Vasculitis (LVV, MVV, SVV, MVV), PMR Flashcards
** Rheumatic diseases assoc’d w aortitis**
-Seronegative SpA
-Relapsing polychondritis
-Behcet’s
-Cogan’s
-Sarcoid
-LVV: GCA, TAK
-AAV
DDx ascending aorta vasculitis/aneurysm
Inflamm: GCA, TA, Behcet
-Noninflam: Marfan, Ehlers-Danlos type 4, familial thoracic aneurysm/dissection, loey-diatz
DDx isolated descending aorta vasculitis/aneurysm
TA (india/pakistan)
-Inflammatory abdominal artery aneurysm
DDx isolated pulm artery vasculitis/aneurysm
Behcet’s (huge’s stovin syndrome)
DDx periaortic vasculitis/aneurysm
-IgG4 related disease
-Lymphoma
-Erdheim Chester
GCA Presentation
Cranial: H/A, scalp tenderness, jaw/tongue claudication, trismus, diploplia, visual sx
-Extracranial: abnormal pulse, stroke, dizziness, claudication, abdo pain, HTN, angina
-Systemic: fever, wt loss, UE claudication, arthritis, cough
Non-productive Cough in GCA Mechanisms
-Pharyngeal artery vasculitis (branch of external carotid)
-Stimulation of cough receptors in the bronchus due to aortic inflammation
Physical findings GCA
-Scalp tenderness
-Temporal artery tenderness, reduced pulse
-Visual sx: diploplia, amaurosis fugax, vision loss, optic neuritis, optic atrophy
-BP >10mmHg diff between arms
-Carotid/Subclavian bruits
What single physical exam finding has the highest likelihood of having a positive temporal artery biopsy?
Temporal artery beading
Ocular Symptoms GCA
Diplopia (Blurry vision)
-Amaurosis fugax, aura
-Painless vision loss
-AION, PION
-Iritis, Conjunctivitis
-Ophthalmoplegia (ischemia EoM)
Mech of vision loss in GCA
-1. AION caused by vasculitis of posterior ciliary artery
-2. central retinal artery occlusion
-3. posterior ischemic optic neuropathy (PION)
-4. occipital cortex ischemia
Nonvasculitis ocular symptoms in GCA patients on prednisone
Glaucoma
-Cataracts
-Central serous retinopathy
GCA vessels
Aorta
-Subclavian
-Vertebral
-Carotid
-Iliac
-Mesenteric
-Renal
-Coronary
-Rarely pulm artery
Aneurysm monitoring/management
MRA/CTA q6-12mo
-Periodic CXR, TTE, Abdo US
-
-Surgery/Endovascular repair >5cm or dissection
-
GCA Imaging - US (vessels, signs, adv, disadv)
Vessels: Temporal, axillary, subclavian
-Signs: halo sign, compression sign
-Adv: cheap, no radiation, noninvasive
-Disadv: operator dependent
GCA Imaging - MRI (vessels, signs, adv, disadv)
Vessels: Temporal, Cranial, aorta, intrathoracic/intraabdo large/medium branches
-Signs: active vessels uptake contrast, structural changes (stenosis, aneurysm)
-Adv: no radiation, noninvasive, good sensitivity
-Disadv: cost, access, procedural time
GCA Imaging - CT (vessels, signs, adv, disadv)
Vessels: Aorta, intrathoracic/intraabdo large/medium branches
-Signs: active vessel uptake contrast, structural changes (stenosis/aneurysm)
-Adv: fast, noninvasive
-Disadv: radiation, cost higher than US (lower than MR)
GCA Imaging - FDG PET (vessels, signs, adv, disadv)
