10112122, 10381 AM NOTES \Vascults | Osmosis NOTES AoA aay PATHOLOGY & CAUSES + Inflammation of blood vessels + Vasculitides categorized by blood vessel size: small, medium, large CAUSES Damaged endothelium = Damaged endothelium —+ exposed collagen, tissue —+ increased blood coagulation ~+ weakened. damaged blood ‘vessel walls > aneurysms — vessel wall heals, stifens as fibrin deposits Autoimmune disease + Direct method: bady mistakes endothelial layer of blood vessel for foreign pathogen Svattacks + Molecular mimicry: immune sysiem white blood cells (WBCs) mistake normal antigens of endothelial cells for foreign invaders (eg. bacteria) * Medium, large-vessel vasculitides + Indirect method: immune system attacks healthy cells near vascular endothelium —+ darnages endothelial cells * Smal-vessel vascultides {exception Henoch-Schanlein purpura) SIGNS & SYMPTOMS + Inflammatory response symptoms: fever. weight loss, malaise, fatigue + Ischemia "Blood cells clump to exposed collagen inside blood vessels -» blood clots > restricted blood flow * Fibrin deposits in vessel wall + wall ‘thickens, bulges into vessel -» stenosis “restricted blood flow: 162 OSMOSISORG hitps:iwwwcosmosis.orgleamVascultis GENERALLY, WHAT IS IT? LAB RESULTS + C-reactive protein (CRP, erythrocyte sedimentation rate (ESR), complete blood count {CBC}, various autoantibodies + Biopsy vessel segment TREATMENT MEDICATIONS Reduce inflammatory response + Corticosteroids/immunosuppressive drugs e (OSMoSis.org 2022 Edition na10112122, 10:31 AM \Vascults | Osmosis Chapter 25 Vasculitis EAU ttle) Ct TY ary pbeA CD Bay Cate Temporal branch of recs Headache, cial stress, lw pans Se) set Ore pet CR! Ciecr) Dea lod asin sal sors, oe tssvesin oxtremtios prerred oe cy a resting ets, les wih aCe ‘ody enughing. deceated ne sroduetion erated ned presse Pea ney infomation, aight os, Baer ry lin son fever nerve drag Cd ‘Sint ng damage. Sets ‘ener carage Caer ‘Symptoms dopond on where PURPURA A otacs smal ood vessels ecurent ara cer, genital ews tn popes eerste vison headaches, Dany Berard ‘Swot joint teeing kes, wrists als (Osmosis.org © sitosison ies hitps:iwwwcosmosis.orgleamVascultis ana\Vascults | Osmosis BEHCET'S DISEASE Cus PATHOLOGY & CAUSES + Autoimmune multisystem vasculitis affecting any sized vessel, arterialvenous RISK FACTORS + Individuals who are 20-30 years old, of Middle EasterrVAsian descent, biologically male COMPLICATIONS. + Blindness from untreated uveitis {inflammation in eves} SIGNS & SYMPTOMS + Recurrent, painful, sterile oraligenital ulcers (patnergy) + Skin papules indistinguishable from acne + Uveitis, optic neuritis, conjunctivitis itis + Neurologic involvement meningoencephaitis, intracranial HTN, stroke, headache) + Arthritis (knees, ankles} + Fever, weight loss OTHER DIAGNOSTICS. Clinical presentation + Recurrent oral ulcers (three in one year) + ‘wo of following + Recurrent genital ulcers + ye lesions, uveitis, + Skin lesions + Positive patheray test + 2 2mm papule 24-48 hours after oblique insertion Sm inta skin with 20-gauge needle, often performed on forearm 164 OSMOSISORG hitps:iwwwcosmosis.orgleamVascultis /behcet: ood TREATMENT MEDICATIONS + Skin creams, mouth rinses, eye droos * Corticosteroids: eg. prednisone} control inflammation + Medications: (e.g. azathioprine, cyciosporine, or cyclophosphamide) suppress immune system + Medications: (eg. interferon alfa-2b) alter Immune system response Figure 284 Mucosal ulcer in an ingividual with Behcet's disease. (OsMosis.org ana10112122, 10381 AM Vasculitis | Osmosis Chapter 25 Vasculitis BUERGER’S DISEASE PATHOLOGY & CAUSES + Nonatherosclerotic, segmental inflarnmatory disease affecting small, rmegium-sized veins, arteries of extremities — inflammatory occlusive thrombus — distal extremity ischemia, digit uicers gang * AKA thromboangiltis obliterans + Associated with use of toba ne —+ autoamputation RISK FACTORS + Individuals < 45 years old, who are biologically male, use tobacco + Chronic anaerobic periodontal infection (% lof people with Buerger disease) SIGNS & SYMPTOMS + Ulceration of digits + Ischemic claudication: cold, painful cyanotic distal extremities + Subcutaneous nodules, superficial ‘chrombophlebitis + Paresthesias of extremities + Raynaud phenomenon DIAGNOSTIC IMAGING Angiogram * Lack of atherosclerosis, * Collateralization, segments of diseased vessel interspersed: smoking atherosclerosis + Buerger disease simultaneously hitps:iwwwcosmosis.orgleamVascultis LAB RESULTS Biopsy * Definitve; rarely (healing a concern) + Histopathologically. acute-phase lesions show highly cellular, inflammatory ‘rrombus with minimal inflammation of blood vessel TREATMENT + Immediate smoking cessation Fenonat_aaver’ J Figure 25.2 A volume rendered CT angiogram demonstrating obliteration fof the right femoral artery secondary to thromboangitis obliterans. There is also stenosis of the femoral artery on the lft. OSMOSISORG 165 ana\Vascults | Osmosis CHURG-STRAUSS SYNDROME CSUR eRe Ue Uae PATHOLOGY & CAUSES + Smal, medium vessel granulomatous vasculitis involving many organ systems (cardiac, gastrointestinal, respiratory, skin, renal, neurologic) in individuals with allergy-related respiratory conditions (esp. asthma) = AKA eosinophilic granulomatosis with polyangitis EGPA), allergic granulomatosis + P-ANCA reacting with neutrophilic smyelaperoxidase * Etiology unknown RISK FACTORS + Age 30-50; asthmainasal issues SIGNS & SYMPTOMS + Allergies Asthma, chronic rhinosinusit’s, usually precedes vascultic phase by 8-10 years + Neurological + Peripheral neuropathy (usually mononeuritis multiplex) + Subarachnoid, cerebral hemorrhage, cerebral infarction, cranial nerve palsies + Palpable purpura, subcutaneous nodules + Cardiac «Damage ~> heart failure, arrhythmias +“ Accounts ve deaths * Other organ systems (renal, gastrointestinal) -+ symptoms similar to mediurn-vessel vascultides 166 OSMOSISORG hitps:iwwwcosmosis.orgleamVascultis DIAGNOSTIC IMAGING Chest X-ray + Transient, patchy, symmetrical opacities, often in hilarfperisheral distribution + Puimonaty hemorrhage + Bilateral nodular disease without cavitation High-resolution CT scan + Peribronchial, septal thickening + Widely scattered indistinct opacities Pulmonary function test * Obstructive pattern consistent with asthma Bronchoalveolar lavage + High 9 of eosinophils LAB RESULTS + Eosinophila > 1500/mieroL, > 10% on ditferential leukocyte count = P-ANCAJMPO-ANCA antibodies + Acute phase reactants: 1 ESR, CRP Lungiskin biopsy * Definitive + Prognosis poor {ive year survival, 25% without treatment; 50% with treatment} MEDICATIONS * Corticosteroids, immunosuppressive drugs (Osmosis.org ona10112122, 10381 AM Figure 25.3 Hstological appearance of vvascults in Churg-Strauss syndrome, The background is composed almost entirely of eosinophils \Vascults | Osmosis Chapter 25 Vasculitis GIANT CELL ARTERITIS Cause PATHOLOGY & CAUSES + Chronic vascuits of large-, medium-sized vessels + AKA temporal arteritis + Cranial branches of arteties originating ‘rom aortic arch ‘Temporal branch of carotid artery + Aorta, carotids also affected + Most common systemic vascultis, CAUSES + Unknown: possibly genetic, environmental autoimmune-related RISK FACTORS + Almost always in