VASCULITIS

DERMATOLOGY LECTURE NOTES

DEBABRATA BANDYOPADHYAY
PROFESSOR & HEAD, DEPT. OF DERMATOLOGY,
R. G. KAR MEDICAL COLLEGE, CALCUTTA, INDIA

	The vasculitides are a heterogeneous group of clinicopathological entities that share the common feature of vascular inflammation and injury. There is no universally acceptable classification of these group of disorders. While a number of underlying causes can be identified in some disorders, the aetiology is unknown in many. The pathogenetic mechanisms involved are mainly immunological, immune complex mediated tissue injury being the most commonly incriminated factor.
	ETIOLOGY & PATHOGENESIS
	While some forms of vasculitides may be ascribed to underlying factors like infections, malignancy, drug reactions or connective tissue disorders, the cause may remain undetermined in many vasculitic syndromes. Immunologic damage by immune-complex deposition or cell-mediated hypersensitivity is responsible in the majority of cases. The possible immunopathologic mechanism in the causation of vasculitis are: 1. Deposition of circulating antigen-antibody complex or in -situ formation of immune complex within the vessel wall. This leads to complement activation and chemotactic attraction of neutrophils by complement components. Subsequent phagocytosis of such complexes with liberation of neutrophil granular products leads to vascular damage. 2. Cell-mediated hypersensitivity: Antigenic exposure may attract lymphocytes which liberate cytokines causing tissue damage and further activation of macrophages and lymphocytes. 3. Failure to clear the antigen may lead to persistent inflammation and eventual formation of epithelioid cells and giant cells, giving rise to a granulomatous tissue reaction. Whatever the underlying mechanism, vascular inflammation and necrosis ensues which is often accompanied by thrombosis. These pathologic changes result in tissue ischaemia, necrosis and infarction, leading to a variety of clinical manifestations depending on the anatomic structures involved. PATHOLOGY: Perivascular cellular infiltration is a common histological finding in many disease entities, but for a definitive diagnosis of vasculitis, the presence of vascular damage, particularly in the form of fibrinoid degeneration, is necessary. Vasculitis may involve blood vessels of varying calibers and this feature forms the basis of a useful pathological classification of vasculitis. An infiltrate, composed of a variety of cell types, like neutrophils, lymphocytes, and histiocytes may invade the vessel wall and the surrounding tissue. Extravasation of red cells is a prominent feature in many vasculitides. Granulomatous inflammation with giant cell formation is a characteristic finding in some types.
	CLASSIFICATION
	Vasculitis is a taxonomist’s nightmare. Diseases with diverse causes and pathology may share the same symptomatology. On the other hand, a disease may show different histopathologic features at different periods in its evolution. Many diseases have overlapping features and it is impossible to formulate a classification scheme that unifies clinicopathological, etiological and immunological features of different diseases. Immunopathologic classification: I. Immune- complex mediated vasculitis: Polyarteritis nodosa Microscopic polyangiitis Hypersensitivity vasculitis (Leukocytoclastic vasculitis) Henoch-Schonlein purpura II. Vasculitis due to cellular hypersensitivity (Granulomatous vasculitis) Giant cell arteritis Takayasu’s arteritis Churg-Strauss disease (allergic granulomatosis) Wegener’s granulomatosis Isolated CNS vasculitis Classification based on caliber of blood vessel involved: I. Large vessel vasculitis: Giant cell arteritis Takayasu’s arteritis II. Medium vessel vasculitis: Polyarteritis nodosa Kawasaki disease III. Small vessel vasculitis: Microscopic polyangiitis Leukocytoclastic vasculitis Wegener’s granulomatosis Churg-Strauss disease Classification based on cellular composition of the infiltrate: I. Leukocytoclastic vasculitis (LCV): Neutrophils are predominant. Cellular fragments and nuclear debris (leukocytoclasia) are found in the infiltrate. Polyarteritis nodosa Henoch-Schonlein purpura Vasculitis due to drugs, infections, and connective tissue diseases Erythema elevatum diutinum Granuloma faciale etc. II. Lymphocytic vasculitis: Lupus erythematosus Lymphoma