VASCULITIS SYNDROME

(VS)

dr. JULIUS ROMA, Sp A

Vasculitis  inflammation of blood vessel’s wall :

 Idiopathic

 After exposure to antigen :

 Infection

 Drugs

 Autoimmune disease

 Connective tissue disease

• Primary / secondary
VS blood vessel’s degree of damage

Mild / moderate :

 Henoch – Schönlein Purpura

Severe : Polyarteritis Nodosa

VS classification based on :

Size of blood vessel

 Large  Takayasu Arteritis

 Arteriolar / capillary oclusion henoch schoen p

Inflammatory cell : PMN, mononuclear, eosinophil

HENOCH – SCHÖNLEIN PURPURA (HSP)

Anaphilactoid purpura

Non trombositopenic purpura

 HSP  vasculitis of small blood vessel signed by :

disorder of skin, joint, gastrointestinal tract and

kidney

 2 -15 years old (4-7 years), ♂ > ♀

 Ethiology : -?

- URTI, drugs, food, immunization

Patophysiology

 Lesion biopsy : IgA immune complex

deposit

 Inflammation of small blood vessel

Clinical
1. Skin :

 Eritematous macula  purpura

 Hip, inferior extremity (simetric)

 Happen for several weeks  disappear, can

be recurrent
2. Artralgia or artritis :

 Simetric, migrant

 Large joint of inferior extremity

 Occur first before skin disorder, can be

recurrent, deformity (-)

3.  Abdominal pain and gastrointestinal tract

bleeding

 Occur after skin disorder

 Can cause perforation and invagination

4. Kidney

 Hematuria / nefritis

 1%  persistent  kidney failure

 Skin disorder last 2-3 months  nefropathy

5. Other organ disorder  rare

Disorders above doesn’t always happen at the same

time
LAB  not spesific

Trombocyte > N, BBS , complement N, IgA 

Hematuria, blood in faeces

Lesion biopsy : vasculitis

Diagnosis  only based on clinical features

Therapy

Supportive & symptomatic

Invagination  operation

Progessive renal disorder  steroid

Prognosis

Commonly good

Spontaneous recovery within several

days/weeks

Recurrent in 50% cases

Chronic Nefritis  renal failure

 KAWASAKI DISEASE

Tomisaku Kawasaki 1967

1974  outside Japan

Indonesia ?

Age >> under five years  1 – 2 years old

Etiology :  ?

 Infection  KD

immune system disorder  KD

Heart disease in children :

 Develop country  >> KD

 Developing country  >> Rheumatic heart

disease

KD :

 All around the world

 > Asia  Japan & Korea

 Social status – middle to upper economy

class
KD :  Systemic vasculitis

 Middle size artery  coronary artery

Acute :

Vascular wall : - endothel edema & smooth muscle +

infiltration of inflammatory cells

Severe  3 vasculer layers  dilatation & aneurysma

Trombus  blood stream obstruction

 Recovery  intima proliferation (fibrosis)  stenosis 

obstruction

Sub-acute : all imunoglobulin 

 CLINICAL MANIFESTATION

Clinical features : not patognomonic

Clinical  KD devide into 3 phase :

I. Acute phase (first 10 days)

1. High fever (410 C), sudden, remiten

No response to antibiotic therapy

Can last 3 – 4 weeks

2 – 5 days after fever, other symptoms occur

2. Bilateral conjunctiva injection, non exudate

3. Reddish on lips, mouth, faring & tongue (Strawberry

tongue)

4. Edema & reddish of hand and foot

5. Polymorphic exanthem  similar to urticaria, measles.

Disappear – recurrent.

6. Neck Limphadenopathy, unilateral, > 1 ½ cm

Other findings on KD :

Cardiovascular disorder :

Tachycardia, gallop rhytm, heart murmur,

cardiomegaly, pericard effusion, EKG changes

Coronary arteri anomaly :

• Occur at the end of week I – II

• As long as it still active, the disorder still exist

II. Subacute phase :

Hand and foot fingertip descuamation (spesific)

Exanthem, fever & lymphadenopathy disappear

Clear cardiovascular disorder : dilatation / coronary

aneurysm, pericard effusion, heart failure & miocard

infarc

Thrombocytosis
III. Recovery phase :

Clinical improvement, heart disorder can continue :

BSR & thrombocyte normal

 DIAGNOSIS

Clinical feature

I. High fever, sudden, remitten, duration > 5 hr

II. 1. Bilateral conjunctiva injection non exudate

2. Anomalies on lips, tongue and mouth

3. Hand and foot disorder : eritema & edema

4. Polymorphic exanthem

5. Cervical unilateral limphadenopathy, diameter > 1,5 cm

I + > 4 II  KD

I + < 4 II + coronary disorder  atipic KD

DD :

