Polyarteritis nodosa

Poly arteritis nodosa

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

5. 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.

6. 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

7. 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 .

8. 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 .

9. Polyarteritis Nodosa Ischemic ulcers

10. 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 .

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

12. 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.

13. 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.

14. 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.

15. 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 .

16. 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.

17. 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.