Vessels: Temporal, axillary, subclavian
-Signs: FDG uptake
-Adv: Can look for GCA and mimickers (ca, infxn)
-Disadv: radiation, high cost, access, NOT GOOD for cranial vessels
** GCA Bx Histology**
** Media thickening with TRANSMURAL GRANULOMATOUS inflammation (panarteritis)
- GIANT CELLS, lymph/macrophages, eo
-Intimal hyperplasia/fibrosis
-Fragmentation/destruction internal elastic lamina’
-SHOULD NOT SEE FIBRINOID NECROSIS (look for GPA)
4 reasons for negative TAB in someone you suspect GCA in
- Previous corticosteroid use
– GCA with phenotype not associated with cranial arteritis
– Sampling error& missed lesion due to skip lesions
– Inadequate sample
– Delayed biopsy after treatment
– Biopsied temporal vein
– Patient does not have GCA, i.e alternative diagnosis
**4 clinical or lab findings that are associated with positive TAB **
Limb claudication (positive LR, 6.01; 95% CI, 1.38-26.16),
-Jaw claudication (positive LR, 4.90; 95% CI, 3.74-6.41),
-Temporal artery thickening (positive LR, 4.70; 95% CI,2.65-8.33),
-Temporal artery loss of pulse (positive LR, 3.25; 95% CI, 2.49-4.23),
-Platelet count of greater than 400 (positive LR, 3.75; 95% CI, 2.12-6.64),
-Temporal tenderness (positive LR, 3.14; 95% CI, 1.14-8.65),
-Erythrocyte sedimentation rate greater than 100 mm/h (positive LR, 3.11; 95% CI, 1.43-6.78).
Layers of vessel from lumen outwards
Tunica Intima
-Internal elastic lamina
-Tunica media
-External elastic lamina
-Tunica adventitia
-Vasa vasorum
Where is vasovorum and what does it do
Located in adventitia
-Microvascular network supplies O2 and nutrients to vessel
GCA labs
Elevated ESR, CRP
-Anemia, Thrombocytosis, Transaminitis (ALP)
-
GCA Pathogenesis
Ag trigger activates vascular DCs at adventitia → T cell activation, attraction of lymphocytes/macrophages → granuloma formation, proinflammatory cytokines (IL 1,6, 21, 17), injury of media and external lamina, neoangiogenesis, hyperplasia of intima, and vessel stenosis/occlusion
-DCs at adventitia
→ recruit macrophage, lymphocytes
→ produce IL12/18 for Th1 differentiation → IFNg (macrophage activation and granuloma formation)
-→ IL1/6/21 → Th17 differentiation → IL17 (vascular inflamm, MMP production to destroy internal elastic lamina)
-VEGF brings more T cells and macrophages
-Plt derived growth factor IL1 → intimal proliferation and ischemia
GCA HLA link
HLA DR4
HLA DRB1*04
GCA Tx
Pred 1mg/kg (pulse if vision threatening/loss) x1 mo or until ESR/CRP normal, then long taper
-ASA 81 daily for critical/flow limiting involvement of vertebral/carotid arteries
-MTX if diabetes, relapsing, or steroid side fx
-Toci 162mg SC weekly(if visual sx or critical cranial ischemia)
TA presentation phases
1: prepulseless inflammatory period: fevers, arthralgias, wt loss
-2: vessel inflammation: pain/tenderness
-3: fibrotic: bruits and ischemia
TA clinical manifestations
Bruits, decreased pulse, asymmetric BP
-HTN, arthralgia, constitutional sx
