(Polyarteritis nodosa periarteritis)
The polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that typically affects medium-sized and occasionally small muscular arteries muscular arteries and leads to secondary tissue ischemia. Kidneys, skin, joints, muscles, peripheral nerves and gastrointestinal tract are most often affected, but any organ can be affected. However, the lungs usually remain spared. Patients present themselves usually with systemic symptoms (eg., Fever, fatigue). The diagnosis is made by biopsy or arterial angiography. Treatment with corticosteroids and immunosuppressive drugs is effective.
A polyarteritis nodosa (PAN) is a rare disease (about 2-33 cases / million.). It mainly affects adults in middle age; the incidence increases with age too, and peaked at people in the 6th decade of life.
The polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that typically affects medium-sized and occasionally small muscular arteries muscular arteries and leads to secondary tissue ischemia. Kidneys, skin, joints, muscles, peripheral nerves and gastrointestinal tract are most often affected, but any organ can be affected. However, the lungs usually remain spared. Patients present themselves usually with systemic symptoms (eg., Fever, fatigue). The diagnosis is made by biopsy or arterial angiography. Treatment with corticosteroids and immunosuppressive drugs is effective. A polyarteritis nodosa (PAN) is a rare disease (about 2-33 cases / million.). It mainly affects adults in middle age; the incidence increases with age too, and peaked at people in the 6th decade of life. Etiology Most cases are idiopathic. Approximately 20% of patients suffering from hepatitis B or C. The etiology is unknown but it is assumed that an immunologically mediated etiology. The variety of clinical and pathological findings suggests multiple pathogenic mechanisms. Even drugs can play a triggering role. Usually there is no predisposing antigen. In patients with certain lymphomas and leukemias, RA or Sjögren’s syndrome, systemic vasculitis, which is similar to the PAN developed (sometimes also referred to as secondary PAN). Pathophysiology The PAN is characterized by a segmental or transmural necrotizing inflammation occurring in muscular arteries, most commonly found in the range of bifurcations. Unlike other vasculitis postcapillary venules or veins are not affected in the PAN. The lesions are commonly found in all stages of formation and healing. Fresh lesions contain polymorphonuclear leukocytes and occasional eosinophils, older lesions rather lymphocytes and plasma cells. Granulomatous inflammation does not occur. An intimal proliferation with secondary thrombosis and vascular occlusion and tissue infarctions due organ. The instability of the muscular artery wall can cause small aneurysms and arterial dissection. A healing often leads to nodular fibrosis of the adventitia. Most commonly, kidney, skin, peripheral nerves, joints, muscles and the gastrointestinal tract are affected. the liver and the heart are affected. At the kidney to ischemia and infarction show, however, the glomerulonephritis is not a feature of the PAN. Purpura (usually caused by small vessel inflammation) is not a feature of the PAN. Symptoms and complaints The PAN can mimic many other diseases, their course is acute or protracted, subacute with fatal turn after several months or even insidiously in the form of a chronic and debilitating disease. The symptoms of PAN depend mainly on the location and severity of arteritis and the extent of secondary ischemia. Perhaps only one organ or organ system is affected. Patients present themselves usually with fever, fatigue, night sweats, loss of appetite, weight loss and general weakness. Myalgia with focal areas of ischemic myositis and arthralgias are common. Affected muscles are painful to touch and may be weak. Arthritis can occur. Signs and symptoms vary depending on which organ or organ system is mainly affected: Peripheral nervous system: Patients suffer usually under asymmetric peripheral neuropathy as a multiple mononeuropathy mononeuritis multiplex with signs of motor and sensory involvement of peroneal, median or ulnar nerves. If extra nerve branches are affected, patients seem to have a distal symmetrical polyneuropathy. CNS: Headache and seizures may occur. Some patients experience stroke and brain hemorrhage, which is sometimes due to hypertension. Renal: If small and medium-sized arteries are affected in the kidneys, patients show hypertension, oliguria, uremia and a non-specific urinary sediment with hematuria, proteinuria missing cell cylinders. Hypertension is deteriorating rapidly. Rupture of renal arterial aneurysm can cause perirenal hematoma. In severe cases, multiple renal infarcts with back pain and hematuria may occur. Renal ischemia and infarction can lead to kidney failure. Gastrointestinal: Vasculitis the liver or gall bladder causes pain in the right upper quadrant. A perforation of the gallbladder with acute abdomen may occur. Vasculitis of medium-sized mesenteric arteries causes abdominal pain, nausea, vomiting (with or without bloody diarrhea), malabsorption, intestinal perforation and acute abdomen. In the arteries of the liver or the celiac artery aneurysms can develop themselves. Heart: Some patients have coronary heart disease, which is usually asymptomatic, but leads to angina. Ischemic cardiomyopathy or hypertension can lead to heart failure. Kutan: livedo reticularis, skin ulcers, druckdolente, reddened nodules, bullous or vesicular rash, infarction and gangrene of the fingers or toes, or a combination thereof are described. The nodules in PAN are similar to those in erythema nodosum, but unlike these, the nodules may ulcerate at PAN and show necrotizing vasculitis within the walls of medium-sized arteries, which is visible in Bioptat, usually localized in the deep dermis and subcutaneous adipose tissue , Genital: A orchitis with testicular pain and tenderness may occur. Diagnosis Clinical findings biopsy arteriography, if not clinically involved tissue is available for a biopsy due