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The contribution of capillaroscopy to the differential diagnosis of connective autoimmune diseases

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Raynaud's phenomenon (RP) is one of the earliest clinical hallmarks of microvascular involvement in several connective autoimmune rheumatic diseases. The direct observation of the microvasculature with nailfold videocapillaroscopy (NVC) is useful for an early diagnosis of connective autoimmune diseases (secondary RP) and differentiation from primary (unsymptomatic) RP. Generally, to detect early pathologic capillaroscopic changes, the following parameters are considered: presence of enlarged and giant capillaries, haemorrhages, disorganization of the vascular array, ramified/bushy capillaries and loss of capillaries. Careful capillaroscopic analysis of subjects affected by primary RP can detect the earliest signs of the transition to secondary RP and thus screening procedures for further differential diagnosis within connective autoimmune diseases can be undertaken. In systemic sclerosis, the recognition of clear and different NVC morphological patterns (“early”, “active”, “late”) should suggest including this analysis in the classification criteria of the disease.

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Raynaud's phenomenon

Raynaud's phenomenon (RP) (Figure 1) is the clinical hallmark of microvascular involvement in several connective autoimmune rheumatic diseases, and is particularly important in systemic sclerosis (Ssc).1 The occurrence of RP should lead to a prompt microvascular analysis through a capillaroscopic examination to obtain an early differential diagnosis between primary (uncomplicated) and secondary (disease-associated) RP.

According to population-based surveys of various ethnic groups, the

Dermatomyositis

A defined pattern has been reported in patients affected by dermatomyositis.31 This pattern, often associated with aspects of the scleroderma pattern, includes the presence of two or more of the following findings in at least two nailfolds: enlargement of capillary loops, loss of capillaries, disorganization of the normal distribution of capillaries, ‘budding’ (‘bushy’) capillaries and capillary haemorrhages (Figure 10).32

Systemic lupus erythematosus

The SLE pattern generally includes morphological alterations of capillary loops, venular visibility and sludging of blood with variability of capillary loop length (Figure 11).33, 34 A recent study evaluated the association between the nailfold capillary abnormalities frequently observed in patients with SLE and the presence of RP, anti-U1RNP and anticardiolipin (aCL) antibodies.35 One hundred SLE patients were studied. Wide-field nailfold capillaroscopy was considered abnormal according to

Antiphospholipid syndrome

Interesting microvascular alterations have been observed in patients affected by antiphospholipid syndrome (APS). One study reported symmetrical microhaemorrhages at the nailfold analysis; these were found to be particularly significant in patients with both serum IgG and IgM aCL (Figure 12).37 Marked microcirculatory damage was found to be related to the occurrence of thrombotic manifestations in patients with APS in other studies, confirming the pattern.38

A more recent study confirmed that

Sjögren syndrome

Capillaroscopic changes have been observed in primary Sjögren syndrome (SS).41 Forty patients with SS (14 without RP, 16 with RP, 10 with aCL), 20 patients with Ssc (10 with limited and 10 with diffuse disease) (disease control group) and 40 healthy controls (control group) were evaluated by nailfold capillaroscopy. Capillaroscopic abnormalities in SS ranged from non-specific findings (crossed capillaries) to more specific findings (confluent haemorrhages and pericapillary haemorrhages) or

Other diseases

Patients with mixed cryoglobulinaemia show a variety of microcirculatory changes, often clustered in a characteristic pattern of abnormally oriented, short capillaries and neoangiogenetic phenomena. Capillary changes are more numerous in nephritic patients.43 Altered microvasculature has also been reported in psoriasis and psoriatic arthritis.44 A large study showed a significant decrease in capillary loop density in patients with either psoriasis plus nail disease (14.5 ± 5.7 capillaries per

Transition from primary to secondary Raynaud's phenomenon: the role of nailfold videocapillaroscopy

Nailfold videocapillaroscopy is a tool that allows us to distinguish between primary and secondary RP and that allows, through the recognition of the ‘early’ microvascular pattern, the early differential diagnosis at least of Ssc.48 The recognition of different NVC morphological patterns (“early”, “active”,“late”), and a significant and gradual increase of these latter microvascular abnormalities, are observed during the disease and seem to reflect the possible evolution of the pathophysiologic

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