Polyarteritis nodosa
| Classification according to ICD-10 | |
|---|---|
| M30.0 | Polyarteritis nodosa |
| ICD-10 online (WHO version 2019) | |
The polyarteritis nodosa (PAN, even Kussmaul-Maier disease ) is a vasculitis (inflammation of blood vessels), to the group of autoimmune diseases belongs. It is classified as ANCA -negative vasculitis of medium-sized vessels, it comes to inflammation of the smaller and medium arteries with a pearl-like arrangement of the inflammatory nodules, whereby the calves, forearms and internal organs are particularly affected.
Name and nomenclature
PAN is also known as periarteritis nodosa , panarteritis nodosa and panarteritis Kussmaul-Maier . However, according to the 1994 Chapel Hill Consensus Conference, polyarteritis nodosa is the official name. It is also more commonly referred to as cPAN. (classic PAN) abbreviated to distinguish it from microscopic polyangiitis (MPA or mPAN), which is nowadays viewed as a separate disease.
Epidemiology
PAN is one of the rare diseases. About three of every million people develop PAN every year. Men get sick about twice as often as women, mostly in middle age
root cause
As with almost all autoimmune diseases, the exact cause of primary (idiopathic) PAN is not known. However, 30% of the cases are associated with chronic hepatitis C (predominantly genotype 2) or hepatitis B , which is known as secondary polyarteritis nodosa.
Pathogenesis
During the inflammation leads to a necrosis of the vessel wall with infiltration of neutrophilic granulocytes . The necrosis leads to a constriction of the vessel with thrombosis and infarction of the terminal vascular system in the affected tissues.
Symptoms
- General: Almost 95% of patients experience unspecific symptoms such as fever (> 38.5 ° C), muscle and joint pain, night sweats and weight loss.
- Nervous system: The majority (80%) of those affected have neuropathy that typically affects larger, mixed nerves . Involvement of the central nervous system , which manifests itself in visual disturbances, strokes or seizures , can also occur. However, it is usually secondary to the existing high blood pressure .
- Digestive tract: colicky abdominal pain, intestinal infarction, bleeding
- Kidneys: Microaneurysms of the kidney vessels can lead to secondary high blood pressure . A glomerulonephritis is atypical for the PAN and has an MPA down.
- Heart: angina pectoris , heart attack
- Skin: livedo racemosa , subcutaneous aneurysms as nodules ( nodi ) palpable
Lung involvement is usually not found and is indicative of MPA .
In the laboratory, CRP and erythrocyte sedimentation rate are found to be increased as signs of inflammation . Leukocytosis occurs . The condition is usually considered ANCA negative.
diagnosis
According to the ACR criteria, the diagnosis of PAN can be made if at least 3 of the following 10 criteria are met.
- Weight loss> = 4 kg that cannot be attributed to other causes
- Livedo racemosa
- Pain or tenderness in the testicles that cannot be attributed to other causes
- Weakness and muscle pain
- Mono- or polyneuropathy
- high blood pressure
- Increase in serum creatinine or serum urea
- Evidence of hepatitis B or hepatitis C infection
- angiographic abnormalities
- corresponding pathological finding of an artery biopsy
Differential diagnosis
- microscopic polyangiitis
- other vasculitis
- systemic lupus erythematosus
therapy
PAN is usually treated with corticosteroids and cyclophosphamide or azathioprine . As part of hepatitis B, this is also treated, which leads to an improvement in the symptoms.
Since the success of the treatment depends crucially on the severity of the polyarteritis nodosa, a classification is usually based on 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 immunosuppressant - usually cyclopshosphamide or azathioprine. In severe forms or with kidney infarction and visceral involvement, a combination therapy is usually started immediately. There are no comparative studies on this, but high-dose therapy with glucocorticoids for several weeks is usually carried out. Then the dose is slowly reduced gradually over six to twelve months. Cyclophosphamide is usually not given for more than a year because of its toxicity .
ACE inhibitors are mostly used for high blood pressure , which often occurs due to illness .
forecast
If left untreated, the five-year survival rate is only 13%, with kidney failure , myocardial infarction or stroke in particular leading to death. With the right therapy, however, the five-year survival rate is 80%.
Initial description
The description of "Periarteritis nodosa" in 1866 is to be regarded as a classic work of medicine. The clinician Adolf Kussmaul and the pathologist Rudolf Robert Maier demonstrated their interdisciplinary cooperation in an exemplary manner. The authors presented the case of the journeyman tailor Carl Seufarth, who had visited the Freiburg clinic in May 1865 with signs of an acute illness (anemia, feeling weak, “chlorotic marasmus”). The patient's condition deteriorated noticeably, general muscle weakness with hyp or hyperesthetic effects of the skin and muscles, insomnia and diffuse pain appeared : “On May 30th, small pea-sized nodules were discovered under the skin of the abdomen and chest. “Seufarth died on June 3rd.
Above all, the dissection revealed numerous nodular thickenings along the arteries of small to medium caliber, in particular on the coronary arteries of the heart , the arteries of the stomach , intestines , kidneys , spleen and on the muscles . The microscopic examination revealed strong cell growths in the affected vessels in the area of the media and adventitia , less so in the intima . Arteries were occasionally enlarged aneurysmally or narrowed by the formation of connective tissue and thrombosis . The kidneys showed the appearance of chronic, diffuse nephritis (" Bright's disease "). For the authors at the time, the causes of this disease remained a mystery.
Kussmaul and Maier not only provided a detailed description of the clinical picture and the macro- or microscopic substrate, but also found the appropriate name for this clinical picture, as they assumed that the inflammatory processes progressed centripetally into the inner layers of the vessel wall. They recognized the inflammatory nature of the disease and the multiple aneurysms as the cause of the noticeable nodule formation on arterial vessels. Finally, in the event of suspicion, they performed biopsies (now mandatory) and provided therapeutic advice.
Web links
Individual evidence
- ↑ JC Jennette et al. a .: Nomenclature of systemic vasculitides. Proposal of an international consensus conference. In: Arthritis Rheum . (1994) 37, p. 187. PMID 8129773
- ↑ Wolfgang Brückle: German Rheuma League - information sheet Panarteriitis nodosa (Polyarteriitis nodosa). 3. Edition. 2013.
- ↑ Gerd Herold: Internal Medicine. 2005, p. 579.
- ↑ RW Lightfoot et al. a .: The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. In: Arthritis Rheum. (1990), 33, p. 1088. PMID 1975174 .
- ^ Russell Goodman, Paul F. Dellaripa, Amy Leigh Miller, Joseph Loscalzo: An Unusual Case of Abdominal Pain. In: New England Journal of Medicine , 2014, Volume 370, Issue 1, Jan. 2, 2014, pp. 70-75; doi: 10.1056 / NEJMcps1215559
- ^ RH Major: Classic Descriptions of Disease. 3. Edition. Springfield 1948, p. 245.
- ↑ A. Kussmaul, R. Maier: About a peculiar arterial disease (periarteritis nodosa) that has not yet been described, which is associated with Brightii disease and rapidly progressing general muscle paralysis. In: Dtsch Arch Klin Med. , 1, 1866, p. 484.