-
-Subclavian/Iliac → limb claudication
-Carotid/Vertebral → H/A and presyncope
-Pulm artery → pHTN
-Cardiac → angina, MI, HF, death, regurg
TA Dx
MRA > CTA, FEG-PET, US
-Biopsy NOT necessary to diagnose
TA histopath
-Different from GCA : cellular infiltrate in adventitia and outer parts of media (including vasa vasorum) with gamma-delta T lymphocytes, NK cells, and CD8 T cells (vs Th1/Th17 lymphocytes in GCA)
TA Tx
-Initial
-Adjunct
-Refractory
-Salvage
-Other
-Refractory to meds
GC + MTX/AZA/TNFi
-Adjunct: ASA (if cranial/vertebrobasilar involvement)
-Refractory: LFN, MMF, AZA, ustekinumab, infliximab, toci
-Salvage: CYC
-Other: BP control, antiplatelet, smoking cessation, lipid mx
-Surgery only if ischemic once dz quiescent (angioplasty, stenting, bypass graft surgery)
TA morbidity
Cardiac:
-Aneurysm, rupture, dissection
-LVH
-Aortic root dilatation, regurg
-pHTN
-MI
-Stroke
Infectious aortitis causes
-TB
-Syphilis (tertiary)
-Bacterial (Staph, Strep, Salmonella
-Viral
Idiopathic isolated aortitis
-Isolated idiopathic thoracic or abdo aortitis
-Giant cell aortitis: indistinguishable from GCA
-IgG4RD
-Chronic periaortitis (abdo)
-Inflamm abdo aortic aneurysm
LVV mimickers
-FMD
-Atherosclerosis
-Radiation fibrosis
-IgG4
-Sarcoid
-Inflamm aortic aneurysm
-Syphilitic aortitis
-Genetic disease (marfan, loeys dietz)
-Congenital coarctation of aorta
-Neurofibromatosis type 1
** PAN organ involvement **
Peripheral nerves > skin > abdo > muscle > kidney
-Unlikely: ENT, lungs
**Clinical features PAN **
- Classification criteria
- Other features
3+ of the following
-Weight loss
-Livedo
-Testis: pain (more w/ hep B)
-Myalgias (excluding shoulder/hip girdle) or weakness
-Nerves: Mononeuritis multiplex (motor/sensory), foot drop, polyneuropathy
-dBP>90mmHg
-Elevated BUN/Cr
-HBV
-Arteriographic abN
-Biopsy small/medium artery showing PMN
Others:
-CNS: stroke, seizure, jaw claudication
-Eye: retinal hemorrhage, optic ischemia
-Skin (LE): Purpura, ulcers, livedo, nodules, fingertip infarct, necrotic lesions
-Cardiac: MI, HF
-Abdo: pain, liver fcn, mesenteric arteritis
-Renal: infarcts, HTN, Proteinuria
Clinical features more found in HBV assoc’d PAN vs PAN
HBV assoc’d: more orchitis, HTN, renal infarct
PAN cutaneous findings
Sam as GPA/eGPA/IC SVV
-Purpura
-Ulcers,
-Livedo,
-Nodules,
-Fingertip infarct,
-Necrotic lesions
Labs PAN
-AoCD, Thrombocytosis, ESR/CRP,
-Low albumin
-Can hv elevated Cr (from renal vessel occlusion) with BLAND urine
-Classically: negative ANA, cryo, RF, ANCA
- HBV
Dx PAN
Biopsy symptomatic (skin, sural nerve, muscle, rectum, testicle)showing granulocytes +/- mononuclear leukocytes in artery wall
-Angiogram if cannot biopsy (usually abdo: renal, liver, spleen, stomach, large/small bowel; rarely hand/foot) showing aneruysm/occlusion of visceral arteries (not due to atherosclerosis, FMD, or other cause)
PAN histology
