The Relationship Between Nailfold Capillaroscopic Assessment and Telangiectasia Score with Severity of Peripheral Vascular Involvement in Systemic Sclerosis Y
Total Page:16
File Type:pdf, Size:1020Kb
The relationship between nailfold capillaroscopic assessment and telangiectasia score with severity of peripheral vascular involvement in systemic sclerosis Y. Yalcinkaya, O. Pehlivan, A. Omma, N. Alpay, B. Erer, S. Kamali, L. Ocal, M. Inanc Division of Rheumatology, ABSTRACT Introduction Department of Internal Medicine, Objective. To determine the association Systemic sclerosis (SSc) is a connective Istanbul University, Istanbul, Turkey. of nailfold video-capillaroscopy (NVC) tissue disease characterised by autoim- Yasemin Yalcinkaya, MD findings and telangiectasia score with mune inflammation, vascular injury Ozlem Pehlivan, MD digital ulcer (DU) history and sever- and fibrosis (1). Vascular injury starts Ahmet Omma, MD ity of peripheral vascular involvement in the early phases of the disease and Nilufer Alpay, MD Burak Erer, MD (PVI) in systemic sclerosis (SSc). can cause digital ischaemia-gangrene Sevil Kamali, MD Methods. Fifty-nine SSc patients fulfill- and pulmonary arterial hypertension Lale Ocal, MD ing Leroy & Medsger criteria were eval- (PAH) which were major factors of Murat Inanc, MD uated including telangiectasia score, high morbidity and mortality in SSc. Please address correspondence to: disease activity and severity scores. (2). Raynaud’s phenomenon (RP) is ac- Dr Yasemin Yalcinkaya, NVC was performed according to quali- cepted as the initial clinical symptom Division of Rheumatology, tative (early, active and late patterns) reflecting the microvascular injury be- Department of Internal Medicine, and semi-quantitative assessments. fore the onset of other manifestations Istanbul University, Results. When DU+ and DU- groups Millet Caddesi - Gapa, (3, 4). Fatih 34093, were compared; the mean score of Microvascular injury induces structural Istanbul, Turkey. capillary number (CN) was 2.0±0.5 changes of capillary array and can be E-mail: [email protected] vs. 1.4±0.7 (p<0.001), irregularly en- established by a non-invasive imag- Received on September 22, 2014; accepted larged capillaries (IEC) was 1.8±0.6 ing tool, nailfold video-capillaroscopy in revised form on January 12, 2015. vs. 1.4±0.7 (p<0.05), microangiopathy (NVC), from the early stages of the di- Clin Exp Rheumatol 2015; 33 (Suppl. 91): evolution score (MES) was 2.5±1.5 vs. asese. Therefore it has been accepted as S92-S97. 1.8±1.0 (p<0.05) and ‘early’ pattern a useful method for differentiation of © Copyright CLINICAL AND was significantly less frequent in DU+ primary RP from secondary, early diag- EXPERIMENTAL RHEUMATOLOGY 2015. patients (1 vs. 9, p=0.016). The fre- nosis of SSc and follow-up of the pro- quency of severe-PVI (Medsger sever- gression of microangiopathy in SSc (5- Key words: systemic sclerosis, ity score of 2–4) was 22% in females 8). NVC assessment has been shown to capillaroscopy, telangiectasia score, (12/54) and 80% in males (4/5). When be a reliable method for the prediction peripheral vascular involvement severe and non-severe groups were of development of digital ulcers (DUs) compared; the mean score of CN was and severity of the organ involvement 2.1±0.4 vs. 1.5±0.7 (p<0.001), MES (9-13). Improvement in NVC abnor- was 2.8±1.6 vs. 1.8±1.1 (p<0.05) and malities after 12 months of treatment ‘early’ pattern was significantly less with an endotelin receptor antagonist, frequent in patients with severe PVI (0 bosentan, supports that NVC may be vs. 9, p=0.049). The mean values of tel- also used for establishing the efficacy angiectasia score were similar between of treatment (14). groups. RP and chronic digital ischaemia lead- Conclusion. DU history and severe ing to DUs can be seen in all subsets PVI in SSc were associated with capil- of disease but more frequent in dcSSc. lary loss and microangiopathy. ‘Early’ DUs heal slowly especially when com- NVC pattern was very rare in patients plicated with infections or gangrene with DU history and was not found in (15, 16). Early stages of the SSc were severe PVI. Severe PVI in males was shown to have higher risk of DU de- more frequent than females. Telan- velopment in a French cohort; first DU giectasia scores were not found to be occured in 43% within 1 year following related to PVI. NVC may be a helpful the first non-Raynaud’s symptom and method in the assessment of SSc pa- 73% within 5 years (17). Digital lesions tients for PVI prognosis, warranting also have a tendency to recur. In the Competing interests: none declared. prospective studies. study by Nihtyanova et al., 1168 SSc S-92 NVC, telangiectases and digital lesions in SSc / Y. Yalcinkaya et al. patients analysed, 16.6% had at least if RP requiring vasodilators, 2 if digital disorganisation of the normal capillary one episode of DU and 12% hospital- pitting scars, 3 if DU, 4 if gangrene was array, ramified/bushy capillaries (9). ised at least once for vasodilatory treat- present] (23) were