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

Objective: Nailfold 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.

Methods: One-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.

Results: Giant 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).

Conclusion: This 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.

Keywords: Sjogren's syndrome; antiphospholipid syndrome; dermatomyositis; lupus erythematosus, systemic; scleroderma, systemic.

Figure 1

Microvascular morphological alterations detectable on NVC in autoimmune CTDs. (A) Giant capillaries in an SSc patient with an ‘active’ scleroderma pattern. (B) Scleroderma-like pattern in a patient with MCTD. (C) ‘Comb-like’ microhaemorrhages in a patient with primary aPS. aPS, antiphospholipid syndrome; CTDs, connective tissue diseases; MCTD, mixed connective tissue disease; NVC, nailfold videocapillaroscopy; SSc, systemic sclerosis.

Figure 2

Mean capillary density, rates of giant capillaries, abnormally shaped capillaries and microhaemorrhages in CTDs. aPS, antiphospholipid syndrome; CTDs, connective tissue diseases; DM, dermatomyositis; MCTD, mixed connective tissue disease; SLE, systemic lupus erythematosus; SS, Sjögren’s syndrome; SSc, systemic sclerosis; UCTD, undifferentiated connective tissue disease.