CT Disorders and Vasculitidies Flashcards
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
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
Cause of cardiovascular related death in SLE?
Accelerated atherosclerosis increases risk for MI
SLE serology
Anti-dsDNA Ab; Anti-Smith Ab
Prevention of cardiovascular death in SLE
Minimize risk factors for atherosclerosis
*Smoking cessation, tx for HTN, cholesterol, obesity, sedentary
Diagnosis?
Pt with venous thrombosis/miscarriage
Serology + for anti-cardiolipin ab. lupus anticoagulant, beta 2 glycoprotein
Anti-phospholipid antibody syndrome (APS)
Anti-phospholipid antibody that can produce false positive tests for syphilis
Anti-cardiolipin
Anti-phospholipid antibody that shows prolonged PTT
Lupus anticoagulant
- pro-coagulant in vivo
- anticoagulant in vitro (i.e. during PTT testing)
Retinal cotton wool spots can appear in
SLE and APS
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
Drug induced Lupus serology
+ anti-histone antibodies
+ ANA
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
Diagnosis?
May or may not have manifestations of SLE
Well-defines inflammatory plaques that evolve into atrophic disfiguring scars
Discoid Lupus
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
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
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
