Nodular periarteritis is a systemic vasculitis characterized by inflammatory and necrotic lesions of the walls of small and medium visceral and peripheral arteries. Symptoms begins with fever, myalgia, arthralgia, which are joined by thrombangiitic, cutaneous, neurological, abdominal, cardiac, pulmonary, renal syndromes. To confirm the diagnosis, a morphological examination of skin biopsies is performed. Corticosteroids, immunosuppressants, cytostatics are used in the treatment. Prognosis is largely determined by the severity of the lesion of internal organs.
Nodular periarteritis refers to pathologies with unclear etiology. The role of viral infection (including hepatitis B) in the development of this disease is considered; in this case, vaccination, administration of serums, medications, insolation or hypothermia may be triggering factors. In response to these factors, a hyperergic reaction develops with the formation of immune complexes that are fixed in the walls of blood vessels and cause autoimmune inflammation in them. These processes are accompanied by the release by the endothelium of damaged vessels of factors of increased clotting and thrombosis. Periarteritis mainly affects the male population from 30 to 50 years old.
There are classical (with renal-visceral or renal-polyneuritic symptoms), asthmatic, cutaneous-thrombangiitic and monoorgan variants of the clinical development of pathology. Benign development of nodular periarteritis is noted in the cutaneous form without visceropathy. Patients are preserved in somatic and social terms; remissions are persistent, but exacerbations of vasculitis are possible.
Slow progression is characteristic of the thrombangiitic variant. At the same time, arterial hypertension, peripheral neuritis, microcirculatory disorders in the extremities may be noted. Recurrent nodular periarteritis is provoked by the cancellation or reduction of the dosage of cytostatics, glucocorticoids, drug allergy, infection, cooling.
The rapid progression of nodular periarteritis is associated with kidney damage and a malignant form of arterial hypertension. In rare cases, the disease develops at lightning speed, leading to the death of the patient after 5-12 months. In the clinic of nodular periarteritis, active, inactive and sclerotic phases are distinguished.
With disease, there is a high wave-like fever, decreasing in response to taking glucocorticoids or aspirin, weight loss up to cachexia, adynamia, weakness. The skin is characterized by pallor, marbling, the appearance of a reticulated livedo, skin rashes (erythematous, spotty-papular, hemorrhagic, necrotic), subcutaneous nodules in the forearms, shins, thighs.
Musculoskeletal manifestations in nodular periarteritis include myalgia, weakness, soreness, muscle atrophy; polyarthralgia, migrating arthritis of large joints. Renal symptom complex in 70-97% of patients with nodular periarteritis occurs with vascular nephropathy: microhematuria, proteinuria, cylindruria, rapid development of renal failure. Possible outcomes are ruptured renal aneurysms, renal infarction.
Cardio-vascular insufficiency syndrome includes the development of coronaritis leading to angina and myocardial infarction, myocarditis, cardiosclerosis, conduction disorders, arrhythmias, mitral valve insufficiency. Arterial hypertension is a characteristic cardio-vascular manifestation of nodular periarteritis. When the lungs are affected, pulmonary vasculitis and interstitial pneumonia develop, manifested by coughing, shortness of breath, hemoptysis, thoracalgia, respiratory noises and wheezing, lung infarctions.
Gastrointestinal tract lesions occur with nausea, diarrhea, epigastric pain. With a complicated variant, the development of pancreatic necrosis, jaundice, perforated ulcers of the stomach and 12p. intestine, bleeding is possible. Involvement of the nervous system is manifested by asymmetric polyneuropathy: muscular atrophy, pain in the projection of nerve trunks, paresthesia, paresis, trophic disorders. In case of severe lesions, strokes, meningoencephalitis, epileptiform seizures are likely to occur.
Visual disturbances in nodular periarteritis are expressed by malignant retinopathy, aneurysmal dilation of the fundus vessels. Disorders of peripheral blood supply to the extremities cause ischemia and gangrene of the fingers. With lesions of the endocrine system, orchitis and epididymitis, dysfunction of the adrenal glands and thyroid gland are noted.
A variant of asthmatic nodular periarteritis occurs with persistent attacks of bronchial asthma, skin manifestations, fever, arthralgias and myalgias. The dominant manifestations of the cutaneous thrombangitic form of nodular periarteritis are nodules, livedo and hemorrhagic purpura. Subcutaneous nodules are characterized by their location along the vascular bundles of the extremities. This symptom develops against the background of myalgia, fever, sweating, weight loss. Nodular periarteritis, occurring in a monoorgan type, is characterized by visceropathy and is established after histological examination of a biopsy or a removed organ.
Complicated forms of nodular periarteritis may be accompanied by the development of heart attacks and organ sclerosis, rupture of aneurysms, perforation of ulcers, intestinal gangrene, uremia, stroke, encephalomyelitis.
In the general clinical analysis of urine, microhematuria, proteinuria and cylinduria are determined; in the blood – signs of neutrophilic leukocytosis, hyperthrombocytosis, anemia. Changes in the biochemical picture of the blood in nodular periarteritis are characterized by an increase in the fractions of γ- and α2-globulins, sialic acids, fibrin, seromucoid, PSA.
To clarify the diagnosis of nodular periarteritis, a biopsy is performed. Inflammatory infiltration and necrotic changes of vascular walls are detected in the musculoskeletal biopsy of the abdominal wall or lower leg. With nodular periarteritis, HBsAg or antibodies to it are often detected in the blood. An examination of the fundus reveals aneurysmal vascular changes. The ultrasound of the renal vessels determines their stenosis. During the overview radiography of the lungs, the strengthening of the pulmonary pattern and its deformation are traced. To diagnose cardiopathies, an ECG and ultrasound of the heart are performed.
The large diagnostic criteria for nodular periarteritis include the presence of kidney lesions, abdominal syndrome, coronaritis, polyneuritis, bronchial asthma with eosinophilia. Additional (small) criteria are myalgia, fever, weight loss. When diagnosing nodular periarteritis, three large and two small criteria are taken into account.
Therapy is characterized by continuity and duration (up to 2-3 years), complexity and individual selection of funds. Taking into account the form of the disease, it is carried out by the joint efforts of a rheumatologist, cardiologist, nephrologist, pulmonologist and other specialists. The course of early and uncomplicated forms of nodular periarteritis can be corrected by corticosteroid therapy with prednisone with repeated courses 2-3 times a year. In between corticosteroid courses, pyrazolone-type drugs (butadion) are prescribed or acetylsalicylic acid.
With nodular periarteritis complicated by malignant hypertension or nephrotic syndrome, cytostatic immunosuppressants (azathioprine, cyclophosphane) are prescribed. Correction of DIC syndrome and hyperthrombocytosis includes therapy with heparin, pentoxifylline, dipyridamole. Biopreparations that block TNF (infliximab, etanersept) can quickly reduce inflammation.
With chronic nodular periarteritis, occurring with muscular atrophy or neuritis, physical therapy, hydrotherapy, massage are performed. Extracorporeal hemocorrection techniques (plasmapheresis, hemosorption, cryoapheresis) reduce the severity of autoimmune reactions and blood viscosity due to the removal of CEC, autoantibodies, and excessive thrombosis factors from the bloodstream.
Prognosis and prevention
The course of nodular periarteritis is unfavorable in terms of prognosis. Severe vascular lesions (renal insufficiency syndrome, arterial hypertension, cerebral disorders, thrombosis, perforative complications, etc.) can lead to death. Remission and stopping the progression of nodular periarteritis is achieved in 50% of patients.
Preventive tasks include taking into account drug intolerance, reasonable and controlled immunization, blood transfusions, protection from infections.