Pityriasis lichenoides III. Eosinophilic vasculitis : Churg-Strauss vasculitis IV. Granulomatous vasculitis : Wegener’s granulomatosis and Churg-Strauss V. Giant cell arteritis: Temporal arteritis Takayasu’s arteritis Clinical Classification: I. Systemic necrotizing vasculitis : Polyarteritis nodosa · Classical · Microscopic polyangiitis Allergic granulomatosis Polyangiitis overlap Wegener’s granulomatosis II. Giant cell vasculitis Temporal arteritis Takayasu’s disease III. Predominantly Cutaneous small vessel vasculitis : A. Idiopathic B. Secondary: Infection: ( Streptococcus, Staph, TB, Leprosy, Hep.B and C, HIV, Subacute bacterial endocarditis, EBV, parvovirus, Rickettsia) Drugs :Penicillin, sulpha, phenytoin, allopurinol, gold, thiazide, NSAIDs, Frusemide, quinidine, thiouracils, mefloquine Connective tissue disease (SLE, Sjogren’s syndrome, RA, Scleroderma, Dermatomyositis ) Malignancy ( Lymphoma, leukemia, solid organ tumors ) Other diseases: Cryoglobulinemia, complement deficiency, alpha1 antitrypsin deficiency Inflammatory bowel disease, Chronic active hepatitis, intestinal bypass surgery, primary biliary cirrhosis ,Relapsing polychondritis C. Clinical syndromes with leukocytoclastic vasculitis: Henoch-Schonlein purpura Urticarial vasculitis Serum sickness Erythema elevatum diutinum Granuloma faciale Hyperimmunoglobulinemia D Acute hemorrhagic edema of children Familial Mediterranean fever. IV. Other vasculitic syndromes: Behcet’s disease Buerger’s disease Kawasaki’s disease Isolated CNS vasculitis Cogan’s syndrome
	CLINICAL FEATURES
	Features in an individual patient will depend on the specific vasculitic syndrome and the degree and extent of organ systems involved. Constitutional: Fever, malaise, headache, weight loss etc Cutaneous: Palpable purpura Persistent urticaria Subcutaneous nodules Ulcers Necrotic papule Infarcts Hemorrhagic pustules Musculoskeletal: Arthritis Arthralgia Myalgia Renal Proteinuria Hematuria Acute and chronic renal failure Respiratory: Nasal obstruction, discharge, bleeding Cough, Hemoptysis Dyspnea GI : Dysphagia Pain Vomiting Bleeding CVS: Hypertension Thrombosis Ischemia Eyes: Uveitis Retinal vasculitis Nervous system: Peripheral neuropathy Stroke For salient features of major vasculitic syndromes : CLICK HERE
	LAB TESTS
	1. Screening Tests: CBC ESR CRP ASO titre Throat swab culture Stool for OBT Urinalysis Chest skiagram ECG Renal screen LFT 2. Focused Tests: ANA C3, C4, CH50 Rh factor Cryoglobulins APLA CK ANCA screen Serum protein electrophoresis Mantoux Test HepB/C serology Tests to detect other infections EMG-NCV Echocardiography Sinus X-ray, CT scan Angiography 3. Biopsy of appropriate tissue: Skin Temproral artery Kidney Testes etc
	CLINICAL APPROACH
	I. Suspect diagnosis: The following features should raise the suspicion of an underlying vasculitic process: Unexplained multisystem disease, PUO CVA, particularly in young age mononeuritis multiplex Persistent sinus symptoms Glomerulonephritis Pneumonitis GI bleeding Eye symptoms Arthritis/arthralgia Cutaneous signs: Palpable purpura Persistent urticaria Subcutaneous nodules Hemorrhagic pustules Livedo reticularis Infarcts Digital gangrene II. History, Clinical exam and Lab investigations to detect general features of inflammation and tissue ischemia and to delineate organ systems involved. III. Syndrome recognition: recognize vasculitic syndromes based on clinical findings and lab features and the patterns of organ system involved. III. Confirmation of diagnosis: By biopsy and angiography IV. Exclusion of underlying cause like infections, connective tissue disorder or malignancy
	MANAGEMENT
	Treatment will depend on the specific syndromes, the degree and severity of organ system involvement and the underlying cause if any. 1. Remove underlying cause: Withdraw drug, treat infection. 2. In mild cases no treatment or symptomatic measures like simple analgesics and rest will suffice. 3. Predominantly cutaneous vasculitis may respond to Dapsone, colchicine, hydroxycloroquine. 4. Systemic vasculitides or severe cutaneous vasculitis will require systemic steroids with or without immunosuppressive agents like cyclophosphamide, azathioprine or methotrexate. Cyclosporine may also be used in certain cases. 5. Symptomatic / specific measures for organ system failure.
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