Focal & segmental transmural **necrotizing vasculitis ** of medium/small arteries (>arterioles; no vein or large vessel involvement; uncommon splenic, rarely pulmonary)
PAN purpura Biopsy histology
LCV
PAN angiogram findings
Microaneurysm (saccular aneurysm)
-Occlusion/cutoff
-Luminal irregularities
-Stenoses
-Rosary bead sign
Ddx aneurysms on abdo visceral angio
Segmental arterial mediolysis (SAM)
-FMD
Segmental arterial mediolysis (SAM)
-Pathogenesis
-What vessels
-Dx
-Tx
** Vasospasm/FMD variant**
-Nonatherosclerotic & ** noninflamm** lytic loss of medial muscle → dilation/aneurysm (muscular, splanchnic, basilar cerebral, coronary arteries)
-Dx: Angiogram, Biopsy
-Tx: Surgery, BP control
2 imaging findings to differentiate segmental arterial mediolysis (SAM) from PAN
-SAM: NO significant vessel wall thickening. String of beads (stenosis, dissection, aneurysm, thrombosis) at multiple renal/ mesenteric arteries
1 lab finding to differentiate SAM from PAN
PAN has elevated ESR, CRP, abnormal UA
-SAM DOES NOT
PAN pathogenesis theories
Immune complex (complements / complexes seldom found)
-Endothelial injury → cytokines/inflamm (no Ag or trigger found)
Conditions assoc’d w/ PAN or PAN vasculitis
Viral infxn: HBV > CMV, HTLV1, HIV, parvo, EBV, Hep C,
-Hairy cell leukemia
-Genetic: loss of adenosine deaminase 2 (DADA2)
-Autoimmune: SLE, RA, childhood DM, Cogan
-Med rxn: allopurinol, sulfa
Tx systemic PAN
Mild (No cardiac, GI, CNS): pred 1mg/kg x4 wks, taper 6-12mo + AZA (2mg/kg) or MTX (20-25/wk)
-Mod/severe: pred 1-2mg/kg + CYC
-Organ/life threatening: IV pulse GC + CYC (2mg/kg PO or 0.5-1g/m2 /mo IV) x4-12mo. (PLEX ONLY if catastrophic)
CYC → AZA/MTX once in remission for total 18mo (MMF if can’t AZA/MTX, Ritux if can’t CYC) + pred taper
-Adjunct: HTN control, PJP ppx, OP prevention
- PT if nerve/muscle involvement
- DADA2: TNF > GC alone
Tx HBV assoc’d PAN (HBsAg+ and HBeAg+)
HBV assoc’d PAN:
-Pred 60 mg/d x1 wk then taper by 50% dose daily until off by end of 2nd week
-PLEX to remove immune complex 3x/wk for 3 wks, 2x/wk for 2 wks, then once/wk until HBeAg-neg
-Antiviral agents on day of PLEX after procedure
5 factor score - Indications and Components w/ 5 year survival
At diagnosis to predict prognosis of PAN, MPA, eGPA
-Cardiac sx
-GI involvement
-CNS involvement
-Creatinine >140umol/L
-Proteinuria >1g/24h
0 = 88% 5 year survival
1 = 75%
2+ = 54%
PAN Cause of death
-1 year
->1 year
1 year: uncontrolled vasculitis
>1 year: complication tx, infxn, vascular event (stroke, MI)
Tx Isolated PAN
-Which organs
-How to treat
Cutaneous (nodular lesions, livedo, polyneuropathy):
-Pred 20-40mg/d w/ taper
-Steroid sparing: MTX, AZA, colchicine, dapsone
-
-Localized gallbladder, uterus, breast, appendix:
-Sugical excision
-No immunosuppression
Thromboangiitis obliterans aka Buergers
-What is it
Inflamatory , obliterative, nonatheromatous vascular dz of small/medium arteries AND veins.