individuals = 50 + More common in individuals who are biologically female + Strong association with polymyalgia theummatica (40-50% of GCA individuals}, COMPLICATIONS. + Ireversible Bingness ff untreated} hitps:iwwwcosmosis.orgleamVascultis Maro SIGNS & SYMPTOMS + New-onset headache (most common} temporal branch of carotid artery + Jaw claudication (pain when chewing) + Transient unilateral vision loss (amaurosis fugax): ophthalmic artery + Carotid brits, decreased pulses in arms, aortic regurgitation + Tender, palpable nodules, absent temporal pulse + Increased risk of aortic dissection, aortic aneurysm \_/— MNEMONIC: TEMPORAL ~ Characteristis of Temporal {Giant cell) arteritis Temporal artery tenderness ESR >100 Multnucleated giant cels Pain Onset 250 years old polymyaig's Rheumatica ‘association Ammaurosis fugax (Osmosis.org ~~ OsMosis‘ORG ‘167 ena10112122, 10:81 AM 168 OSMOSISORG \Vascults | Osmosis DIAGNOSIS LAB RESULTS + Extremaly elevated ESR (> L00mnvhe, IL-B associated with active disease STERNAL ELASTIC LANA ‘Temporal artery biopsy + Tightly packed monocytesimacrophages, as fone giant cel, in internal elastic larnina: segmental pattem; 90% sensitivity TREATMENT + Corticasteroids ‘GIANT CELL DESTRUCTION} Figure 25.4 A histology photomicrograph demonstrating giant cell arteritis. The ‘external elastic arina to the right has been completely destroyed by granulomatous inflammation, GRANULOMATOSIS WITH POLYANGIITIS osms.it/granulomatosis-with-polyangiitis PATHOLOGY & CAUSES + Small-vessel vascultis involving rhasopharyn, lungs, kidneys + AKA Wegener's granulomatosis + Granulomatous disease of respiratory tract — systemic necrotizing vascultis + B-cells release cytoplasmic antineutrophil cytoplasmic antibodies (e-ANCA} ~+ binds to proteinase 3 (neutrophil granule} in neutrophils — neutrophils release {ree radicals — free radicals damage neighboring endothelial cells — vasculit's “Tad + Focal, necrotizing vascults + Necrotizing granulomas in upper airway, lungs * Necrotizing glomerulonephritis (renal vasculitis) RISK FACTORS. + Middle aged individuals who are biologically male hitps:iwwwcosmosis.orgleamVascultis \_) MNEMONIC: 3C3 = *C" drawn from upper Fespiratory tract to lungs, kidneys (all involved} Canca Corticosteroids! cyclophosphamide {treatment} SIGNS & SYMPTOMS + Chronic pain: oral ulcers, bloody nasal ‘mucus, chronic sinusitis, saddle nose (nose ‘caves infeurls) + Hemoptysis, dyspnea, cough, pleuritic ‘chest pain (infiarnmation of lung vessels} * Decreased urine production, hypertension, hematuria, red cel casts, proteinuria {glomerular inflammation} o (OSMOSIS.org 202. Edition m410112122, 10381 AM DIAGNOSTIC IMAGING Abnormal chest X-ray \Vascults | Osmosis Chapter 25 Vasculitis TREATMENT + Relapse common if ANCA still present = Nodes fed ifitrates, cavities, bronehia) MEDICATIONS seas = Corticosteroids, cylophospharide! tra ‘LAB RESULTS + ANCA 80% thrombocytopenia * Abmorral unary ecient mirscopi herman thot ed cel ast pen tng biopsy ~ Cont agnosis ofanlomatous Inrnmaton of arteryberwaselr sea OTHER DIAGNOSTICS Nasalora inflammation + Oral ulcers; painful/painless + Pure body nasal esenorge * Chon sini, sods nosedestuctve sinonasal diease : VASCULITIS tna . | {teed greteharals Small vessel [fis Groculomatasis vin Parganas (68) ‘affects ~ iddle-aged. malts Treatment oN ASoPHARENK - > 3 oe sNews psf Sui 2 - Seb Sea tings - 2 atcaty raring Titeees 2 woody Coughs — seancm pres | dates apes ood 40 GuomeRuLs During production = Newod pressure Figure 25.5 llustration demonstrating the affects of granulomatosis with polyangils hitps:iwwwcosmosis.orgleamVascultis (Osmosis.org © atlodeOR6 “ido ana10112122, 10:81 AM Vasculitis | Osmosis HENOCH-SCHONLEIN PURPURA PATHOLOGY & CAUSES TREATMENT + Small vessel vasculitis secondary to IgA. + Self-resolving, may reoccur immune complex deposition. + Elevated IgA in blood targets sol MEDICATIONS endothelial cells: molecular mimicry + Most common systemic vascultis of childhood + Frequently follows upper respiratory infection + Associated with Berger cisease (IgA nephropathy) = Unknown cause; immune-mediated ‘vasculitis triggered by infections! immunizations + Steroids, only if severe + Self-limited disease sTevad + Palpable purpura, without coagulopathy! thrombocytopenia; mainly lower extremities + Acthritisfarthralgias + Renal disease Aedominal pain SIGNS & SYMPTOMS + Palpable purpura of buttocks, legs {skin discolouration, as if blood collected under skin surface); abdominal pain: arthritis! ralgias; hematuria, decreased kidney ‘unction (associated with lgA nephropathy) LAB RESULTS Biopsy + Definitive, not necessary Figure 25.6 The clinical appearance of Jenach-Sehénlein purpura 170 OSMOSISORG hitps:iwwwcosmosis.orgleamVascultis ona10112122, 10381 AM \Vascults | Osmosis Chapter 25 Vasculitis KAWASAKI DISEASE PATHOLOGY & CAUSES * Coronary arteries: inflammation + aneurysms + AKA mucocutaneous lymph node syndrome + Most common type of vasculitis in children + Usually self-limited RISK FACTORS * Infants, children < five years old, Asian descent, biologically male COMPLICATIONS. + Coronary artery aneurysin + Decreased myocardial contractility -» heart failure + Myocardial infarction (MI + Athythmias + Peripheral artery ecclusion SIGNS & SYMPTOMS \) MNEMONIC: CRASH & . BURN (™) Signs & Symptoms Conjunctivitis: bilateral, ut onexudative Polyenorphous Rash: desquarrating Cervical iymphAdenopathy Strawberry tongue: cracked red lips, oral mucositis Hand-foot erytherna/ desquamation: edema, ‘erythema Fever: “burn” hitps:iwwwcosmosis.orgleamVascultis DIAGNOSTIC IMAGING Chest X-ray + Cardiomegaly Echocardiography * Coronary artery aneurysms, pericardial effusions, decreased contractility LAB RESULTS +1 CRP, ESR, platelet count (reactive thrombocytosis) OTHER DIAGNOSTICS + Four of five CRASH symptoms, high fever lasting five days ECG * Arthythmias, abnormal Q waves, prolonged PR, QT intervals (RIGHT CORONARY ARTERY Figure 25:7 A coronary angiogram demonstrating a massive right coronary artery aneurysm, OSMOSISORG 171 ron10112122, 10:31 AM \Vascults | Osmosis TREATMENT MEDICATIONS 1yenous imumunogiobulin (VIG) + Aspirin MICROSCOPIC POLYANGIITIS PATHOLOGY & CAUSES + Necrotizing vasculitis: kidney, lung vessels. LAB RESULTS + No granulomas present + Associated with perinuclear anti-neutrophil cytoplasmic antibodies (p ANCAMPO ANCA + Pauci-immune glomeruionestrits (rninimal immunefluorescent staining for IgG) + Older adults + Similar to granulomatosis with polyanaits, without nasopharyngeal involvernent + Fever, weightloss, fatigue, myaloia, arthralgias += Cough, dyspnea, hemoptysis, pleuritic chest pain + Decreased urine output, hematuria, red cell casts, proteinuria + Skin lesions (especially lower extremities) purpura + focal necrosis — ulceration + Relapse common MEDICATIONS = Corti 172, OSMOSISORG hitps:iwwwcosmosis.orgleamVascultis + p-ANCA/MPO-ANCA levels; elevated ESR, CRP, anemia, increased creatinine + Protein, red blood cells (RBCs} TREATMENT ids, cyclophosphamide (Osmosis.org 202.2 Edition ne10112122, 10381 AM \Vascults | Osmosis Chapter 25 Vasculitis POLYARTERITIS NODOSA PATHOLOGY & CAUSES + Immune system forms antibody antigen ‘complex {sometimes associated with hepatitis 