Polyarteritis Nodosa is an inflammation of the veins involving small and medium-sized arteries. It is a disease whose cause is not known exactly and is seen with a frequency of one in a hundred thousand.
The disease manifests itself with fever of unknown origin. The clinical picture is variable. Fatigue, weight loss, loss of appetite can be seen in addition to fever. It involves the skin, joints, kidneys, peripheral nerves and gastrointestinal system, and signs and symptoms of these systems may be encountered.
Although the disease can be seen at any age, it is most common between the ages of 40-60.
Types of polyarteritis nodosa:
- PAN associated with hepatitis B,
- PAN associated with FMF,
- Classic PAN,
- Cutaneous PAN (no internal organ involvement),
- PAN with genetic anomalies (joint pains, paralysis, etc.).
Causes of polyarteritis nodosa:
The causes of polyarteritis nodosa are not fully known. It is an autoimmune disease that develops as a result of the immune system perceiving and attacking the body’s own vascular structures as foreign. However, it is not known why it attacks some vascular structures and not others.
Some types of the disease are associated with hepatitis B and FMF (Familial Mediterranean Fever).
Symptoms of polyarteritis nodosa:
Symptoms usually differ according to the involved organ. The most common signs and symptoms in patients are:
- Weight loss,
- Muscle pains,
- Joint pains,
- Skin rashes,
- Stomach ache,
- Numbness in hands and attacks,
- Tingle,
- Bloody stool,
- Vision problems,
- Respiratory problems,
- Testicular pain,
- Blood pressure,
- There may be bleeding in the urine.
Diagnosis of polyarteritis nodosa:
There is no specific diagnostic test for the disease. The disease can be suspected by anamnesis and physical examination findings, but it usually does not give classical findings. Tests that may be required to diagnose the disease:
- Exclusion of other possible diagnoses: Diseases such as granulomatous polyangiitis, eosinophilic granulomatous polyangiitis and microscopic polyangiitis should be excluded.
- Biopsy: Skin lesions and nerve biopsy may be required.
- Genetic analysis: Genetic analysis can be done especially for the hereditary species.
- Blood tests (signs of inflammation),
- Angiography, MR Angiography: Vascular inflammation can be shown.
- ANCA test: It is a cluster of autoantibodies seen in autoimmune diseases where the immune system attacks its own body cells. ANCA test is negative in polyarteritis nodosa.
ACR Classification Criteria (at least 3 criteria are required for diagnosis):
- Weight loss of more than 4 kg without diet,
- Pain, sensitivity in the testicles,
- Muscle pain, fatigue,
- Nerve damage (polyneuropathy, mononeuropathy),
- Diastolic blood pressure above 90 mmHg
- Fluid loss findings, BUN above 40 mg/dL, creatinine above 1.5 mg/dL,
- Positive hepatitis B tests
- Presence of atherosclerosis, aneurysm, etc. in the arteries in angiography,
- The appearance of inflammatory cells in the artery wall.
Treatment of polyarteritis nodosa:
The goal of treatment is to suppress the immune system to control inflammation. For this purpose, different treatments can be applied. High-dose corticosteroids, anti-TNF therapy, and rarely plasmapheresis are used most often. The course of treatment can take at least a year. If there is a concomitant viral disease (hepatitis B, C etc.), treatment may be required.
Suppression of the immune system may be the cause of susceptibility to infections. Patients should be vaccinated against pneumonia and flu, and receive supportive treatments. In addition, it should be followed regularly and supported in terms of possible side effects (osteoporosis, weight gain, high blood sugar, etc.) due to long-term use of corticosteroids.