Polyarteritis nodosa

The polyarteritis nodosa (PAN, also Kussmaul -Maier disease) is a vasculitis (inflammation of blood vessels), which belongs to the group of autoimmune diseases. She will be classified as ANCA -negative vasculitis of medium-sized vessels, there is inflammation of the small and medium-sized arteries with a perlschnurartigen arrangement of the inflammatory nodules, with particular calves, forearms and inner organs are affected.

Identification and Nomenclature

The PAN is also known as periarteritis nodosa, polyarteritis nodosa or polyarteritis Kussmaul -Maier. According to the Chapel Hill Consensus Conference of 1994, however, polyarteritis nodosa, the official name. Furthermore, it is more frequent than the CPAN ( classic PAN), abbreviated to distinguish them from the microscopic polyangiitis (MPA or MPAN ), but which is now regarded as a separate disease.

Epidemiology

The PAN is a rare disease whose annual incidence is assumed to be 5 per 100,000 inhabitants. The disease affects more men (male: female = 3:1)

Cause

An exact cause of primary ( idiopathic ) PAN is not known as in almost all autoimmune diseases. However, 30% of cases with chronic hepatitis C (predominantly genotype 2) or hepatitis B are associated, which is referred to as secondary polyarteritis nodosa ..

Pathogenesis

During inflammation there is a necrosis of the vessel wall with the infiltration of neutrophils. By necrosis, it leads to the narrowing of the vessel with thrombosis and infarction of the terminal vascular bed in the affected tissues.

Symptoms

• General: in almost 95 % of patients experience non-specific symptoms such as fever ( > 38.5 ° C), muscle and joint pain, night sweats and weight loss.
• Nervous System: The majority ( 80%) of those affected has a neuropathy that typically affects larger, mixed nerves. Moreover, an involvement of the central nervous system, which manifests itself in vision problems, strokes or seizures, may occur. However, it is usually caused by the existing secondary hypertension.
• Gastrointestinal: colicky abdominal pain, bowel infarction, bleeding
• Kidneys: microaneurysms the renal vessels can lead to secondary hypertension. A glomerulonephritis is atypical for the PAN and indicates an MPA.
• Heart: Angina pectoris, myocardial infarction
• Skin: livedo reticularis, subcutaneous nodules aneurysms ( nodi ) palpable

Pulmonary involvement is usually found and is not indicative of an MPA.

In the laboratory, one finds CRP and erythrocyte sedimentation rate increased as a sign of inflammation. It comes to leukocytosis. The disease is usually seen as a negative ANCA.

Diagnosis

According to the ACR criteria, the diagnosis of the PAN can be provided if at least 3 of the following criteria are met 10.

Differential Diagnosis

• Microscopic polyangiitis
• Other vasculitides
• Systemic lupus erythematosus

Therapy

The PAN is usually treated with corticosteroids and cyclophosphamide or azathioprine. As part of a hepatitis B and this is treated, resulting in the improvement of symptoms.

Since the success of treatment depends crucially on the severity of the polyarteritis nodosa, most have been subdivided according to a five-factor score. In a mild form ( score = 0) 50% of patients achieve remission with glucocorticoids alone, although 40 % suffer a relapse and then need an additional immunosuppressive agent - usually Cyclopshosphamid or azathioprine. In severe forms or in renal infarction and visceral investments is usually started immediately with a combination therapy. Although this lack comparative analyzes, but is usually done several weeks of high-dose therapy with glucocorticoids. Then the dose is gradually reduced step by step over six to twelve months. Cyclophosphamide is no more than a year, mostly given because of the toxicity.

Most ACE inhibitors are used for high blood pressure often occur due to illness.

Forecast

If left untreated, the five-year survival rate is only 13 %, with especially kidney failure, heart attack or stroke lead to death. With proper treatment, the five-year survival rate is 80 %, however.

First description

The description of " periarteritis nodosa " 1866 is to be regarded as a classic work of medicine. The clinician Adolf Kussmaul and the pathologist Rudolf Robert Maier demonstrated here a shining example of their interdisciplinary collaboration. The authors presented the case of the journeymen tailors Carl Seufarth who had visited in May 1865, the signs of an acute disease process (anemia, weakness, " chlorotic marasmus " ), the Freiburg clinic. The patient's condition steadily deteriorated and there were a general muscle weakness with hyp - or hyper-aesthetic effects of skin and muscles, insomnia and achiness added: " On May 30, were discovered under the skin of the abdomen and chest small pea-sized nodules. " Seufarth died on June 3.

Autopsy revealed especially numerous nodular thickenings present along the arteries of small to medium caliber, especially in the coronary arteries of the heart, the arteries of stomach, intestine, kidney, spleen and muscle. Microscopic examination revealed strong cell growths of the affected vessels in the area of ​​media and adventitia, less the intima. Arteries were occasionally aneurysmal expanded or narrowed by fibrous tissue formation and thrombosis. The kidneys showed the picture of a chronic diffuse nephritis ( " Bright 's disease "). Then the causes of this disorder remained a mystery to the authors.

Kussmaul and Maier submitted not only the detailed description of the clinical picture and the macro - or microscopic substrate, but also found the appropriate name for this disease, since they thought that the inflammatory processes progressed centripetally into the inner vessel wall layers. They recognized the inflammatory nature of the disease and the multiple aneurysm formation as the cause of abnormal nodule formation on arterial vessels. Finally, they led in case of suspicion (now obligatory ) biopsies and gave therapeutic indications.

Swell

• Autoimmune