Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that causes inflammation of small and medium arteries. It predominantly affects the arteries of the kidneys, heart, liver, and gastrointestinal tract, causing ischemia and tissue damage. The pathology is characterized by segmental necrotizing inflammation of the arterial walls. PAN is diagnosed based on biopsy of an affected organ showing arteritis or angiography demonstrating aneurysms of small and medium arteries. Treatment involves high-dose glucocorticoids and cyclophosphamide to induce remission, though relapse can occur in 10-20% of cases.

1. POLY ARTERITIS NODOSA
Dr Upendra Rathore

2. • Vasculitis is a clinicopathologic process characterized by
inflammation of and damage to blood vessels.
• Vasculitis and its consequences may be the primary or sole
manifestation of a disease; alternatively, vasculitis may be a
secondary component of another disease.

3. Blood vessels

4. CLASSIFICATION OF VASCULITIS
LARGE SIZED VESSELS
– Temporal arteritis
– Takayasu’s arteritis
MEDIUM SIZED VESSELS
– Polyarteritis nodosa
– Kawasaki’s disease
SMALL SIZED VESSELS
– Wegener’s granulematosis
– Churg-Strauss syndrome
– Microscopic polyangiitis

6. Polyarteritis Nodosa
• Disease of young adults.
• There is segmental necrotizing inflammation of arteries of
medium to small size, in any organ (esp kidney) except the
lung(pulmonary vessels).
• Granulomas, significant eosinophilia, and an allergic diathesis
are not observed.
• Most frequently affected organs are kidneys, heart, liver, and
gastrointestinal tract.

7. Polyarteritis Nodosa
• Clinical manifestations result from ischemia and infarction of
affected tissues and organs.
• Weakening of the arterial wall due to the inflammatory process
may cause aneurysmal dilation or localized rupture. Aneurysmal
dialatations of 1 cm are characteristic of PAN.
• In the acute stages of disease, polymorphonuclear neutrophils
infiltrate all layers of the vessel wall and perivascular areas,
which results in intimal proliferation and degeneration of the
vessel wall. Mononuclear cells infiltrate the area as the lesions
progress to the subacute and chronic stages

8. • The pathology in the kidney in classic polyarteritis nodosa is
that of arteritis without glomerulonephritis.
• Polyarteritis nodosa has been associated with hepatitis B or
hepatitis C infection and hairy cell luekemia.

9. CLINICAL MANIFESTATIONS
• Nonspecific signs and symptoms are the hallmarks of
polyarteritis nodosa. Fever, weight loss, and malaise are
present in over one-half of cases.
• Patients usually present with vague symptoms such as
weakness, malaise, headache, abdominal pain, and myalgias
that can rapidly progress to a fulminant illness.
• In polyarteritis nodosa , renal involvement most commonly
manifests as hypertension, renal insufficiency, or hemorrhage
due to microaneurysms.

11. Polyarteritis Nodosa
Ischemic ulcers

12. Lab Investigations
• There are no diagnostic serologic tests for polyarteritis
nodosa.
• In >75% of patients, the leukocyte count is elevated with a
predominance of neutrophils.
• Elevated ESR and anemia are present.
• Eosinophilia is seen only rarely.
• Hypergammaglobulinemia may be present, and all patients
should be screened for hepatitis B and C.
• Antibodies against myeloperoxidase or proteinase-3 (ANCA)
are rarely found in patients with polyarteritis nodosa.

13. • In polyarteritis nodosa, small aneurysms are strung like the
beads of a rosary, therefore making "rosary sign" an
important diagnostic feature of the vasculitis.

14. Diagnosis
• Its made by biopsy of the involved organ/vessel.
• Arteriographic demonstration of involved vessels, particularly
in the form of aneurysms of small and medium-sized arteries
in the renal, hepatic, and visceral vasculature.

15. • A patient is said to have polyarteritis nodosa if he or she has three of the
10 signs known as the 1990 American College of Rheumatology (ACR)
criteria:
• Weight loss greater than/equal to 4.5 kg
• Livedo reticularis (a mottled purplish skin discoloration over the
extremities or torso)
• Testicular pain or tenderness (occasionally, a site biopsied for diagnosis)
• Muscle pain, weakness, or leg tenderness
• Nerve disease (either single or multiple)
• Diastolic blood pressure greater than 90 mmHg (high blood pressure)
• Elevated kidney blood tests (BUN greater than 40 mg/dl
or creatinine greater than 1.5 mg/dl)
• Hepatitis B or hepatitis C virus tests positive (for surface antigen or
antibody)
• Arteriogram (angiogram) showing the arteries that are dilated
(aneurysms) or constricted by the blood vessel inflammation
• Biopsy of tissue showing the arteritis (typically inflamed
arteries): The sural nerve is a frequent location for the biopsy.

16. Polyarteritis nodosa with segmental inflammation and fibrinoid necrosis and occlusion of the lumen of
this artery. Note that part of the vessel wall at the left side is uninvolved.

17. Prognosis
• The prognosis of untreated polyarteritis nodosa is extremely
poor, with a reported 5-year survival rate between 10 and
20%.
• Death usually results from gastrointestinal complications,
particularly bowel infarcts and perforation, and cardiovascular
causes.
• Intractable hypertension often compounds dysfunction in
other organ systems, such as the kidneys, heart, and CNS,
leading to additional late morbidity and mortality in
polyarteritis nodosa.

18. Treatment Of PAN
• Prednisolone and cyclophosphomide combination is used.
• In less severe cases of polyarteritis nodosa, glucocorticoids
alone have resulted in disease remission.
• In patients with hepatitis B who have a polyarteritis nodosa–
like vasculitis, antiviral therapy represents an important part
of therapy and has been used in combination with
glucocorticoids and plasma exchange.
• Following successful treatment, relapse of polyarteritis
nodosa has been estimated to occur in 10–20% of patients.

19. Particularly characteristic of PAN is that all the
different stages of activity ( i.e. active and
chronic stages) may coexist in same artery or
in different vessels at the same time.
Fatal if untreated, but steroids and
cyclophosphamide are curative.