The Place of Nailfold Capillaroscopy Among Instrumental Methods for Assessment of Some Peripheral Ischaemic Syndromes in Rheumatology.

Micro- and macrovascular pathology is a frequent finding in a number of common rheumatic diseases. Secondary Raynaud's phenomenon (RP) is among the most common symptoms in systemic sclerosis and several other systemic autoimmune diseases including a broad differential diagnosis. It should be also differential from other peripheral vascular syndromes such as embolism, thrombosis, etc., some of which lead to clinical manifestation of the blue toe syndrome. The current review discusses the instrumental methods for vascular assessments. Nailfold capillaroscopy is the only method among the imaging techniques that can be used for morphological assessment of the nutritive capillaries in the nailfold area. Laser-Doppler flowmetry and laser-Doppler imaging are methods for functional assessment of microcirculation, while thermography and plethysmography reflect both blood flow in peripheral arteries and microcirculation. Doppler ultrasound and angiography visualize peripheral arteries. The choice of the appropriate instrumental method is guided by the clinical presentation. The main role of capillaroscopy is to provide differential diagnosis between primary and secondary RP. In rheumatology, capillaroscopic changes in systemic sclerosis have been recently defined as diagnostic. The appearance of abnormal capillaroscopic pattern inherits high positive predictive value for the development of a connective tissue disease that is higher than the predictive value of antinuclear antibodies. In cases of abrupt onset of peripheral ischaemia, clinical signs of critical ischaemia, unilateral or lower limb involvement, Doppler ultrasound and angiography are indicated. The most common causes for such clinical picture that may be referred to rheumatologic consultation are the antiphospholipid syndrome, mimickers of vasculitides such as atherosclerosis with cholesterol emboli, and neoplasms.

Capillaroscopic pattern in systemic sclerosis - an association with dynamics of processes of angio- and vasculogenesis.

Мicrocirculation is the main environment of the pathologic processes in systemic sclerosis (SSc). Nailfold capillaroscopic abnormalities in SSc are highly specific. It is well known that capillaroscopic examination reveals different changes in the different stages of SSc. Dilated, single giant capillaries and haemorrhages, preserved arrangement are the characteristic features of the "early" capillaroscopic phase; frequent giant capillaries and haemorrhages are the findings in the "active" phase, while in the "late" stages extensive avascular areas are usually found. Although tissue hypoxia normally is a strong inducer of neovascularization, there is no evidence of significant vascular recovery in SSc patients. Formation of new blood vessels is possible through two different mechanisms - angiogenesis (formation of new vessels from differentiated endothelial cells of prior vessels) - and vasculogenesis (from endothelial progenitor cells-EPCs). Disturbed function of EPCs has been found in all the stages of the disease. Only EPCs from SSc patients in the early phase of the disease have shown preserved ability for differentiation and maturation to endothelial cells in vitro. The recovery of the injured microvessels is also disturbed due to a predominance of angiogenic inhibitors. The capillaroscopic changes in the different stages of SSc mirror the dynamics in processes of angio- and vasculogenesis. The formation of new capillaries after therapeutic influence of vascular recovery by transplantation of autologous bone-marrow derived stem cells supports this conclusion.

Raynaud's phenomenon in common rheumatic diseases.

Raynaud's phenomenon (RP) is caused by a reversible spasm of the smallest arteries and the arterioles of the fingers and toes. Two forms of RP have been described: primary and secondary. Secondary RP is often present in patients with rheumatic diseases. This review presents the characteristics of the clinical pattern, the immunological profile and the capillaroscopic pattern in patients with primary and secondary RP in common rheumatic diseases. Attention is paid to standard examinations usually used in RP patients. Primary RP appears to be more common in women beginning usually at puberty. This disorder is caused by vasospasm; no abnormalities of the endothelium are observed. Primary RP shows benign progression. Laboratory tests - erythrocyte sedimentation rate (ESR) and antinuclear antibody test (ANA) - are normal. Capillaroscopy is normal too. Secondary RP tends to begin later in life. Of the rheumatic diseases, scleroderma is the one that is the most often associated with RP (in 90-95% of all cases). The pathogenesis of secondary RP in scleroderma is explained with abnormalities of the endothelium through different mechanisms. Motor ulcerations are frequently observed. Anticentromeric and antitopoizomeric antibodies get positive. Capillaroscopy appears to be very important for the early diagnosis and prognosis of scleroderma. The capillaroscopic pattern is abnormal - enlarged capillaries, hemorrhages and avascular areas are observed. Part of the patients with systemic lupus erythematosus, Sjogren's syndrome and polymyositis / dermatomyositis develops secondary RP and it usually shows benign progression. RP is the main symptom in mixed connective tissue disease and trophic abnormalities of the fingers are frequently observed. Elevated anti-U1-RNP antibody titers and an abnormal capillaroscopic pattern are specific for the condition. In older patients isolated RP may represent a paraneoplastic manifestation. RP is frequently found in the rheumatologic practice. The differentiation of the primary form from the secondary one is essential because of the differences concerning the heaviness of the disorder, the prognosis and the therapeutic approach.