8) + deposits in vessel wall —+ immune reaction — invasion of polyenorphonuclear leukocytes —» segmental, transmural inflammation of muscular arteries -+ necrosis of three artery layers {tunica intima, media, adventitia) —» fibrosis as walls heal fibrinoid necrosis} = fibrosed vessel wall weakens, prone ‘to aneurysms —» fibrotic aneurysms (hard bulges} develop, + Different stages of inflammation indifferent vessels, RISK FACTORS + Individuals > 40 years old, biologicaly male + Active hepatitis B (H8Vi/hepatitis C (HCV) infection Hiv + Prescrptionilicit drug exposure, amphetamines SIGNS & SYMPTOMS + Systemic: fever, atigue, weight loss, arthralgia + End organ ischemic damage += Renal arteries: HIN + Mesenteric artery: mesenteric ischemia, severe abdominal pain, gastrointestinal bleeding + Mononeuropathy multiplex: motor, sensory deficits occur in > one nerve throughout ody + Skin arteries: skin lesions (eg. ulcers, erythemstous nodules resembling erythema nodosum, purpura, livedo reticularis) hitps:iwwwcosmosis.orgleamVascultis DIAGNOSTIC IMAGING Mesenteric angiogram + “Sting of beads" pattern along artery, spasms LAB RESULTS + HBV, HCV serologies, Cr, muscle enzymes, urinalysis Biopsy OTHER DIAGNOSTICS. Physical exam * Vascular lesions, motor weakness (due to ischemia} TREATMENT MEDICATIONS += Corticosteroids * Cyclophosphamide: supplement corticosteroids in moderate to severe cases (Osmosis.org © osmosis ore “373 rane10112122, 10:31 AM \Vascults | Osmosis Pournercernis wooosh IMMUNE Ceus ——-> Ewporwctiv > ORGAN IscHEMIA ee ped — 5 7 sree Sing of ad i io: va unter ayaeey pin ANGioGPAM Sanvemesstol wieding Grin erterits _ newrologil syuptoms Teemment: conncastcrons : atch S¥in aweries syin \esions, Figure 25.8 Illustration showing polyarterits nodosa’s characteristic “string of beads” pattern running along the artery. TAKAYASU ARTERITIS + Segmental, patchy granulomatous vasculitis of sortc arch, major branches + Stenos's, thrombosis, aneurysm CAUSES + Unknown: possibly bacterial (eg. spirochetes, Mycobacterium tuberculosis, streptococcal) * Genetic RISK FACTORS: + Individuals of Asian descent, < 40 years ld, biologically female COMPLICATIONS. + Limb ischemia; aortic aneurysm: aortic regurgitation: stroke: secondary hypertension (HTN) due to renal artery stenosis 174 OSMOSISORG hitps:iwwwcosmosis.orgleamVascultis + Inflammation + Aortic branches, upper extremities: ‘weaklabsent pulse + Aortic branch, head: neurological ‘symptoms (e.g. headaches, syncope, stroke) » Coronary arteries: angina Renal arteries: HTN, + Visual disturbances: ocular vesselsiretinal hermorthage + Constitutional symptoms: fever, night sweats, arthralgias, malaise, fatigue + Ischemia in areas of stenosis DIAGNOSTIC IMAGING CT angiography (CTA), magnetic resonance angiography {MRA) * Luminal narrowinglocclusion of major aortic branches + Vessel wall thickening + Aattie valve ~v 8 EMOSis.org rane10112122, 10:31 AM \Vascults | Osmosis Chapter 25 Vasculitis stenosis} + Aneurysmal dlationfpseudoaneurysm formation Ultrasound, + Homogeneous and circumferential thickening of arterial wall (contrast to atherosclerotic plaque: ron-homogeneous, calcified, regular wal’) + Vascular occlusion due to intimal thickening/secondary thrombus formation + Loss of pulsatlity of vessel ‘LAB RESULTS + TESR TREATMENT MEDICATIONS: * Corticosteroids. = Treat HTN Figure 25.9 An angiogram demonstrating SURGERY ‘multiple stenosis of the aortic arch vessels, 2 ‘feature of Takayasu arteritis, + Angioplasty (when no acute inflammation) bypass aratting it severe (OsMoSiIS.org OSMOSISORG 175 hitps:iwwwcosmosis.orgleamVascultis vane