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Case History of Systemic Vasculitis

The characteristic clinical appearance depends on the involved vascular segment (table 1).
General symptoms equally occur in PSV and SSV and are initially not characteristic simulating infectious or neoplastic diseases. These include a gB-symptomatologyh with fever, night sweats and weight loss. Additional symptoms such as arthralgy, arthritis or myalgy may suggest a connective tissue disease (see criteria of the American Rheumatism Association for systemic lupus erythematosus (SLE) or rheumatoid arthritis (RA): ARA crtiteria). Leading anamnestic symptoms suggesting PSV are inflamed (gredh) eyes and upper respiratory disorders (episcleritis and sinusitis in Wegener's disease), temporal head ache (in Horton's disease, arteritis temporalis), as well as abdominal pain and sensibility disorders (in panarteritis nodosa and in Schoenlein-Henoch's purpura: abdominal pain only).

Table 1: Vascular Involvement and Clinical Appearance

Vessel Clinical Findings w Granuloma w/o Granuloma
Small episcleritis, vertigo, hemoptyses,
Melaena, microhematuria,
Poly (mono-)neuritis, palpable purpura
Peri-myocarditis 		Wegener's disease microscopic poly-
angiitis
Medium infarction: brain, myocardium, kidneys,
Intestines, extremities;
Bleeding: ruptured microaneurysms 		Churg-Strauss syndr. class. Panarteritis
nodosa
Large stenoses, e.g. subclavian-steal syndr.
Aortic arch syndr., phlebothromboses 		Giant cell arteritis
Takayasu's arteritis

In the diagnosis of secondary systemic vasculitis (SSV) a history of sun allergies (in SLE), Raynaud syndrome, hair loss or dryness of mucous membranes (SLE, Sjoegren's syndrome) can be helpful.
Certain dermtological findings such as livedo, purpura or ulcerations are suggestive of PSV or SSV (table 2).

Table 2: Dermatologic Findings in Systemic Vasculitis

Purpura petechiae ekchymoses
Erythematous maculae papules nodules
Urticaria livedo reticularis necroses
Ulceration vesicles pustules
Bullae pyoderma gangrenosum erythema nodosum

Figure 1: Livedo vasculitis
Figure 2: Erythema nodosum in panarteritis nodosa
Figure 3: Necrotizing vasculitis in Wegener's disease
Figure 4: Leukocytoclastic vasculitis (see also fig. 57)
Figure 5: Schoenlein-Henoch's disease
Figure 6 & Figure 7: Cryoglobulinemic vasculitis
Figure 8: Relapsing perichondritis accompanying secondary vasculitis
Figure 9: Raynaud's syndrome

Any associated specific organ pathology must lead to excluding a malignant tumor.
Radiologic studies may assist in identifying additional organ involvement and prompt histological proof by biopsy. Angiographic demonstration of microaneurysms in kidney, liver or mesentery suggest panarteritis nodosa.

Clinical Presentation According to Involved Vascular Segment (table 1)

Essentially every vascular segment can be afflicted by vasculitis. Involvement of large arteries can cause stenosis and poststenotic hypoxemia (e.g. aortic arch syndrome in Takayasu's arteritis). Arteritis of medium size vessels with focal lesions usually cause aneurysms, with more circumferential lesions cause occlusion and infarction as characteristically seen in panarteritis nodosa . Small vascular disease including "capillaritis" is frequently associated with alveolar hemorrhage and /or glomerulitis (pulmorenal angiitis, glomerulonephritis). Affection of capillaries and venules usually cause purpura (vasculitis allergica).