Takayasu Arteritis, IgA Vasculitis ( Henoch Schonlein ) , Cryoglobulinemic vasculitis , Kawasaki Disease

1. SYSTEMIC VASCULITIDES
Dr.Rohit C K

2. TAKAYASU ARTERITIS
Inflammatory and stenotic disease of medium and
large sized arteries
Strong predilection for aortic arch and its branches

3. PATHOPHYSIOLOGY
Panarteritis with inflammatory mononuclear cells
and occasionally giant cells
Marked intimal proliferation and fibrosis
Scarring, disruption and degeneration of the elastic
lamina
Narrowing of lumen – thrombosis +/-
Vasa vasorum – frequently involved

5. CLINICAL MANIFESTATIONS
Malaise, fever, night sweats, arthralgia , anorexia ,
wt loss
Pulses absent – esp. subclavian artery
HTN – in 32-93% patients

6. LABS
ESR raised
Mild anemia
Elevated immunoglobulin levels

7. DIAGNOSIS
 Mainly clinical
 Strongly suspect in – young woman with decreased/absent
peripheral pulses, discreptancies in BP + arterial bruits
 Confirmed by arteriography  irregular vessel walls,
stenosis , post stenotic dilation, aneurysm, increased
collateral circulation
 Histiopath – vessel wall inflammation – predominantly
lymphocytic with granuloma formation + giant cells 
involving media and adventitia

8. TREATMENT AND PROGNOSIS
 5 yr mortality rate – 0 to 35%
 Mortalitiy often due to CCF, cerebrovascular events, MI, aneurysm
rupture, renal failure
 Glucocorticoid therapy – 40-60mg/d prednisolone reduces symptoms
 Refractory cases – methotrexate upto 25mg / week

9. Introduction, Aims & Objectives Methods & Results Conclusions
A Rare case of Takayasu’s Arteritis presenting with Congestive
Cardiac Failure
)Institute Name (AJ Institute Of Medical Sciences)
Authors (Dr.Rohit Rajeevan Chodaparambil K, Dr.Shafeel Ibrahim K)
Case Report- A Rare case of Takayasus
Arteritis presenting with Congestive
Cardiac Failure
Author’s Name- Dr.Rohit Rajeevan
Chodaparambil K,
Dr.Shafeel Ibrahim
Presenting Author- Dr.Rohit Rajeevan
Chodaparambil K
Institution name- A J INSTITUTE OF MEDICAL
SCIENCES, MANGALORE.
INTRODUCTION-
Takayasu’s arteritis is an inflammatory disase
often affecting the ascending aorta and
aortic arch, causing obstruction of the
aorta and its major arteries.
The pathology is a panarteritis characterized
by mononuclear cells and occasionally
giant cells, with marked intimal
hyperplasia, medial and adventitial
thickening and in the chronic form,
fibrotic occlusion.
Cardiac involvement in Takayasu’s arteritis is
rare and seen in ~ 10% individuals.
CASE- A 45 year old female with no pre-existing co-morbidities
presented with exertional dyspnea and bilateral pitting oedema
since a month.
On examination, radial and brachial pulses in bilateral upper limbs
were not palpable and blood pressure was recorded as
130/110mmHg. Apex beat was displaced laterally and
downwards and was well sustained. Jugular venous pressure
was elevated. Blood pressure in bilateral lower limbs was
200/100mmHg.
2D echo showed moderate pulmonary hypertension with PASP =
60mmHg and multiple regional wall motion abnormalities. Her
blood parameters and abdominal ultrasound were within normal
limits. VDRL was negative
CT aortogram was performed which showed pancardiomegaly,
narrowing in the abdominal aorta and multiple intraluminal
calcifications.
As per ACR criteria, she was diagnosed to have Takayasu’s arteritis
and given supportive treatment with diuretics, ACE inhibitors,
low dose corticosteroids following which her condition improved.
She was discharged and advised to continue her medications and
regular follow up.
The prevalence of cardiac involvement
in Takayasu’s arteritis in the form of
congestive cardiac failure is rare
<10%. Steroids may be used in
conservative management. Surgical
management may be indicated in
cases with recurrent stenosis.