1. Morbili

2. Steven Johnson Syndrome

3. Scarlet fever

4. Drug reaction
 SUPPORTIVE EXAMINATION

1. Lab : leucocytosis, CRP & BBS , thrombocytosis,

pyuria, liver and muscles enzyme

2. EKG :  after diagnosis

 Not spesific
3. Echocardiography

To all patient / suspected KD

Coronary artery disorder / other disorder detection

ECHO (+)  repeat 2 weeks & 6 weeks after treatment

ECHO (-), BBS N  stop

Acute phase  ECHO (+)  repeated everyweek 

aneurysm / trombus forming

 Tricuspid regurgitation, mitral & aorta;

pericard effusion
4. Chest X-ray

5. Catheterization / heart angiography  if ECHO (+)

 MANAGEMENT
Acute - hospitalization / bed rest
Aim of theraphy  decrease the coronary inflammation &
myocard, prevent thrombos
Gammaglobulin  first 10 days
 High dose, intravenous
 Immediately after diagnosis
 2 gr/kgBW, single dose, 10-12 hours
 Monitor heart rate/blood pressure
 Decrease the occurrence of coronary disorder
 Small baby  400 mg/kgBW/days, 4 continous days
 Fever remain  repeat dose
 Expensive
Acetyl salisylic acid/ ASA

Together with gammaglobulin

80-100 mg/kgBW/days, oral, 4 dose  14 days after

treatment, or 2-3 days free of fever  3-10 mg/kgBW/days,
6-8 weeks treatment  ECHO (-)  stop

Dose 2-5 mg/kgBW/days  permanent coronary disorder

High dose  anti inflammation

Low dose  anti thrombocyst

Steroid :

 No clear function

 High dose :

 Decreasing fever/other symptoms

 Not preventing coronary disorder

Surgical measure  severe coronary stenosis

KD :

 “Self limiting” disease

 Heart disorder as serious complication :

 Can be permanent & progressive

 KD’s cause of death

 R/ gammaglobulin give faster clinical improvement

Large Aneurysm  obstruction stenosis + trombosis 

clotting  infarc
 POLYARTERITIS NODOSA

  Small – middle artery vasculitis

 Adult > child, ♂ > ♀

 Etiology : ?

 Drugs

 Infection : streptococcus, hepatitis B

 Inflammation of whole blood vessel’s wall  necrosis,

trombosis, aneurysm, tissue infarc
 Clinical Manifestation

Depends on the organ where vasculitis occur

Fever, nausea, letargy, weakness, severe body weight

reduction

Skin : eritema, nodular lesion, petechi, purpura, skin ulcus

& edema

Child : sub cutan nodular lesion, fever, weakness, arthritis

/ arthralgia, minimal systemic disorder, myocytis & (rare)

ganggren
Peripheral neuropathy

Abdomen : pain, bleeding, infarc & ulceration

Renal failure  death

Cough, wheezing, pleuritis

Seizures, encephalopati & stroke

Tachycardia, heart failure, infarc

LAB :

Not spesific

BSR & CRP 

Sometimes leucocytosis, eosinophilia, anemia

Hematuria

Diagnosis

Clinical features + histological features

Prognosis :
Depends on the broad of polyarteritis

Severe, can be fatal

Death: kidney failure, heart failure, CNS

disorder & gastrointestinal tract disorder

Theraphy
Steroid : - supressing acute inflammation

- prolonging life time

Cytostatic drugs
 GRANULOMATOSA WEGENER

Destructive granulomatous lesion : upper respiratory

tract & lung + kidney and lung systemic necroticans
vasculitis

Can lead by years of systemic disorder

♂>♀

Etiology :- ?

- Drugs, allergy & parvovirus infection

Respiratory complaint  main clinical manifestation :

nasal obstruction, secret, lesion in nose  septum

perforation, obliteration of nasal sinus, palatum

ulceration, farynx, larynx & trachea

Cough, hemoptisis, fever, BW , nocturnal sweat

Artritis, neuropathy, rash, splenomegaly, severe

glomerulonephritis  renal failure

Post mortem examination : diffuse vasculitis

LAB

Not spesific

Eosinophilia

Antineutrophil cytoplasmic antibodies (ANCA) (+)

Urine : nephritis

Kidney function : abnormal

X-ray

Sinus disorder, nasal & lung infiltration

Diagnosis

Clinical features

Confirmation : serologic test, histological features

Prognosis

Without therapy  bad

Limited anomaly can live longer

Therapy

Steroid : - supressing vasculitis inflammation

- preventing disorder progressivity

Cyclosphosphamid

Sulfa ?
 TAKAYASU ARTERITIS
(PULSELESS DISEASE)

Rare

Aortic vasculitis & main branch

Especially young ♀ , ASIA – AFRICA

Etiology :-?

- congenital disorder ?

Aneurysm dilatation  rupture

Large blood vessel involvement :

 Weakness/disappearance of pulse on superior

extremity

 Blood pressure of foot > arm

If kidney  hypertension

Neurologic disorder, eyes

Artritis, myalgia, pleuritis, etc  often happen before

symtomatic aortitis
Lab :

 Not specific

 BSR & gammaglobulin 

Angiography  affected blood vessel

Hypertension with unknown cause + fever & BSR 

suspect TAKAYASU arteritis

Prognosis

 Varies

 Adult > better from child

therapy

 Steroid

 Cytostatic

 Operation