Vasculitis

Epidemiology/Aetiology

• Vasculitis of varying pathogenesis can be an important cause of occlusive disease of the upper extremity [29].

• Digital ischaemia should initiate a search for the underlying pathological condition that precipitated the process of vasculitis.

• Arteritis due to systemic infections (typhus, paratyphus, diphtheria, influenza, pneumonia), allergy (also after drugs [40], penicillin), cannabis arteritis [13], or in association with neoplastic disease are quite rare.

DigitalArteritis

• The principle symptom is Raynaud's phenomenon.

• In the presence of cryoglobulinemia it should be established whether tumour (multiple myeloma, leukaemia, and bronchial or breast carcinoma), collagen disease, liver disease (cirrhosis), or systemic infection (sepsis, endocarditis, hepatitis, malaria, pneumonia, among others) is the underlying aetiology.

• Treatment of digital arteritis is dietetic, hygienic and medical.

Fig. 3.1.11 Implantation of a cervical spinal cord stimulator. Immediate pain relief with excellent long-term result (8 years). To treat the dermatomes C6-C8, the lead has to be placed between C3 and C5

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Fig. 3.1.12 Same patient as Figs. 3.1.8 and 3.1.9, 4 months after stopping smoking, 2 weeks of intravenous prostaglandin analogues and cervical spinal cord stimulation

HypersensitivityVasculitis

• Antigens causing hypersensitivity vasculitis can be drugs, bacteria (^-haemolytic streptococcus), tumour antigen or serum protein; allergic (or hypersensitivity)

vasculitis affects small vessels, especially postcapillary venules, and has a good prognosis under medical treatment.

• Henoch-Schönlein purpura.

• Malignancies.

ConnectiveTissue Disorders

Antigen-antibody complex deposition in digital vessels is a common factor in this group of heterogeneous conditions. These changes (fibrinoid degeneration, intimal thickening) produce the clinical features of digital isch-aemia.

• Systemic lupus erythematosus (SLE) - vasculitis accounts for the Raynaud's phenomenon [41] of SLE, which occurs in one-fifth of SLE patients. Digital ischaemia may indeed be the presenting symptom of SLE and may predate arthritic symptoms. Arteriolar endothelial destruction, with eosinophilic infiltration of the intima of the common digital arteries, narrows the lumen.

• Scleroderma - Patients with CREST syndrome (calcinosis cutis, Raynaud's phenomenon, oesophageal dysmotility, scleroderma and telangiectasia) develop vasculitis and digital ischaemia, quite frequently predating systemic disease by many years. Arterial involvement occurs in the digital arteries. There is in-timal thickening secondary to fibrin deposition in the vessel wall, resulting in narrowed arterial lumen and digital ischaemia [27].

• Polyarteritis nodosa - Polyarteritis nodosa affects small- and medium-sized vessels with segmental involvement [50].

• Rheumatoid arthritis - Seropositive rheumatoid arthritis gives rise to endoarteritis obliterans in the digital arteries. Postcapillary venules in the skin are also affected. Generally, severely affected hands and digits are subject to vasculitis. A careful search for vasculitis must be made before surgical reconstruction of these deformed hands.

FibromuscularDysplasia

• The pathology of fibromuscular dysplasia (see Chapter 2.4, Fibromuscular Dysplasia, by M. Sechas) involves the media of the arteries of small and medium calibre.

• In the upper extremity [9, 11, 34], these lesions are extremely rare and found more commonly in women who are generally younger than those seen because of atheromatous disease.

• Fibrous dysplasia and atherosclerosis may coexist in the same artery.

• In its most common form the media develops annular thickenings separated by segments of normal or thinned artery.

• The result is an artery that is elongated and has the appearance of a string of beads, with alternating stenoses and dilatations.

• Segmental narrowings may occur and create turbulence and a critical stenosis.

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