"The causes are unknown and treatment will depend on the number of organs affected".
DR. Mª CRUZ LAIÑO PIÑEIRO
SPECIALIST. RHEUMATOLOGY SERVICE
Panarteritis nodosa (PAN) is a disease that causes inflammation of the blood vessels, which is known as vasculitis. Since it can also occur with destruction of the affected vessels, it is called necrotizing vasculitis.
It affects medium and small-caliber muscular arteries, although it can also affect other vessels such as arterioles and blood vessels.
It is considered a systemic disease, since any organ or system can be affected by this disease.
It is a very rare disease. Its incidence is estimated at around 4-10 cases per million inhabitants and it mainly affects men in a proportion at least double that of women.
Although it can appear at any age, it is more frequent between the 5th and 7th decade of life, and is exceptional in childhood.
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What are the symptoms of panarteritis nodosa?
The clinical presentation is very variable. The symptoms can be divided into two large groups: general symptoms, as a consequence of the generalized inflammatory process, and local symptoms, depending on the organ or vascular territory affected.
The general manifestations include fever, asthenia, slimming, arthromyalgia and affectation of the general state. The most frequent of all of them is the fever and in some occasions it can be the only symptom that the patient presents/displays.
The located manifestations, are very variable and although in principle all the organs can be seen involved, the kidney is the one that is affected with greater frequency.
Most common symptoms:
Do you have any of these symptoms?
You may have panarteritis nodosa
What are the causes of panarteritis nodosa?
As with most vasculitis, the causes are unknown.
However, in some patients it has been seen in association with other diseases such as hepatitis B virus, hepatitis C, inflammatory bowel disease. It has also been described in hairy cell leukemia (or tricholeukemia), as well as in rheumatoid arthritis and lupus.
What is your prognosis?
The prognosis of these patients depends largely on the degree of injury and the number of organs affected.
However, it has varied substantially based on the incorporation of the various treatments mentioned above. Before the introduction of corticoids, survival at 5 years was around 10-15%; it rose to 50-60% after their incorporation, and to 80% with the association of cyclophosphamide.
In return, vascular and infectious complications have increased in relation to the increased survival achieved and by immunosuppressive therapy.
How is panarteritis nodosa diagnosed?
In panarteritis nodosa, there is no complementary test or study that can define with certainty the diagnosis of the disease.
Analytically, there are parameters that although unspecific, are frequent and can guide the diagnosis, such as increased sedimentation rate, inflammatory anemia, discrete eosinophilia and elevation of acute phase reactants.
There are criteria established by the American College of Rheumatology (ACR) for the diagnosis of PAN. The presence of at least three of these criteria provides a sensitivity and specificity for diagnosis above 80%.
- Weight loss greater than or equal to 4 kg. since the onset of the disease.
- Livedo reticularis. Mottled reticular pattern on skin portions of extremities and trunk.
- Testicular pain or inflammation.
- Myalgia, weakness or sensitivity to touch in the legs.
- Mononeuropathy or polyneuropathy.
- Diastolic blood pressure greater than 90 mm. Hg.
- Elevation of urea nitrogen (BUN> 40 mg./dL.) or creatinine (> 1.5 mg./dl.)
- Presence of surface antigen or antibody to hepatitis B virus in serum.
- Pathological arteriography (microaneurysms or thrombosis)
- Biopsy of medium or small size arteries: Presence of granulocytes and mononuclear leukocytes in the arterial wall.
How is panarteritis nodosa treated?
The treatment of PAN is aimed at treating the inflammatory and immunological mechanism by which vascular lesions are produced.
In this sense, glucocorticoids (Prednisone) constitute the main therapeutic weapon against this disease.
When the degree of affectation is serious, immunosuppressive drugs must be associated to try to stop the immune mechanism. On the other hand, it is of vital importance the correct treatment of the local manifestations, mainly the arterial hypertension, the renal insufficiency and the neurological affectation.
In cases of refractory vasculitis, intravenous immunoglobulins and anti-TNFs may be useful. Plasmapheresis is reserved for Hepatitis B virus-associated PAN and for refractory vasculitis, especially in severe complications such as pulmonary hemorrhage or renal failure requiring dialysis.
Where do we treat it?
IN NAVARRA AND MADRID
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In addition, we have doctors specialized in assisting pregnant women with autoimmune diseases, in order to guarantee the maximum safety of the fetus.
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