TO Pathology
Acute: hypercellular inflammatory thrombus with microabscess within thrombus
-Subacute: microabscess surrounded by granulomatous inflamm –> organization/recanalization of thrombus
-Late: mature thrombus w/ vascular fibrosis
-Internal elastic lamina INTACT
TO etiology
Unknown
-Possible:
-Smoking (pipe, cigar, tobacco, weed)
-Autoimmune: antiendothelial cell antibodies in active dz (not remission)
-DNA fragments of anaerobic bacterial in arterial lesions and oral cavities
TO features
Ischemia/claudication feet > hands (distally progressing up)
-Instep/pedal claudication
-Digital ulcerations, gangrene, rest pain
-Dysesthesias
-Cold sensitivity
-Superficial migratory thrombophlebitis
-Raynaud’s, rubor, cyanosis
-Arthralgias
TO DDx
Collagen Vascular dz: SLE, SS, MCTD
-Vasculitis: PAN, GCA, TA, cocaine, SVV
-APLA
-Blood dyscrasias: PV, ET, hyperviscosity Occupational: vibratory tools, hypothenar hammer syndrome
-Embolic: thoracic outlet syndrome, myxoma, cholesterol emboli
-Ergot/emphetamine tox
-Atherosclerosis
-Pernio
-Frostbite
TO Ix
Normal: CBC, LFT, UA, BG, ESR/CRP, ANA, RF
-TTE for cardiac thrombi
-Duplex US / ABI
-Arteriogram
TO arteriographic findings
B/L focal segments of stenosis or occlusion with NORMAL proximal or intervening vessels (no emboli or atherosclerosis). Collaterals around occluded areas (treet root, spider web, corkscrew appearance)
-Small/medium artery AND veins involved
-Common: digital arteries fingers/toes, palmar, plantar, tibial, peroneal, radial, ulnar arteries
TO Tx
-Treat ischemic ulcer (wound care, CCB, pentoxifylline, iloprost, sympathectomy, bosenta, GCSF, fibroblast/hepatocyte growth factor, BM mesenchymal stem cells)
-
-Treat cellulitis (abx)
-
-Treat superficial phlebitis (NSAIDs)
-
-Angioplasty, Revascularization, Bypass graft
-
-Amputate (last resort)
PACNS / PCNSV manifestations
H/A
-Neuro sx: cog dysfcn, stroke, FND, sz, hemorrhage, encephalopathy, myelopathy, radiculopathy, neuropathy, ataxia, coma
-
-Systemic vasculitis sx ABSENT (fever, rash, mononeuritis multiplex)
PACNS/PCNSV DDx
-Malignancy: CNS lymphoma, lyphomatoid granulomatosis
-
-Drugs: Cocaine, heroine, amphetamines (all cause vasospasm vs vasculitis)
-
-CTD: SLE, Sjogren, Behcet, PAN, AAV, APLA
-Susac: endotheliopathy w/ sensorineural hearing loss, encephalopathy, retinal artery occlusion
-
-Others: Sarcoid, CADASIL, FMD, Moyamoya, cardiac emboli
PACNS/PCNSV Dx
Brain biopsy (higher yield from granulomatous vasculitis vs lymphocytic vasculitis)
-Imaging:
-Angiogram (not specific: stenosis/ectasis in 1+ vascular bed) > CTA/MRA
-MRI (cortical/subcortical infarcts, white matter hyperintesity, gadolinium enhanced intracranial lesions)
-CSF: lymphocytic pleocytosis, elevated protein, occasionally high IgG and oligoclonal bands. (consider RCVS if normal CSF)
-exclude intravascular lymphoma if LDH elevated
PACNS/PCNSV Tx
High dose pred + CYC (or ritux) x3-6mo (while taper pred)
-Remission (no new sx or MRI lesion): AZA or MMF x1y +
RCVS manifestations
-Acute thunderclap headache
-Seizures
-Brain edema
-SAH (lobar/convexity)
-PRES
RCVS Investigations
CSF and labs NORMAL
-Angiogram: reversible arterial narrowing of intracerebral arteries in bilateral hemispheres
Vasogenic edema
SAH
RCVS Tx
-CCB (nipodipine 60mg q6h, verapamil 80mg BID< verapamil SR 180-20 daily)
-Sometimes short course high dose prednisone
MVV mimickers
