VERIFIED CONTENTAuthor: lek. Katarzyna Banaszczyk

Nodular arteritis is one of the inflammatory vascular diseases, and thus it is part of a broad medical field called rheumatology. It is a rather rare disease as it is estimated to occur in 2 to 30 cases per million. What is polyarteritis nodosa? How is this disease manifested and what are the treatment options?

Nodular arteritis(Latin: polyarteritis nodosa) is a relatively rare disease that affects primarily medium and small arteries located in the kidneys, digestive tract and nervous system , the cardiovascular system, as well as in the skin, as well as in the testicles and epididymides. Vessels in many locations can be involved, which makes the clinical picture of the disease diverse and it is sometimes difficult to make a quick diagnosis. Polyarteritis nodosa is more common in men, and the mean age of onset is 40-60 years.

Nodular arteritis - pathophysiology of changes in the vessels

What exactly happens to the blood vessels during this condition? Inflammation affects the vessel in sections, which means that the affected fragments are intertwined with the unchanged sections.

The inflammatory process in the vessel wall leads to the formation of blood clots and dilatation of the vessels (which are called microtransmitters), as a result of which these vessels can rupture, and this results in ischemia of the organs and tissues vascularized by these arteries.

Blood clots, in turn, lead to a restriction of blood flow in the vessel and, consequently, also to ischemia.

Which organs are most often affected by polyarteritis nodosa? List here arterial vessels located within:

  • kidney - up to 90% of cases
  • hearts (around 70%),
  • liver (about 50-60%),
  • digestive system (50-70%),
  • pancreas and spleen (45%).

Causes of polyarteritis nodosa

Unfortunately, the exact causes of this disease are not known. Research shows that more than 10% of polyarteritis nodosa cases are associated with HBV or HCV infection, i.e. hepatitis C orhepatitis B.

Nodular arteritis, associated with the viruses causing hepatitis, is considered by some scientists to be a separate entity or secondary polyarteritis nodosa.

This is due to some differences - the disease accompanied by HBV or HCV infection responds well to treatment with antiviral drugs, and the clinical picture is dominated by skin lesions.

Symptoms of polyarteritis nodosa

The onset of the disease is characterized by the appearance of so-called general symptoms, not related to a specific body system. One of them is:

  • weakness,
  • pain in muscles and joints,
  • weight loss,
  • fever.

Symptoms related to the involvement of arterial vessels within a given organ or tissue appear only after a few months. Symptoms depend on the specific area involved and include:

  • in the case of nervous system involvement - nerve paralysis, muscle paralysis, manifested, for example, by foot drop, sensory disturbances, damage to the cranial nerves, and sometimes even central nervous system ischemia (strokes) and cerebral hemorrhages,
  • when the vessels in the kidneys are involved - arterial hypertension occurs, as well as a consequence of ischemic kidney infarction, with typical acute pain in the lumbar region and accompanying haematuria. Involvement of the kidneys can lead to kidney failure,
  • in the case of gastrointestinal vascular involvement - abdominal pain due to intestinal ischemia predominates, as well as nausea, vomiting, diarrhea or constipation,
  • in the case of vascular involvement in the heart - pericarditis is diagnosed, and the pathological involvement of the coronary vessels results in myocardial ischemia and the occurrence of symptoms of a heart attack,
  • when pulmonary vessels are involved - typically pulmonary infiltrates are diagnosed,
  • when vessels in the reproductive system are involved - typically unilateral orchitis occurs,
  • when vessels within the eyeball are involved - visual disturbances, amblyopia - these symptoms result from the involvement of the retinal vessels,
  • in the case of vascular involvement in the musculoskeletal system - patients report muscle pain, muscle atrophy, muscle weakness, there is also arthritis, especially of the large joints of the lower limbs.

Nodular arteritis and skin changes

Skin changes are quite a characteristic symptom of this disease. The following can be observed on the skin of patients:

  • reticular cyanosis - these are bluish, reticulated spots that may fit into the clinical picturemany rheumatological and systemic diseases, but the blueness can also be only a cosmetic defect,
  • painful subcutaneous nodules - most often located on the lower limbs, typically on the front surface of the shins and on the backs of the foot, their diameter usually does not exceed 2 cm, these nodules may ulcerate, leaving visible scars on the skin,
  • trophic changes to the fingers - resulting from ischemia of the distal parts of the limbs, may end with the amputation of the fingers.

