Systemic vasculitis unites a group of diseases in which there is inflammation and destruction of vascular walls, leading to ischemia of organs and tissues. Disease is represented by Takayasu arteritis, Wegener’s granulomatosis, nodular periarteritis, Behcet syndrome, obliterating thrombangiitis, giant cell arteritis, etc. Pathology is aggravated by damage to the skin, kidneys, heart, lungs, joints, organs of vision, etc. The diagnosis is confirmed by laboratory tests, biopsy results and visceral angiography. Therapy with glucocorticoids, cytostatics, vascular drugs can reduce the damage to vital organs, achieve clinical remission.
Meaning
Diseases represented by primary systemic vasculitis develop independently and are characterized by nonspecific inflammation of the vascular walls. The occurrence of primary systemic vasculitis is associated with a violation of immune reactivity caused, as a rule, by an infectious agent. Inflammation in systemic vasculitis affects all layers of the vascular wall: it can be destructive, cause vessel occlusion, microcirculatory disorders and, as a consequence, organ ischemia up to necrosis and heart attacks.
Secondary vasculitis is an element of another pathology and is considered as its local facultative manifestation or complication (for example, vasculitis in scarlet fever, meningitis, typhus, sepsis, psoriasis, tumors, etc.). More often, men of the middle age group suffer from systemic vasculitis.
Classification
There are systemic vasculitis with vascular lesions of small, medium and large caliber. The group of vascular lesions of small vessels is represented by Wegener’s granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, essential cryoglobulinemic vasculitis, Schoenlein-Genoch purpura. Systemic vasculitis involving medium-sized vessels includes Kawasaki disease and nodular periarteritis (polyarteritis). Systemic vasculitis of large vessels includes Takayasu disease and giant cell temporal arteritis.
Symptoms
The clinic of systemic vasculitis includes various general nonspecific symptoms: fever, loss of appetite, asthenization, weight loss. The skin syndrome in systemic vasculitis is characterized by hemorrhagic rash, ulceration, necrosis of the skin. Musculoskeletal lesions are manifested by myalgia, arthralgia, arthritis. Changes from the peripheral nervous system in systemic vasculitis occur in the form of polyneuropathy or multiple mononeuropathy. The clinic of visceral lesions can be manifested by strokes, myocardial infarction, eye, kidney, lung damage, etc.
With nonspecific aortoarteritis (Takayasu’s disease), the aortic arch with branches extending from it is involved in granulomatous inflammation. The disease is manifested by generalized pain, microcirculatory disorders in the upper extremities, involvement of mesentery and lung vessels, angina pectoris, heart failure. Giant cell temporal arteritis (Horton’s disease), which is part of the group of this disease, occurs with inflammation of the carotid, temporal, and rarely vertebral arteries. The clinic of arterial lesions is manifested by headaches, hyperesthesia and swelling of the skin of the temporal region, ocular symptoms, neurological disorders.
Kawasaki disease, occurring with mucosal-skin-glandular syndrome, affects children. The clinic includes fever, conjunctivitis, diffuse spot-papular rash, erythema, swelling, peeling of the skin. With this form of this disease, lymphadenopathy, damage to the tongue, mucous lips are noted. With the involvement of the coronary arteries, aneurysms and heart attacks may develop.
Nodular panarteritis (polyarteritis, periarteritis) is characterized by necrotizing changes in the arteries of medium and small caliber and signs of polysystemic lesions. Against the background of general malaise, renal syndrome develops (glomerulonephritis, malignant hypertension, renal failure), arthralgia, polyneuritis, pneumonitis, coronaritis, micro-strokes.
Schenlein-Genoch disease refers to systemic vasculitis affecting small vessels as a result of pharyngitis of streptococcal etiology. It usually occurs in children; it occurs with small-spotted hemorrhages, polyarthralgia and polyarthritis, abdominal syndrome, immunocomplex glomerulonephritis.