-Cholesterol emboli
-Atrial myxoma
-FMD
-Lymphomatoid granulomatosis
-Angioblastc Tcell lymphoma
-Thomboembolic disease
-Ergotism
-Type 4 EDS
-Segmental arterial mediolysis
-Grange syndrome
-Pseudoxanthoma elasticum
PMR clinical features
SECRET
-Stiffness/pain (prox muscles, NO weakness, >2wks)
-Elderly (>50, mostly >60, mean age onset 70)
-Constitutional, Caucasian
-Rheumatism (arthralgias/arthritis - NONEROSIVE, asymmetric distal joint, Knee/wrist/SC joint; MTP/ankle RARE)
-ESR>40 +/- high CRP
-Temporal arteritis
-Absence of RF/CCP
-Rapid GC response
-AM stiffness >45min
-Nocturnal
-Constitutional sx (fever, wt loss, sweats, fatigue)
PMR Pathogenesis
- HLA DR4 association
-Dendritic cells activated at adventitia-media border of large vessel →IL1/6 production and suppression of Treg/Th17 response
PMR ACR/EULAR classification criteria
Required:
– Age >50,
– Bilateral shoulder discomfort,
– Abnormal inflamm markers
-Points (at least 4 required or 5 if optional US criteria used)
-AM stiffness >45min (2)
-Hip pain/ROM limitation (1)
-No RF/CCP (2)
-Absence of other joins (1)
- Shoulder bursitis, biceps tenosynovitis, or glenohumeral synovitis AND at least one hip w/ synovitis and/or trochanteric bursitis (1)
- Bilateral shoulder w/ subdeltoid bursitis, biceps tenosynovitis, or GH synovitis (1)
Source of PMR symptoms
Tenosynovitis (biceps)
-Bursitis (subdeltoid, subacromial, trochanteric, interspinous)
-Synovitis (hip/shoulders)
PMR labs
Inflammatory markers
-Normocytic anemia
-Thrombocytosis
-INCREASED GAMMA GLOBULINS
-Elevated ALP
Indications for TAB in PMR
Sx of GCA:
– Fever,HA, Claudication (jaw, tongue) scalp tenderness
– Visual changes,
– Tender/enlarged arteries, bruits,
– Decreased pulsation or discrepant BP
– Failure of prednisone 20mg/d to improved symptoms or normalize ESR/CRP within 1mo
PMR DDx
-Chronic pain, depression
-Hypothyroid
-Polymyositis (weakness w/o pain)
-Malignancy (esp lymphoma, myeloma)]
-Occult infxn (TB, HIV, SBE)
-Late onset spondyloarthropathy
-RA
-Shoulder OA, Rotator cuff, Frozen shoulder
PMR Tx
12.5-25mg pred daily x1-2yrs
OR
-IM methylpred 120mg q3wks
OR
-MTX 7.5-10mg/week + GC (if DM, OP, or can’t taper GC)
-NEWLY FDA APPROVED SARILUMAB (IL6Ra blocker)
-Exercise
-NO TNFi or CHINESE HERBAL SUPPLEMENTS
PMR poor prognostic factors
Older age
-Female
-Very high ESR/CRP
-Failure of CRP to normalize within 1 wk of pred
Pred taper in PMR
-Or tapering 1mg q2mo if flare occurs during taper
Steroid sparing agents for PMR
MTX 10-20mg/wk
Nonmedication treatment plan for PMR
ROM exercises
-Vitamin D
-Calcium supplements
-Regular DEXA scan
-Flu vaccines
** How does cANCA stain and what does it bind? And what disease **
C Anca = diffuse staining of neutrophil cytoplasm
-Binds PR3 (serine protease in neutrophilic granules)
-GPA (cANCA 90% Sens/spec)
** How does pANCA stain and what does it bind? **
pANCA = perinuclear cytoplasmic staining
-Binds MPO, but also elastase, lactoferrin, catalase, lysozymes
** False positive ANCA**
-CTD: RA, SLE, SS, SSc, PM, RP
-Goodpasture
-Autoimmune GI: IBD, Autoimmune liver, PSC, PBC
-Cystic fibrosis
-Infxn: HIV, HBV/HCV, TB, IE, leprosy, malaria, Parvo B19, Mono
-Meds: PTU, methimazole, carbimazole, hydralazine, minocycline, cocaine with levamisole
GPA / MPA pathogenesis
1) Infxn → cytokine release (IL1, TNF) →
neutrophil/monocytes to transport PR3/MPO to cell surface.
