies

+ ANA

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Diagnosis?

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

Neonatal Lupus

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

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Diagnosis?

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

Discoid Lupus

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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

Localized Scleroderma

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Diagnosis? Scleroderma type?

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

Limited Scleroderma (CREST Syndrome)

*RT heart cath to diagnose PAH

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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

Diffuse Scleroderma

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

Treatment for CREST Syndrome (Limited Scleroderma)

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

Treatment for Diffuse Scleroderma

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

Sjogren syndrome serology

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

*dx with labial salivary gland biopsy

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*

Dermatomyositis

check for malignancy!!!
Tx with glucocorticoids

Diagnosis?

Bilateral proximal muscle weakness

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

Polymyositis

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

Diagnosis?

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

Inclusion body myositis

*refractory to treatment, supportive

Vasculitis diagnosis?

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

IgA Vasculitis aka Henoch-Schonlein Purpura

Tx glucocorticoids

Vasculitis diagnosis?

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

Anti-GBM Glomerulonephritis aka Goodpasture syndrome

Tx plasmapheresis (remove abs) and glucocorticoids

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

Granulomatosis with Polyangitis aka Wegner's Granulomatosis *Tx high dose glucocorticoids

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

Eosinophilic Granulomatosis with Polyangitis aka Churg-Strauss Syndrome Tx glucocorticoids and no smoking

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)

Behcet Syndrome Tx low dose glucocorticoids

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

Thromboangitis Obliterans aka Bueger Disease Tx STOP SMOKING (glucocorticoids and anticoag dont work)

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 ```

Polyarteritis nodosa

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

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)

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

Takayasu Arteritis *Aortic complications include dilations, regurg, aneurysm, rupture

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

Giant Cell Arteritis (GCA) aka Temporal Arteritis Tx with glucocorticoids BEFORE biopsy!!! Pt will go blind without treatment

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 ```

Polymyalgia Rheumatica (PMR) Tx glucocorticoids *polymyositis has objective muscle weakness, PMR does not

Polymyalgia rheumatica commonly occurs alongside what vasculidity?

Giant Cell Arteritis aka Temporal Arteritis