**5.3 Small vessel vasculitis**

• Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis

Microcirculation abnormalities of granulomatosis with polyangiitis, formerly Wegener's granulomatosis, were described in one study involving 12 patients [45]. The main NFC changes detected were avascular areas; crossed and bushy capillaries; and microhemorrhages. No relationship was established with disease activity or its clinical aspects.

No valuable information was found about NFC in microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis-former Churg-Strauss vasculitis.

• Immune complex small vessel vasculitis

NFC changes in cryoglobulinemic vasculitis were disclosed in one study with 29 patients, of which 28 had hepatitis C infection [41]. Microcirculation abnormalities detected were tortuosity, altered orientation, shortened capillaries, and neoangiogenesis (**Figure 14**). No relation was found with disease activity, but glomerulonephritis was associated with a higher score of NFC alterations.

IgA vasculitis (IAV), formerly called Henoch-Schönlein purpura, rarely affects adults and studies about NFC changes have been performed in small samples of patients in pediatric age [46–48]. The NFC changes in IAV are conflicting, including density reduction, increased capillary length, loop dilatation, persistent edema,

#### **Figure 13.**

*Polyarteritis nodosa. This patient presents with capillary slow flow sludge (dashed arrow), reduced capillary density, but also enlarged capillaries (black dot) (4th finger of the left hand). Images were taken using Videocap biomicroscope, version 3.0, magnification ×200. Courtesy of Nailfold Capillaroscopy Clinic of Hospital Curry Cabral.*

#### **Figure 14.**

*Cryoglobulinemic vasculitis. The image reveals tortuosity (arrow), bifurcation with altered orientation (triangle), and neoangiogenesis (star) (2nd finger of the right hand). Images were taken using Videocap biomicroscope, version 3.0, magnification ×200. Courtesy of Nailfold Capillaroscopy Clinic of Hospital Curry Cabral.*

#### **Figure 15.**

*Tortuosity (arrow) in a patient with Behçet disease (4th finger of the left hand). Images were taken using Videocap biomicroscope, version 3.0, magnification ×200. Courtesy of Nailfold Capillaroscopy Clinic of Hospital Curry Cabral.*

tortuosity, and branching capillaries. No statistically significant correlation was found between NFC abnormalities and organ involvement, but Zampetti et al. described normalization of the edema after 6-month follow-up.

There was no available data about NFC in anti-glomerular basement membrane disease and hypocomplementemic urticarial vasculitis-former anti-C1q vasculitis.

#### **5.4 Variable vessel vasculitis**

Behçet disease (BD) relationship with NFC alterations have been described in some studies, but they all used different technical characteristics for visualization of microcirculation [42, 49–51]. The main NFC abnormalities include enlarged capillaries, microhemorrhages, reduced density, and tortuosity (**Figure 15**). None of them were related with disease activity. Still, some NFC alterations were described as being related with clinical aspects of BD: NFC severity corresponded to longer disease duration and positive pathergy test; enlarged capillaries were associated with younger age at disease onset, high blood pressure, and superficial phlebitis.

We did not find any information about capillaroscopy in Cogan syndrome.

#### **5.5 Vasculitis associated with systemic disease**

In rheumatoid vasculitis, some studies correlated with capillary damage and levels of soluble intracellular adhesion molecule-1 (sICAM-1), which is highly expressed during inflammation [52–54]. They found abnormalities in the great majority of

**183**

**Figure 16.**

*Capillaroscopy Clinic of Hospital Curry Cabral.*

*The Impact of Nailfold Capillaroscopy in the Approach of Microcirculation*

**6. Nailfold capillaroscopy in systemic disorders with vascular** 

patients, mainly morphologic changes, and although there was no direct relation with disease activity, severe NFC alterations were associated with disease duration, cutaneous vasculitis, joint erosions, systemic vasculitis, and sICAM-1 levels. In a recent study involving 62 patients, scleroderma-like NFC changes were found in 20% of patients with rheumatoid vasculitis and they were interpreted as varying degree of microvascular inhomogeneity, not being necessarily related to overlap syndromes [55, 56]. No specific information was found about NFC in lupus vasculitis or sarcoid

Since APS does not derive from connective tissue, we consider more appropriated to approach it in a separated part of this chapter. Capillaroscopy has been studied in APS and attempts were made to include it as a diagnostic tool [57]. NFC findings include microhemorrhages and dilated loops. Long loops and slow flow sludge capillaries are suggestive of a primary APS, while hemorrhages are typical of secondary APS [19] (**Figure 16**). A specific pattern of microhemorrhage, symmetrically disposed, has been called the "comb-like" hemorrhage and is highly associated to APS [1]. Further, positivity for anticardiolipin antibody has been related to higher prevalence of hemorrhages [58]. In spite of this, and even if microhemorrhages significantly correlate with the diagnosis of APS and its clinical manifestations, NFC findings are not sufficient to establish APS diagnosis for its lack of sensitivity and specificity.

In DM, NFC changes are apparently associated to the level of glycemic control and the existence of chronic microvascular complications [9]. However, there is an elevated prevalence of comorbidities concurring for microangiopathy, especially in type 2 diabetes, including arterial hypertension, dyslipidemia, and obesity. Still, a "diabetic capillaropathy" was described, which includes tortuosity, capillaries with bizarre shapes, loop dilations, and avascular areas [9, 59]. No differences were found between type 1 and 2 DM, but microvascular complications detected with NFC were correlated with diabetic peripheral neuropathy [60]. It has also been demonstrated that even in prediabetic patients, microangiopathy can already be

*Antiphospholipid syndrome in three different patients, revealing tortuosity (arrow), ingurgitated venous plexus (plus sign), long capillaries (two-way arrow), slow flow sludge (dashed arrow), and "comb-like" hemorrhages (\*\*\*). Images were taken using Videocap biomicroscope, version 3.0, magnification ×200. Courtesy of Nailfold* 

*DOI: http://dx.doi.org/10.5772/intechopen.90525*

**6.1 Antiphospholipid syndrome (APS)**

vasculitis.

**involvement**

**6.2 Diabetes mellitus (DM)**

*The Impact of Nailfold Capillaroscopy in the Approach of Microcirculation DOI: http://dx.doi.org/10.5772/intechopen.90525*

patients, mainly morphologic changes, and although there was no direct relation with disease activity, severe NFC alterations were associated with disease duration, cutaneous vasculitis, joint erosions, systemic vasculitis, and sICAM-1 levels. In a recent study involving 62 patients, scleroderma-like NFC changes were found in 20% of patients with rheumatoid vasculitis and they were interpreted as varying degree of microvascular inhomogeneity, not being necessarily related to overlap syndromes [55, 56].

No specific information was found about NFC in lupus vasculitis or sarcoid vasculitis.
