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VASCULITIS

Tov she-barofim le-gehinom: The best doctors are


destined for hell (Babylonian Talmud, Kiddushin 82a)

Dunkey driver,
Camilla driver,
Train driver,
Sailor,
Sheperd,
Storage man,
Doctor/Physician
Tov she-barofim le-gehinom: The best doctors are
destined for hell (Babylonian Talmud, Kiddushin 82a)

Dunkey driver,
Camilla driver,
Train driver,
Sailor,
Sheperd,
Storage man,
Doctor/Physician
DEFINITION
Autoimmune systemic vasculitis
represents a heterogenous group with
a common pattern:

inflamation followed by the


necrosis of the vascular wall.
ENDOTHELIUM
Semipermeabile halt

Homeostatic balance

Blood flow control

Regulation of the recruitment of leukocytes

Neovascularisation participation
Clasifiacarea Chapel Hill 2012
Large v. Medium v. Small v
Common symptoms
Fever

Fatigue

Weight loss

Anorexia

Malaise
Chapel Hill
Large vessels :
1. Giant cell arteritis /Hortons
headache/Temporal arteritis/Cranial
Arteritis
Age > 50

Temporal artery

Rheumatic polimialgia

The involvement of AO and its main


branches (external branches of carotid)
Chapel Hill
Large vessels
2. Takayasus Arteritis
age <50

granulomatosis inflammation -
AO and its main branches
Chapel Hill
Medium Vessels
1. PAN
Necrotising inflammation of the medium and
small arteries, without glomerulonefritis or
vasculitis
2. b. Kawasaki
Involvement of large, medium, small vessels +
subcutaneous lymphatic nodules
Coronary artery frequently involved
Children
Chapel Hill
Small vessels
1. Granulomatis with polyangiitis
Granulomatosis inflammation of the respiratory airways +
necrotising vasculitis of small vessels or medium vessels
Necrotising glomerulonephritis frequent

2. Eosynophilic granulomatosis with polyangiitis/ Alergic


granulomatosis
Granulomatosis inflammation of the respiratory airways +
necrotising vasculitis of small vessels or medium vessels
Asthma
Eosynophilia

3. Mycroscopic polyangiiis
Necrotising vasculitis
, immune deposits
Necrotising arteritis medium and small vessels involvement
Necrotising glomerulonephritis frequent
Pulmonary capilaritis frequent
ACR 1990 CRITERIA
LARGE VESSELS:
1. Giant cell arteritis /Hortons headache/Temporal
arteritis/Cranial Arteritis
Age > 50

New headache recent onset or new localisation

Temporal artery tenderness or decreased temporal


pulse

ESR > 50mm

HP: necrotising arteritis mononuclear infiltration or


granulomatous inflammation
3 out of 5
ACR 1990 CRITERIA
LARGE VESSELS:
2. Takayasus Arteritis
Age<40

Claudications of the limbs (upper limbs)

brachial arterial pulse

BP difference > 10 mmHg between the 2 limbs

Bruit over subclavian arteries or aorta

Arteriographic narrowing or occlusion of the entire aorta,


its proximal branches, or large arteries in the proximal
upper or lower extremities
Takayasus arteritis
Arterita Takayasu
ACR 1990 CRITERIA
MEDIUM VESSELS
1. PAN (Polyarteritis Nodosa)
Weight > 4kg.
Livedo reticularis
Pain/testicular tenderness
Mialgias
Mononeuropathy/polineuropathy
dBP > 90mmHg
urea or cr. (BUN)
Markers for B hepatitis
Arteriographic modifications: aneurysms, oclussion
HP: granulocytes or granulocytes and mononuclear
leucocytes in the artery wall

2. Kawasakis Disease
Kawasakis vasculitis
Warm CREAM
Warm: fever> 5 d
plus 4 of 5:
Conjunctivitis-bilateral, non-
purulent
Rash-erythematous,
maculopapular
Erythema palms and soles - +
swelling
Adenopathy, cervical 1
unilateral node
Mucous membrane dry, red,
strawberry tongue
Complications: coronary artery
aneurysm; myocarditis Echo!
ACR 1990 CRITERIA
SMALL VESSELS
Immune complexes associations:
1. Henoch Schonleins purpura
age < 20
palpable purpura
Abdominal pain acute
HP: granulocytes in the walls of the arterioles and
venules
2. Hypersensibilisation vasculitis
age > 16
history: medication precipitating factor
Palpable purpura
Maculopapular rash
HP: granulocytes in a perivascular or extravascular
location
ACR 1990 CRITERIA
SMALL VESSELS
ANCA POSITIVE VASCULITIS
1. Granulomatosis with poliangiitis/ Wegeners
granulomatosis
hematuria
Rx: nodules, cavitaties, infiltrations
Oral and nasals ulcers
HP: granulomatosis inflammation
2. Granulomatosis with eosinophylia/ Churg Strauss
Syndrome
Asthma
Eozinophilia > 10%
mononeuropathy (multiplex), polineuropathy
Pulmonary infiltrates
Paranasal sinus abnormality
HP: Extravascular eosinophils
ANCA

