Poly arteries nodosa

1. POLY ARTERITIS NODOSA

2. CLASSIC POLYARTERITIS NODOSA
• It involves purely medium vessel vasculities
• It is a systemic vasculitis characterized by necrotizing inflammatory
lesions ,preferentially at vessel bifurcation resulting in
microaneyrysm formation,aneurysmal rupture with
hemorrhage,thrombosis,and,consequently,organ ischemia or
infarction.
EPIDEMIOLOGY
• PAN is a rare disorder
• With an incidence of about 3 to 4.5 cases per 100,000 population
anually.
• M=F
• Seen in 4th to 6th decade also seen in young patients

3. PATHOPHYSIOLOGY
• The exact mechanism is not known
• Vascular lesions in medium-sized muscular arteries occur mainly at
bifurcations and branch points .
• Inflammation may start in the vessel intima and progress to include the
entire arterial wall destroying the internal and external elastic lamina
resulting in fibrinoid necrosis.
• Aneurysm develop in the weakened vessel,carrying a subsequent risk for
rupture and hemorrhage
• Thrombi may develop at the site of lesions
• As lesion progress,proliferation of the intima or media may result in
obstruction and subsequent tissue ischemia or infarction
• It spares the large vessels [the aorta and its major branches],the smallest
vessels {capillaries andsmall arterioles},and the venous system
• Loss of function of mutations in CER1, the gene that encodes adenosine
deaminase 2 (ADA2),have ben associated with spectrum of vascular and
inflammatory phenotypes that includes PAN.

4. HISTOLOGY
• Fibrinoid necrosis in the vessel wall is present
• Transmural segmental necrotizing inflammation.All
stages of inflammation can be seen in a single vessel

5. ETIOLOGY
• Hepatitis B and PAN
• Hepaititis B virus {HBV} infection is strongly linked with
PAN. Evidence for immune complex-induced disease is
confined to HBV-related PAN;the role of immune
complexes in non-HBV-related PAN remains unclear.
• Impaired function of endothelial cells may be part of
idiopathic PAN or a consequence of it,in HBV-PAN virus
replication may directly injure the vessel wall.
• Endothelial dysfunction can perpetuate the inflammation
through cytokine and adhesion molecule production
• There is 30:1 rule in PAN and HBV

6. IMPORTANT FEATURES
• PAN do not present as
1. petechiae
2. Purpura
3. Vesicle
4. Bulla
• But presents as
1. Nodules
2. Deep ulcers
3. gangrene

7. • Glomerular capillaries and DAH are not
manifested instead,involvement of bronchial
arteries can cause hemoptysis
• No antibodies in PAN. ANCA negative
• Immune complexes and granulomas are absent
• It has transmural involvement with necrotising
vasculitis,fibrinoid necrosis,neutrophilic
infiltration.
• It is focal and segmental
• BAD prognosis commonly assosiated with Hep B,
hairy cell leukemia, and ADA-2 deficiency

8. Other Associations
• Varicella-zoster virus
• Parvovirus B-19
• Cytomegalovirus
• Human T-cell leukemia virus
• Rhematoid arthritis and sjogren syndrome
have been also associated with PAN

9. CLINICAL FEATURES
• PAN is an acute multisystem disease with a relatively
short prodrome (ie,weeks to months)
• Constitutional and musculoskeletal system sumptoms
of PAN includes
• Fever
• Malaise
• Fatigue
• Anorexia and weight loss
• Myalgia
• Arthralgia in large joints or less commonly arthritis

10. RENAL SYMPTOMS
• About 60% of patients have renal involvment
• Flank pain may be present
• Ischemic changes in the glomeruli and renal
artery vasculities can cause renal
failure,hypertension or both
• A small % of patients may require dialysis
• SYMPTOMS
• Hypertension
• Costophrenic tenderness
• Retroperitoneal or intraperitonial hemorrhage

11. CUTANEOUS SYMPTOMS
• Dermatalogic symptoms are very common in
PAN and about 405 of patients manifest with
skin lesions including
• Gangrene,nodules,cutaneous infarcts,livido
reticularis
• Livedo reticularis that does not blanch with
active pressure
• Ulcerations-Especially on lower extremities
near the malleoli and calf

