Polyarteritis nodosa

Polyarteritis nodosa (polyarteritis, clasic polyarteritis nodosa, PAN) is an inflamatory disease of small and medium vessels of the skin, kidneys, peripheral nerves, cardiovascular and gastroinstestinal systems. It affects the entire wall of the vessel and carries a lot of complications (aneurysm, thrombus, infarction and even manifestations of multiorgan failure).

Epidemiology and etiology
It is a rare disease, more often affecting men around 50 years of age. The etiology is not yet clear, immunocomplexes and some viruses are being considered (HBV, CMV, HIV, parvoviry).

Clinical symptoms
The disease is accompanied by general signs of inflammation and varying severity of organ system damage.
 * General signs of inflammation: high temperatures to fever, fatigue, weight loss, muscle and joint pain.
 * Skin manifestations: erythema, painful subcutaneous nodules, petechiae, palpable purpura, livedo vascularis, ulcus, skin necrosis (if only the skin is affected, we speak of polyarteiitis nodosa cutanea).
 * Symptoms of peripheral nerve damage: pain paraesthesia, paresis (peripheral neuropathy with manifestations of mononeuritis multiplex).
 * Symptoms of kidney damage: hypertension, renal insufficiency, proteinuria, erythrocyturia.
 * Symptoms of GIT damage: abdominal pain, gastrointestinal bleeding, pancreatitis, cholecystopathy, etc.

Diagnosis

 * Anamnesis.
 * Physical exam.
 * Laboratory examination methods: ↑ FW + CRP, eosinophilia, 1/3 of patients HbsAg+, c-ANCA+.
 * Angiography: occlusions and aneurysms
 * Biopsy of muscles, liver, kidneys, skin and subsequent histopathological examination.

Note: The American College of Rheumatology has developed classification criteria for polyartiitis nodosa.

Treatment
Corticosteroids are mainly used in the treatment, in severe cases they are combined with cyclophosphamide (pulses of methylprednisolone + cyclophosphamide at a dose of 10–15 mg / kg at weekly to monthly intervals).

Související články

 * Systémové vaskulitidy