Allergic angiitis or Churge-Strauss syndrome is associated with eosinophilic infiltration of vascular walls; it occurs with the clinic of bronchial asthma and eosinophilic pneumonia, polyneuropathy. It usually develops in people with an allergic history, as well as those who have visited tropical countries. With systemic granulomatous vasculitis of Wegener, the vessels of the airway and kidneys are affected. The disease is manifested by the development of sinusitis, ulcerative-necrotic rhinitis, cough, difficulty breathing, hemoptysis, symptoms of nephritis.
Hyperergic systemic vasculitis syndrome (cutaneous or leukocytoclastic vasculitis) occurs with immunocomplex inflammation of capillaries, arterioles and venules. Typical skin manifestations (purpura, blisters, ulceration) and polyarthritis. For microscopic polyangiitis, the development of necrotizing arteritis, glomerulonephritis and capillaritis of the pulmonary bed is specific.
Systemic vasculitis, occurring in the form of essential cryoglobulinemia, is caused by inflammation and occlusion of blood vessels caused by exposure to cryoglobulin complexes. The response symptom complex develops after treatment with penicillins, sulfonamides, etc. medications; manifested by purpura, urticaria, arthritis, arthralgia, lymphadenopathy, glomerulonephritis. It is prone to regression of manifestations after the exclusion of the etiofactor.
Diagnostics
During the diagnosis of systemic vasculitis, a comprehensive examination of the patient is carried out with the advice of a rheumatologist, neurologist, nephrologist and other specialists. ECG, echocardiography, kidney ultrasound, lung x-ray, etc. are performed. The growth of ESR is a characteristic, but non-specific sign of this pathology. In venous blood, At to neutrophil cytoplasm (ANCA) and CEC are determined.
Angiographic examination reveals vasculitis of vessels of small and medium diameter. The greatest diagnostic significance in systemic vasculitis is the biopsy of the affected tissues and their subsequent morphological study.
Treatment
The main stages of systemic vasculitis therapy consist of suppressing the hyperimmune response in order to induce clinical and laboratory remission; conducting an immunosuppressive course; maintaining stable remission, correcting organ disorders and rehabilitation. The pharmacotherapy is based on an anti-inflammatory and immunosuppressive course of corticosteroid hormones (prednisone, dexamethasone, triamcinolone, betamethasone). Pulse therapy with methylprednisolone is effective.
Cystostatic drugs for systemic vasculitis are used in cases of generalization and progression of the process, malignant renal hypertension, central nervous system lesions, ineffectiveness of corticosteroid therapy. The use of cytostatics (cyclophosphane, methotrexate, azathioprine) makes it possible to suppress the immune mechanisms of inflammation. Biological drugs that inactivate TNF (etanersept, infliximab); NSAIDs (ibuprofen, naproxen, diclofenac) effectively and quickly eliminate inflammation.
Therapy with anticoagulants and antiplatelet agents (heparin, dipyridamole, pentoxifylline) is indicated for signs of hypercoagulation and DIC syndrome. Violations of peripheral microcirculation are corrected by the appointment of nicotinic acid and its derivatives. Angioprotectors, vasodilators, calcium channel blockers are used in the treatment of systemic vasculitis.
In addition to medication courses for systemic vasculitis, extracorporeal hemocorrection sessions (cryoaferesis and cascade plasma filtration) are shown to remove circulating antibodies and immune complexes from the blood. Therapy of complicated systemic vasculitis requires coordinated cooperation of a rheumatologist, nephrologist, pulmonologist, otolaryngologist, neurologist, surgeon, oculist, etc.
Prognosis and prevention
The prospect of health and working capacity in systemic vasculitis is determined by the form of pathology, the age of the patient, and the effectiveness of treatment. Complex and step-by-step treatment of disease reduces the number of fatal and disabling outcomes.
Prevention of systemic vasculitis consists in the exclusion of contacts with infectious agents and allergens, refusal of unjustified vaccinations and prescribing medications.