Microvascular damage in autoimmune connective tissue diseases: a capillaroscopic analysis from 20 years of experience in a EULAR training and research referral centre for imaging

Abstract

ObjectiveNailfold videocapillaroscopy (NVC) allows the detection of microvascular damage in autoimmune connective tissue diseases (CTDs). The prevalence of the morphological capillary findings was retrospectively evaluated in a wide cohort of patients with Raynaud’s phenomenon secondary to a CTD at the time of the first single NVC, independently from their current treatment, autoantibody profile and comorbidities.MethodsOne-thousand-one-hundred-eighty-one patients affected by CTDs were included from 2001 to 2021. The considered CTDs were systemic sclerosis (SSc), undifferentiated connective tissue disease (UCTD), mixed connective tissue disease (MCTD), dermatomyositis (DM), systemic lupus erythematosus, Sjögren’s syndrome and primary antiphospholipid syndrome (aPS). The capillaroscopic parameters were distinguished between scleroderma patterns and non-scleroderma patterns.ResultsGiant capillaries were significantly more frequent in SSc, DM and MCTD than in other CTDs (respectively, in 73%, 73% and 61% of patients, p<0.001 when comparing each rate vs the other CTDs). The mean capillary count was significantly lower in SSc, DM and MCTD (respectively, 7.04±0.18 vs 6.5±0.75 vs 7.7±2 capillaries/linear mm) compared with the other CTDs (p<0.001 for each rate vs the other CTDs). The non-specific abnormalities of capillary morphology were significantly more frequent in SSc, MCTD and aPS (respectively, in 48%, 41% and 36% of cases, all p<0.001 vs each other CTDs).ConclusionThis large size sample of patients with CTDs, collected over 20 years of analysis, confirms the highest prevalence of specific capillaroscopic alterations in patients with SSc, DM and MCTD, when compared with other CTDs.

Microvascular status in juvenile Sjögren’s disease: the first nailfold videocapillaroscopy investigation

“…The results confirmed that no specific NVC pattern for SjD can be described as it has been done in adults SjD patients [27][28][29], since no NVC-specific abnormalities (i.e. giant capillaries) or features compatible with a scleroderma pattern or scleroderma-like pattern were detected [43].…”

Raynaud’s Phenomenon: A Current Update on Pathogenesis, Diagnostic Workup, and Treatment

“…The clinical history should delve into the patient’s sex, age at onset, patterns of digital involvement (including thumb sparing, symmetricity, and ulcerations), and frequency of RP attacks [ 2 , 42 ]. Additionally, it is crucial to identify potential secondary causes of RP, such as SSc, other autoimmune diseases, hematologic disorders, paraneoplastic conditions, and neurological or vaso-occlusive conditions (as discussed in the clinical manifestation section) [ 5 , 20 , 43 ]. Occupational risk factors like exposure to manual vibrating tools, chemicals (e.g., polyvinyl chloride), medications (e.g., β-blockers and chemotherapy), and smoking history should also be explored [ 23 , 29 , 44 ].…”