Panarteritis nodosa (cPAN) – causes, symptoms & therapy

Panarteritis nodosa (cPAN)

Panarteritis nodosa is a chronic vascular disease from the so-called rheumatic group. The disease, also known as cPAN for short, is typically associated with inflammation of the blood vessels.

What is panarteritis nodosa?

The rheumatic vascular disease panarteritis nodosa, also known as polyarteritis nodosa or Kussmaul-Maier disease, is one of the vasculitides within the inflammatory rheumatic diseases. As a typical vasculitis, panarteritis nodosa is based on an inflammatory reaction of the blood vessels, with smaller and medium-sized arteries of the systemic circulation being particularly affected.

A constant inflammatory activity on the inner walls of the vessels, intima, is highly pathological and leads to a significant reduction in blood flow behind the damaged areas. As a result, the blood supply to all organs can be dangerously impaired, and in the event of a fulminant course, it can even come to a complete standstill. The term nodosa describes the tendency of the inflammatory process towards nodular changes. The syllable Pan denotes the fact that all vessels can be affected by the inflammatory changes.

causes

The exact causes of the development of vascular vasculitis have not yet been definitively deciphered. Kussmaul-Maier disease is considered a rare but serious condition. The affected patients are usually between 40 and 50 years old when the disease is fully developed. Less than 5 out of 100,000 people develop panarteritis nodosa each year, with men being affected more frequently than women.

It must be assumed that panarteritis nodosa is a disease in which a misguided, overactive immune system plays a major role. In these so-called autoimmune diseases , specific antibodies are formed in the blood of a patient against the body’s own structures for reasons that have not yet been clarified. The antigen-antibody complexes caused by this are in turn recognized by the immune system as foreign bodies which the body tries to get rid of.

That then explains the strong inflammatory reactions. Previous infections with the hepatitis B virus but also other serious infectious diseases also seem to promote the onset of the disease. The exact meaning of these associations with infections is also not exactly known. Certain genetic dispositions are also discussed by rheumatologists as at least one of the triggering causes of polyarteritis nodosa.

symptoms and course

Due to the infestation of large vascular sections, massive symptoms with a severely restricted quality of life can occur in the course of the chronic inflammatory disease. Panarteritis nodosa therefore always requires treatment.

Damage to the vessel walls can also lead to aneurysms , i.e. protrusions, which are also so difficult to control clinically because thrombi , i.e. blood clots, form there, which are carried away by the bloodstream and then lead to life-threatening occlusions, vascular infarctions, elsewhere can. As a result of the infarction, the affected tissue is no longer supplied with blood and threatens to die.

A permanent, strong feeling of illness with night sweats, fever and loss of appetite is predominant. In addition, the majority of those affected usually experience unbearable joint, muscle or abdominal pain. If blood vessels in the intestine become blocked, the dreaded mesenteric infarction can result, which if left untreated can quickly lead to death.

If the vascular occlusions occur intracranially, the result is strokes with severe headaches, dizziness, speech disorders or seizures. If the renal arteries are involved , both kidneys may lose their function completely, which can also be fatal without immediate dialysis. The skin and nervous system can also be affected.

Diagnose

Despite the usually fulminant course of the disease, patients often have to wait a long time for a correct diagnosis. This is due to the colorful bouquet of different symptoms that can be caused by this type of vasculitis. It is not uncommon for patients to present to all medical specialties, all of which make different diagnoses. In the majority of cases, the definitive diagnosis is made by a specialist in rheumatology using imaging methods, vascular examinations and histological microscopic findings from tissue samples. A suspected diagnosis can also be substantiated by immune parameters and other specific blood values.

treatment and therapy

Unfortunately, adequate therapy for vasculitis such as panarteritis nodosa is only given once the exact diagnosis has been made and corroborated by corresponding histological findings. However, an exacerbation can only be prevented if treatment is started as early as possible.

Today there are centers at some German university clinics that specialize in the treatment of patients with rheumatic vasculitis. Patients often stay there for several weeks or months, and no patient can be discharged as finally cured.

However, the symptoms can be reduced by high-dose immunosuppressive drugs in such a way that the quality of life can be restored at least to some extent.

All therapeutic measures with cyclophosphamide or glucocorticoids are aimed at reducing the inflammatory reactions on the inner walls of the vessels, but a cure is not possible. If hepatitis B is present at the same time, it should be adequately treated with antiviral therapy. The therapy for polyarteritis nodosa can only be symptomatic, a causal therapy is unfortunately not possible due to the unknown causes.

prevention

A direct prophylaxis against the occurrence of polyarteritis nodosa is not known. However, since it is considered certain that an infection with the hepatitis B virus promotes the development of the disease, everything should be avoided in terms of infection prophylaxis so that an infection with the hepatitis B virus does not occur. In this context, vaccination against hepatitis B is advisable.

Dorothy Farrar

Hello and welcome to my Health Guide & Encyclopedia! My name is Dorothy Farrar, and I'm the founder and main author of this platform.
My passion for health and wellness started at a young age when I became interested in the connection between the food we eat and the way we feel. This fascination led me to study nutrition and dietetics in college, where I learned about the importance of a balanced diet and the impact of various nutrients on the body.

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