10. IGA VASCULITIS ( HENOCH SCHONLEIN)
Small vessel vasculitis
Palpable purpura ( over buttocks , lower
extremities )
Arthralgias
GI signs and symptoms
Glomerulonephritis

11. INCIDENCE AND PREVALENCE
Usually in children
Age 4 – 7 years
Male to female = 1.5 : 1
Seasonal variation , peak incidence in spring

12. PATHOPHYSIOLOGY
Immune complex deposition
Triggers – upper resp tract infections, drugs, foods,
insect bites, immunizations
IgA antibodies M/C seen in immune complexes,
demonstrated in renal biopsies

13. CLINICAL MANIFESTATIONS
 Pediatric patients – palpable purpura seen in almost all
 Polyarthralgias +
 GI involvement – 70% pediatric patients – colicky
abdominal pain + nausea , vomiting , diarrhea ,
constipation
 Frequently – passage of blood + mucus per rectum
 Intussception may occur

14. Renal involvement – 10-50% patients
Mild glomerulonephritis – proteinuria, microscopic
hematuria
RBC casts
In adults – M/C skin + joint involvement. GI less
common
Myocardial involvement maybe seen

15. LABS
Mild leucocytosis
Normal platelet count
Occasionally eosinophilia
Serum complement levels – normal
IgA levels elevated in 50% patients

16. DIAGNOSIS
Clinical
Skin biopsy – leukocytoclastic vasculitis
IgA and C3 deposition – immunofluorescence

17. TREATMENT AND PROGNOSIS
Excellent prognosis
Prednisolone – 1 mg/kg/day , taper according to
response
Patients with RPGN – intensive plasma exchange +
cytotoxic drugs
Recurrence – 10-40% pts

18. CRYOGLOBULINEMIC VASCULITIS
 Cryoglobulins – cold-precipitable monoclonal or polyclonal
immunoglobulins
 Maybe associated with systemic vasculitis
 Characterized by palpable purpura, arthralgia, weakness,
neuropathy, glomerulonephritis
 Associated with multiple myeloma, lymphoproliferative
disorders, connective tissue disease, maybe idiopathic
 Can be seen with hep C infection

19. PATHOPHYSIOLOGY
 Inflammatory infiltrate surrounding and involving blood
vessel walls
 Fibrinoid necrosis, endothelial cell hyperplasia,
haemorrhage
 Immunoglobulin and complement deposition – common
 MPGN – 80% of all renal lesions

20. CLINICAL MANIFESTATIONS
Cutaneous vasculitis
Arthritis
Peripheral neuropathy
Glomerulonephritis
Renal disease – 10-30% cases

21. LABS
Circulating cryopreciptitates +
RA factor +ve
Hypocomplementemia in 90% patients
ESR elevated
Anemia
Check for hep C infn  hep C RNA , antibodies to
hep C

22. TREATMENT AND PROGNOSIS
 Acute mortality uncommon.
 Presence of glomerulonephritis – poor prognostic sign ;
15% progress to ESRD, 40% later develop fatal CV disease,
infn or liver failure
 If hep C +ve – antiviral therapy maybe improve outcome
 Rituximab – can be used in combination with anti viral
therapy

23. KAWASAKI DISEASE
 Acute, febrile multisystem disease of children
 80% cases < 5yr age
 Peak incidence < 2yr
 Non suppurative cervical adenitis and changes in skin ,
mucus membranes
 Erythema of oral cavity, lips , palms
 Desquamation of skin of fingertips
 Benign and self limiting condition

24.  Coronary A aneurysm ~ 25% cases
 Usually it occurs in 3rd – 4th week of illness in convalescent
phase
 Pathophysio - > intimal proliferation and infiltration of the
vessel wall with mononuclear cells
 Beadlike aneurysms + thromboses along the artery
 Other manifestations – pericarditis, myocarditis, MI ,
cardiomegaly

25. Excellent prognosis
High dose IV γ-globulin ( 2g/kg single infusion
over 10h ) + aspirin (100mg/kg/day x 14 days )
followed by 3-5 mg/kg/d for several weeks 
effective in reducing prevalence of coronary A
abnormalities.
Surgery in pts with giant coronary A aneurysms or
other complications  thromboendarterectomy ,
thrombus clearing , aneurysmal reconstruction,
bypass sx.

26. THANK YOU