Be aware that there is a cutaneous form of nodular arteritis. The changes affect only the skin, and other organs and systems of the body are not affected. This form of the disease rarely turns into a generalized form, usually the changes are limited to the skin. Cutaneous polyarteritis nodosa accounts for approximately 10% of all cases of this disease.

Diagnostics of polyarteritis nodosa

Unfortunately, there is no single specific marker that would allow this diagnosis to be confirmed. So what are the tests to diagnose polyarteritis nodosa? Inflammatory parameters should be determined, because the disease is accompanied by their increase - we are talking here about ESR and CRP concentration.

Deviations are also observed in the blood counts of peripheral blood. It is mainly:

  • leukocytosis - an increase in the number of white blood cells (leukocytes; WBC);
  • thrombocytopenia - an increased number of platelets (thrombocytes; PLT).

With kidney involvement, an increase in serum creatinine concentration is observed, while in the general urine examination there is proteinuria (presence of protein) and hematuria (red blood cells). HBs antigen and anti-HCV antibodies must be determined in order to diagnose the accompanying hepatitis, which, as already mentioned above, may be the cause of polyarteritis nodosa.

In terms of imaging tests, the examination of choice is arteriography, which allows the visualization of characteristic, segmental strictures within the vessels, as well as baggy aneurysms and thromboses. The vessels are alternately changed and unchanged - changes in arteriography are segmental.

The diagnosis is made on the basis of special criteria, which include:

  • weight loss - unintentional loss>4 kg without other reasons,
  • skin eruptions,
  • hypertension,
  • increase in creatinine concentration>1.5 mg / dl,
  • muscle pain,
  • testicular pain,
  • muscle strength reduction,
  • HBV antigen detection,
  • paralysis of peripheral nerves,
  • changes in arteries visualized on imaging,
  • typical changes in the taken tissue sample - most often it is a muscle fragment - however, you should be aware that the taken sample does not always contain pathologically changed vessels.

Treatment of polyarteritis nodosa

Treatment of the disease is based primarily on cytotoxic and immunosuppressive preparations, such as:

  • cyclophosphamide - during therapy with this drug, the prophylaxis of Pneumocystis jiroveci infection is also used (it is necessary to take an additional prophylactic drug - an antibiotic, typically cotrimoxazole),
  • steroid drugs - prednisone (a dose of 1 mg / kg per day), as well as methylprednisolone, these drugs are used orally, but also intravenously.
  • azathioprine, methotrexate - immunosuppressive drugs, used primarily in the maintenance treatment of polyarteritis nodosa after induction of remission.

There are no clear guidelines for the treatment of polyarteritis nodosa, in less severe cases, steroid treatment alone is sufficient, and when the disease is more aggressive, cyclophosphamide is added to the treatment, typically at a dose of 2 mg / kg daily or in several intravenous infusions - monthly at a dose of 1000 mg. As medical reports show, 2-3 times administration of cyclophosphamide controls the symptoms of the disease.

If polyarteritis nodosa is associated with hepatitis B infection, treatment is initially high-dose steroid medication followed by antiviral medication.

If complications of polyarteritis nodosa, such as hypertension or renal failure, occur, the conditions should also be treated. It is worth knowing that in this case, commonly used drugs for hypertension are not recommended - more precisely ACEI, i.e. angiotensin-converting enzyme inhibitors. Typically an illness confined to the skin requires the use of steroids.

Nodular arteritis - prognosis

Unfortunately, without adequate therapy, polyarteritis nodosa is fatal very quickly, usually within 1-2 years. That is why proper diagnostics and prompt initiation of appropriate treatment are so important. The limited cutaneous form, without involvement of other internal organs, has a good prognosis, and symptoms sometimes resolve spontaneously without medical intervention and treatment.

In summary, polyarteritis nodosa is a complex systemic disease that is often severe. If we have symptoms that may suggest this conditionrheumatology, it is definitely worth going for a medical consultation, describe your symptoms in detail and perform the tests recommended by the doctor.

Category:

Cardiology