2) ANCA bind to surface PR3/MPO activating neutrophils/ monocytes →
- degranulate ROS + lysosomal enzymes
- upregulate adhesion molecules on endothelial cells so activated neutrophils bind/transmigrate into vessel wall → vasculitis
- NETs damaging endothelium and causing more AB production
Activation of alternative complement pathway (by infxn/injury) → C5a release to further prime neutrophils for activation and promote NET formation and more PR3/MPO
** 5 causes of DAH with capillaritis**
Primary SVV: GPA, MPA, eGPA, anti-GBM, HSP/IgA
-2ndary vasculitis: SLE, RA, APLA, MCTD, DM, behcet, UC, APLA, SSc
-Cryo vasculitis
-Lung or autologous BM transplant rejection
-Drug induced (chemo, PTU, hydralazine, TNF)
-Infxn: IE, leptospirosis
** 5 causes of DAH without capillaritis**
-Idiopathic pulmonary hemosiderosis
-Coagulopathy (DIC, anticoag, antiplatelets, thrombolytics)
-Mitral stenosis
-Pulmonary veno occlusive disease
Diffuse alveolar damage
- Anything causing ARDS
- PNA
- Drug associated: amio, penicillamine, nitrofurantoin
- Toxin or inhalation injury (crack cocaine)
- PM, SLE
- Radiation therapy
- PE
** DAH workup**
Serologies: ANCA, AntiGBM, ANA
-CT chest
-Bronchoscopy: fungal, viral, bacterial, mycobacterial cultures, PJP
-Biopsy shows hemosiderin laden macrophages
-PFT shows increased DLCO
** DAH tx**
O2 supplementation +/- mech vent
-+/- ECMO
-GC (methylpred 1g IV daily x 3d → 1mg/kg PO)
-CYC IV 15mg/kg q2wk x 3, then q3wk x 3 mo or
-Rituximab 375mg/m2 x 4;
-PLEX.
** Non Rheum DDX for saddle**
Trauma
-Cocaine
-Surgery
** Rheum DDX for saddle**
GPA
-Relapsing polychondritis
-Sarcoid
-IgG4 related disease
-RA
Resp Manifestations of GPA
Upper
- Sinusitis, discharge, ulcers, septal perforation, epistaxis
- Saddle nose
- Laryngeal granuloma –> subglottic stenosis, stridor & hoarseness
Lower
- Granuloma (vessel, airway walls, interstitium →
- Nodules +/- cavitation → post-obstructive PNA
- Capillaritis → DAH
- Resp insuff from fibrosis
Renal Manifestations of GPA
Active urine: blood, WBC, protein, casts
-Acute/ chronic renal failure
Eye Manifestations of GPA
- Proptosis from orbital inflamm disease
-Episcleritis/scleritis
-Peripheral ulcerative keratitis (risk of corneal melt)
-Uveitis
-Conjunctivitis
-Optic neuritis
-Lacrimal duct obstruction
-Retinal artery thrombosis
Ear Manifestations of GPA
-Vertigo
-Hearing loss (conductive and sensorineural)
-Otitis media
-Mastoiditis
Skin Manifestations of GPA/ eGPA / IC SVV
-Purpura, petechiae
-Ulcers , infarctions
-Subcutaneous nodules
-Vesicles
-Livedo reticularis
-Urticarial lesions
-Erythema multiforme(more eGPA and IC SVV)
MSK Manifestations of GPA
Arthralgias
-Myalgia
-Synovitis
CNS Manifestations of GPA
CNS:
- Pachymeningitis,
- Cranial neuropathy,
- Ocular palsy,
- CVA, SAH/SDH
- Seizure,
- Pituitary involvement,
-PNS: mononeuritis multiplex
Cardiac Manifestations of GPA
RARE Pericarditis, or involvement of myocardium/coronary vasculature
GI Manifestations of GPA
Intestinal perforation
GU Manifestations of GPA
Bladder/urethral vasculitis
-Orchitis
-Epididymitis
-Prostatitis