evaluated simultane- Secondly, NVC assessed semi-quanti- ments during 18-month period (18). ously with NVC. tatively and scoring of the capillary ab- Telangiectases are one of the most NVC was performed on all patients af- normalities (CN/IEC/GC/H/CR/CAD) visible clinical manifestations of mi- ter 20 minutes of resting time period in (score:0–3) was performed as follows: crovascular pathology composed of a separate room with a constant tem- 0 = no changes, 1 = less than 33% of vasodilated post-capillary venules in perature of 20–22°C by a video-capil- capillary alterations/reduction, 2 = 33– the papillary and superficial reticular laroscopy (Optilia, Mediscope-Digital 66% of capillary alterations /reduction, dermis of the skin without inflamma- Video Microscope). OptiPix (2010, 3 = more than 66% of capillary altera- tory changes (19). They may be found Version:1.2.0) programme was used tions/reduction, per linear mm at the on hands, face, mucosa, limbs and to store the images. The investigator distal row of the nailfold. Microangi- trunk of both of two subgroups of SSc (YY) who recruited patients into the opathy evolution score (MES) was the patients. In a study of Shah et al., a sig- study and evaluated medical records, sum of CN, CR and CAD scores. The nificant correlation between number of performed NVC studies. After a drop of mean score for each capillary abnor- telangiectases and pulmonary vascular cedar oil on the nailfold, at least 2 rep- mality was calculated from the average disease-PAH was found, but associa- resentative images for each of second to score of two images of 1 mm of each 8 tion with other vascular manifestations fifth fingers of left and right hands were digits (2nd, 3rd, 4th and 5th fingers of both like digital ulcers or gangrene could not captured with the 200x optical probe. hands), added together, and then the fi- be shown (20). All images (16 images for 8 fingers) nal value divided by eight to obtain the In this study we determine the associa- were stored and examined later by a score (10). tion of NVC abnormalities and telangi- second investigator (MI) under blinded The study was approved by local ethics ectasia score (TS) with DU history and conditions. Number of capillaries (CN), committee and informed consent was severity of peripheral vascular involve- irregularly enlarged capillaries (IEC), obtained from all participants. ment (PVI) in SSc. giant capillaries (GC), microhaemor- For statistical analysis of the data ‘SPSS rhages (H), capillary ramifications version 16 for Windows’ programme Patients and methods (CR), capillary array disorganisation was used. Groups were compared with A total of 59 SSc patients with non-RP (CAD) and maximum capillary diam- Pearson chi-square/Fisher’s tests for symptom of <8 years (preferably <2 eter were calculated per 1 millimeter categorical variables or student’s t- and years) and fulfilling Leroy & Medsger (mm) area of the images. Mann Witney U-tests for continuous classification criteria (21) were included Firstly, NVC assessed qualitatively. variables. Results were presented as into the study. Patients with age of <18, Sclerodermic NVC pattern (early or mean ± standard deviation (±SD). P- non-RP symptom of >8 years and not active or late pattern) was investigated values less than 0.05 were considered eligible for performing NVC were ex- and decided as follows: early NVC pat- significant. The distribution and median cluded. Demographics, onset of symp- tern, few giant capillaries (homogene- levels of NVC abnormalities and TS in toms, organ involvement, treatment ously enlarged loop with a diameter patient groups according to DU history details, laboratory and imaging findings >50 μm), few capillary haemorrhages and severity of vascular involvement including serology, echocardiography, (dark mass due to haemosiderin depos- are summarised in boxplot graphics. respiratory function tests, high-resolu- it), regular capillary distribution and no tion computed tomography (HRCT) of significant loss of capillaries (at least Results chest were noted into a pre-defined pro- 9–10 capillaries per linear mm counted Demographics and clinical characteris- tocol. TS (For each body area including at the distal row of the nailfold); active tics of SSc patients were summarised face, hands, arms, chest and abdomen, NVC pattern, frequent giant capillar- in Table I. back, legs and feet; telangiectases were ies, frequent capillary haemorrhages, The mean age of the patients was 45.6 scored as 0 if no telangiectasia was pre- moderate loss of capillaries, mild dis- and 91.5% were females. The mean sent, 1 if there were fewer than 10 tel- organisation of the capillary architec- duration of RP, non-RP symptoms angiectases, 2 if there were 10 or more ture (irregular capillary distribution (NRP) and skin involvement were telangiectases, the total possible telan- and orientation with respect to the 6.1±6.5, 3.1±2.0 and 3.0±2.0 years, giectasia score was 22 (20), modified nailfold along with shape heterogene- respectively.