c ANCA p ANCA
cytoplasmic staining perinuclear staining
Proteinase 3 (PR3) Myeloperoxidase
(MPO)
Microscopic polyangiitis
Microscopic polyangiitis
Microscopic polyangiitis
Granulomatosis with poliangiiitis
Episcleritis
Eosynophilic granulomatosis with
poliangiitis
Date of download: 4/13/2014
Copyright American College of Chest Physicians. All rights reserved.

A Case of Sudden Cardiac Death Due to Isolated Eosinophilic Coronary Arteritis*

Chest. 2005;128(2):1047-1050. doi:10.1378/chest.128.2.1047

Figure Legend:
Hemorrhage overlying all three coronary arteries: top, A: right coronary artery [RCA]; middle, B: Ramus interventricularis anterior
[RIVA]; bottom, C: Ramus circumflex [RCX].
Date of download: 4/13/2014
Copyright American College of Chest Physicians. All rights reserved.

A Case of Sudden Cardiac Death Due to Isolated Eosinophilic Coronary Arteritis*

Chest. 2005;128(2):1047-1050. doi:10.1378/chest.128.2.1047

Figure Legend:
Sections from the coronary arteries (left, A, right coronary artery; middle, B, RCX; right, C, RIVA) showing dissecting hematoma (H)
in false lumina between adventitia (A) and media (M), collaps of the true lumina (L), false channel (FC) and inflammatory cell
infiltrate within adventitia is remarkable (hematoxylin-eosin, original 1.25). See Figure 1 for the definition of abbreviations not
used in the text.
ANCA + vasculitis
Respiratory manifestations
Granulomas

Haemoragic alveolitis

Fibrosis
Pulmonary lesions
Asymptomatics (sometimes)
No equivalent breath tests
Do respond to treatment
IF NOT:
o Mimics
o Side effects (posttreatment)
Do continue the treatment if the lesions
are decreasing
Subglotic stenosis
Subglotic stenosis
(GPA)
Good colaboration with the ENT specialist
To differentiate between an active disease
and a lesion
No early tracheostomia !!!
Laser RX
Locally steroids - (Kenalog - triamcinolona) or
mitomycina C
Diet (regurgitation disease)
Antiacids
Traheobronsic lesions
Subjects with previous respiratory
involvement PFP +
Initial evaluation: bronchoscopy
Treat the infections!!! Nu colonisation!!!
Inhale GC
Virtual CT bronchoscopy
dilatation baloon
Stent CR
Haemoragic alveolitis
Haemoragic alveolitis
Time is essential
Standard immunosupression
GC dose 1g d x 3
Plasmapherasis - PEXIVAS
NOVOSEVEN (factor VII activated)
Respiratory symptoms: causes
Active disease: SRT, lungs, heart, vessels,
kidneys
Sepsis: active disease (history)
Postmanagement complications:
infections; pulmonary toxicity
Other: +/- main disease; +/- treatment -
obstructive apnea
Relapsing
Due to genetic phenotype
Increase risk: GPA/PR3-ANCA/ relapse
Decrease risk: MPA/MPO-ANCA/new
disease
Classic immunosupression (MTX, AZA,
MMF)
RTX high risk patients
Mimics
New bruit subacute bacterial
endocarditis
Necrosis of the lower limbs
cholesterol emboli
Hepatic disfunction hepatita tip C
Drugs (abuse) HIV, hep.C, cocaine
neoplasm
Fever of unknown ethiology
Vasculitis diagnosis
Nonspecific inflammatory syndrome
Fever of unknown cause
Ischemic sign and symptoms young
persons
Mononeuritis (multiplex)
Skin involvement
Multiple systemic organ failure
Reno-pulmonary syndrome
Stroke
Others
Vasculitis diagnosis
Mandibular claudication, vision loss,
thickness and tenderness of the
temporal artery, decreased pulse: :
Giant Cell Arteritis
Absence of the radial pulse or hard to
be appreciate: Takayasus Arteritis,
Giant Cell Arteritis
Mononevritis multiplex: PAN, WG,
Churg-Strauss Syndrome
Vasculitis diagnosis
Testicular tenderness: PAN
Bloody diarrhea + abdominal pain:
Henoch-Schonlein p deposits of Ig
Reno-pulmonary syndrome: WG,
Microscopic polyangeitis, (SLE mimics,
Goodpasture syndrome)
Vasculitis diagnosis
HT clinical context of vasculitis PAN,
Takayasus Arteritis
HTA + glomerulonephritis: WG,
Microscopic poliangeitis
Asthma-Churg-Strauss syndrome
Pulmonary involvement: WG, Microscopic
poliangeitis
Skin involvement
Palpable purpura
Livedo reticularis
Cutaneous nodules
Infarctions
Ulcers
Hemoragias
Macules
Laboratory tests
1. Inflammatory syndrome
Chronic anemia + trombocytosis/
neutrophilia + eosinophilia
ESR 100mm + CRP >10mg/dl
absence of bact. infections and
paranoplastic sdr.