17. CNS SYMPTOMS
• Cerebral arteritis ,which can cause cerebral ischemia and sub-archanoid
heamorrage late presentation usually in second to third year of vasculitis
• Acute or sub-acute myelopathy with paraparesis can occur at any cord level
• Mononeuritis multiplex –infarction of (radial,ulnar,peroneal,sural).although nerve
involvment is initially asymmetrical,the development of additional nerve lesions
can cause the clinical picture to resemble symmetrical polyneurophathy
• Rare less than 10% encephalopathy focal defecits strokes,seizures,and sometimes
brain haemorrages
PERIPHERAL NERVOUS SYSTEM SYMPTOMS :
1. Distal neurophathy
2. Cutaneous neurophathy
GASTROINTESTINAL SYMPTOMS :
• Abdominal pain which may be post prandial
• Nausea and vomiting with or without GI bleeding
• Rare and more serious complications are
• Bowel infarction and perforation
• Cholecystitis
• Hepatic infarction
• Pancreatic infarction

18. CARDIAC SYMPTOMS
• Hypertension
• Tachycardia out of proportion to fever
• Pericardial friction rub
• Arrythmias
• Congestive heart failure
OPTHALMOLOGIC SYMPTOMS
• Retinal vasculitis
• Retinal detachment
• Cotton wool spots

19. DIFFERENTIAL DIAGNOSIS
• Antiphospholipid syndrome
• Eosinophilic granulomatasis polyangitis
(churg-strauss syndrome)
• Granulomatosis with polyangiitis (wegener
granulomatosis)
• Henoch-schonlein purpura
• Small vessel vasculitis
• Microscopic polyangitis

21. DIAGNOSIS
• LAB findings ain PAN are nonspecific but can help to establish the
systemic nature of the disease
• Elevated ESR/C-reactive protein .These markers may be useful in
evaluating some patients for active disease but do not correlate
with activity in all patients
• Thrombocytosis
• Hepatitis B surface antigen and hepatitis C serologies
• Elevated creatinine levels
• Mild proteinuria
• Elevated liver enzymes
• Hypergammaglobulinemia
• Decreased levels of complements (ie,C3,C4)
• MR/CT ANGIOGRAM : microaneurysms are seen
• DANGER SIGNS : abdominal pain/renal and CNS involvement

23. MANAGEMENT OF THE PATIENT
• INVESTIGATIONS
• MR-angiography
• 5 FACTOR SCORE :
CNS,CVS,GIT,proteinuria,sr.creatinine
• ADA 2 in children : deficiency closely
resembles PAN. Pure cutaneous involvement
with small arteries and arterioles sparing the
venules.

25. ACR CRITERIA PAN :
1. Weight loss >4kg
2. Livido reticularis
3. Testicular pain
4. Mononeurophathy
5. Systemic hypertension ,diastolic BP > 90
6. HbsAg
7. Aneurysm on arteriogram
8. BIOPSY: necrotising vasculitis with granulocyte and
monocytes in arterial wall
9. Increase in blood urea or creatinine levels which is
not related with dehydration or obstruction

26. TREATMENT
• Immunosuppression continous to be standard
therapy for PAN
• Corticosteroids and cyclophosmide can prolong
survival for patients with idiopathic PAN
• For non-hep B patients : steroids and
cyclophosphamide(response is poor)
• For hep-B patients : steroids+Antiviral and
plasmopheresis (PLEX). (Response is poor )
• Antiviral agents like tenofovir alaenamide is used.

27. PROGNOSIS
Idiopsthic (non-HBV- related PAN) :
• Recovery from neurological defecits due to PAN can take upto 18
months. CNS involvement carries a worse prognosis than does
peripheral nerve involvement
• The prognosis is markedly worse in patients with acute abdominal
syndromes charecterized by extensive bowel involvement
• Multiple perforations may be found,relapse are common,and the
post operative course is complicated by infections and delayed
healing.
• The prognosisis better in patients with cutaneous PAN without
systemic involvement.This disease is benign and tends to relapse
HBV-related PAN :
• Patients who seroconvert usually recover
• Once HB-PAN goes into remission, the risk of recurrence is very low
HCV-related PAN
• One study found that in patients with HCV-related vasculitis, HCV
PAN exhibits a more severe clinical presentation but a higher rate of
clinical remission.

28. THANK YOU