to the non-specific findings, a PAN can be difficult to diagnose. The diagnosis should be considered in patients with various combinations of symptoms such as unexplained fever, arthralgia, subcutaneous nodules, skin ulcers, pain in the abdomen or limb, a new-onset foot drop or a newly emerged case hand or a rapidly developing hypertension. The diagnosis clarifies further if the clinical findings are merged with the laboratory results and other causes can be excluded. The diagnosis of PAN is confirmed by biopsy showing a necrotizing arteritis, or by arteriography showing the typical aneurysms in the medium-sized arteries. Magnetic resonance angiography can show the micro-aneurysms, but some abnormalities may be too small to detect them. Therefore, magnetic resonance angiography is the method of choice to be used for diagnosis. The biopsy of clinically uninvolved tissue is often useless because the disease is focal. The biopsy should be performed in clinically suspicious areas. Samples of the subcutaneous tissue, the sural nerve and the muscles when they are considered affected, compared to samples from the kidneys or liver preferred; Kidney and liver biopsies may be false-negative and cause bleeding by unexpected microaneurysms basis of an examination error. If clinical findings completely missing or very pronounced, electromyography and studies can help to nerve to select the most appropriate place for a biopsy of muscle or nerve. If lesions are present, should surgical skin biopsies that include the deeper dermis and the subcutaneous fat, done. (In punch biopsies utilizing the epidermis and oberflächlichliche dermis for the tissue sample of the skin, lack the lesions of the PAN.) Although microscopic lesions are common in the testes, a testicular biopsy should not be performed if testicular symptoms are absent and other possible areas are accessible because the success rate is low. In addition, men are unwilling to conduct an testicular biopsy. Laboratory diagnosis provides only non-specific results. These include most leukocytosis of 20,000 to 40,000 / ul, proteinuria and microscopic hematuria. Patients present with thrombocytosis, a significantly elevated ESR, anemia (caused by blood loss or kidney failure), hypoalbuminemia and an increase in serum immunoglobulins. AST and ALT are often slightly increased. A study on hepatitis B and C should be done. Other studies (eg. As anti-neutrophil cytoplasmic antibodies [ANCA], rheumatoid factor, anti-cyclic citrullinated peptide antibody [anti-CCP], antinuclear antibodies [ANA], C3 and C4 complement levels, Kryoglobulinspiegel, nuclear antigens and antibodies to extractable nuclear antigens such as anti-Smith, anti-ro / SSA, anti-La / SSB and anti-RNP) may suggest other diagnoses such as RA, SLE or Sjogren’s syndrome. Prognosis Without treatment, is the 5-year survival rate of <15%. With treatment, the 5-year survival rate is> 80%, but can also be lower in patients with hepatitis B. The prognosis is better if a remission of the disease is achieved within 18 months of diagnosis. Setbacks are less frequent than in other vasculitic diseases. The following findings are associated with a poor prognosis: renal failure, gastrointestinal involvement neurological involvement therapy corticosteroids alone or with cyclophosphamide, methotrexate or azathioprine, depending on the severity of the disease addition, administration of lamivudine and plasma exchange in patients with hepatitis B Treatment of PAN depends on the severity of the disease. In systemic symptoms, but without severe neurological, renal, gastrointestinal or cardiac manifestations, corticosteroids may be sufficient, at least initially. In a severe disease with neurological, renal, gastrointestinal or cardiac manifestations improve cyclophosphamide plus corticosteroids earnings. With moderate disease corticosteroids plus methotrexate or azathioprine can be used. Hypertension should be treated effectively. Hepatitis-B-associated PAN Treatment is aimed at the rapid suppression of inflammation, in addition to the elimination of the virus and the initiation of a seroconversion via plasma exchange. A short-term use of corticosteroids is carried out for a few weeks. Lamivudine (100 mg po once daily) administered for up to 6 months. A lower dose is used in patients with renal insufficiency. Plasma exchange is planned as follows: 3 times / week for 3 weeks, 2 times / week for 2 weeks and 1 times / week until hepatitis B e antigen (HBeAg) in hepatitis B e antibody (anti-HBe is converted) or until clinical recovery is maintained for 2-3 months. Although there is no evidence that this therapeutic approach improves survival compared to immunosuppressive therapy, it can suppress the risk of long-term complications of hepatitis B and suppress the side effects of long-term treatment with corticosteroids and immunosuppressants. The traditional treatment with corticosteroids, sometimes with cytotoxic immunosuppressive drugs (mainly cyclophosphamide), often proved to be effective for a short time, but could relapses and complications (eg. As chronic hepatitis, cirrhosis) due to persistence of hepatitis B virus does not verhinderen. Immunosuppressive therapy in patients with hepatitis B facilitates viral replication, which can lead to an active viral hepatitis and liver failure. Patients with hepatitis C who develop a PAN are treated as Hepatitis C. Summary PAN is a rare systemic vasculitis of medium-sized arteries. Kidneys, skin, joints, muscles, peripheral nerves and gastrointestinal tract are most often affected. Suspicion of PAN exists when patients show a combination of unexplained fever, joint pain, subcutaneous nodules, skin ulcers, pain in the abdomen or limb, new aufgetretem foot drop or new onset cases hand or rapidly entwickelndem hypertension. The diagnosis is confirmed by biopsy or arteriography. Renal, gastrointestinal or neurological involvement means a poorer prognosis. The treatment consists of the administration of corticosteroids or cyclophosphamide, methotrexate or azathioprine alone, depending on the severity of the disease.