Albumine
Laboratory tests
2. Organic involvement
Creatinine
Urea
Liver enzymes
CK
Pulmonary Xray
Cranial MRI or
Abdominal CT
Laboratory tests
3. Immune complexes depot
RF }Never positive in primary vasculitis
AAN
RF + cryoglobulinemia, BSE
AAN + SLE, Sjogren sdr.
Crioglobuline + -hepatitis C
C3,C4 - cryoglobulinemia, PAN (25%),
hypersensitivity vasculitis
Laboratory tests
ANCA + (antineutrophil cytoplasmatic
antibodies)
c-ANCA (serine proteinase 3): WG with
systemical involvement (90%)
p-ANCA (anti myeloperoxidase): Churg-
Strauss sdr., mycroscopic polyangeitis
Pulmonary X ray
Sinus xRay
CT scan
ECG
Angiography
Cutaneous Biopsy
Biopsy dg.
Common sites less common sites
Skin Testicle (PAN)
Sural nerve (PAN, v.CS)-
EMG, NCV
Rect
Temporal artery Giant Liver
Cells Arteritis Heart
Muscle (PAN) Brain (isolating
Kidney ( WG, MP) angiitis)
Lungs (WG, MP)
Sinus (WG)
Angiography
Abdomenal ( celiac br., sup.
Mesenteric artery, renal arteries a.)
PAN
Aortic Arch arterita Takayasu,
art. cu cel. gigantocelulare,
Limbs Buerger disease
Cerebral isolating angiitis CNS
Noninvasive methods
Doppler USGiant Cell Arteritis
Angio MRI aorta (thickness,
stenosis)-Takayasus Arteritis,
Giant Cell Arteritis
PET : aorta, subclavia - Takayasus
Arteritis, Giant Cell Arteritis
FFS (FIVE FACTOR SCORE)
PROGNOSTIC SCORE
PROTEINURIA > 1G/DAY

CREATININE > 140UMOL/DAY

CARDIOMIOPATHY

GASTROINTESTINAL INVOLVEMENT

CNS COMPLICATIONS
FFS >2 INCREASED MORTALITY
MaNAGEMENT GENERAL
ISSUES
Identifications and elimination of the onset
factor (causa) (drugs)
Treatment of co-diseases (AB-miocarditis,
IFN, VIDARABINA, LAMIVUDINA hep. B and
C)
Antiinflamtory and immunosupressive
therapy
Prevention of complications: infections,
osteoporosis, atherosclerosis (BP, lipids-
monitorisations)
THERAPY
Terapia nespecific:
REMMISION:
CS (orally 1 mg/kgc/day, or puls
therapy metilprednisolon 15
mg/kgc/day 3 days a month) +
biphosphonates (glucocorticoids
induced OP)
Therapy
imunosupresion: CYVlofosfamid (orally
2 mg/kgc/day or pulse therapy terapie
0,5-1 g/m2/month), +
trimethoprim/sulfametoxazol pn. -
Pneumocystis carinii + MESNA ( risk of
hemoragic cystitis, bladder cc.)
azathioprine (2 mg/kgc/day),
methotrexat (15-20 mg/week)
Alternative therapy
Transplant immunosupressives :
tacrolimus (FK506 2-6 mg /day),
rapamicina (sirolimus 10-15 ng/ml), MMF
(2000 mg/day), leflunomide (20 mg/day),
cyclosporine A (2-5 mg/kgc/day)
i.v. Imunoglobuline high dose
(2 g/kgc/month)
Alternative therapies
Biologic DMARDS : anti-TNF-alfa
(Etanercept 25 mg s.c. twice a week,
Infliximab 3-5 mg/kgc 0,2,6 and 8 weeks),
interferonalfa (3.000.000u 3 times a week
CS sdr.), (Kineret)
antitimocit globuline ( in WG )
imunoablation: CYC 4g/m2 followed by
STEM cells stimulation - CD34+