Generic placeholder image

Current Rheumatology Reviews

Editor-in-Chief

ISSN (Print): 1573-3971
ISSN (Online): 1875-6360

Research Article

“Scleroderma” and “Scleroderma-like” Capillaroscopic Pattern-Differences and Similarities

Author(s): Sevdalina Nikolova Lambova* and Ulf Müller-Ladner

Volume 20, Issue 3, 2024

Published on: 02 January, 2024

Page: [304 - 316] Pages: 13

DOI: 10.2174/0115733971265291231212045931

Price: $65

Abstract

Introduction: The “scleroderma” type capillaroscopic pattern is a reference pattern in rheumatology that is a diagnostic sign for systemic sclerosis (SSc) in an appropriate clinical context and is observed in more than 90% of scleroderma patients. Similar microvascular changes, the so-called “scleroderma-like”, have been described albeit in a lower proportion of patients with other rheumatic diseases, such as dermatomyositis (DM), undifferentiated connective tissue diseases (UCTD), systemic lupus erythematosus (SLE), etc. Three distinct stages of “scleroderma” pattern have been suggested by Cutolo et al., i.e., “early”, “active”, and “late”. However, disease duration is just one of the factors that contributes to the progression of microvascular changes, and in this regard, “active” or even “late” pattern could be observed in patients with shorter disease duration. In addition, stable microvascular changes could be found for long periods in other cases.

Objective: The aim of the study was to assess the presence of differentiating features between “scleroderma” pattern in SSc and “scleroderma-like” pattern in other rheumatic diseases.

Methods: 684 capillaroscopic images demonstrating a “scleroderma” and “scleroderma-like” pattern have been analysed in the current retrospective cross-sectional study. 479 capillaroscopic pictures were obtained from 50 SSc patients, 105 from 7 DM patients, 38 from 10 rheumatoid arthritis (RA) patients, 36 images from 5 patients with SLE, and 26 images from 9 patients with UCTD. All capillaroscopic images used in the current analysis have fulfilled the criteria for “sclerderma/scleroderma-like” pattern, as the pathological changes in the capillaroscopic parameters have also been confirmed by quantitative measurement of capillary diameters, capillary density, and intercapillary distance. All the images have been categorized into one of the following groups, i.e., “early”, “active” and “late” phases (according to the definition of Cutolo et al.), or “other” findings, the latter being specifically described as they could not be attributed to one of the other three categories.

Results: 479 capillaroscopic pictures were obtained from 50 scleroderma patients. 31 of them showed an “early”, 391 an “active” phase, and 57 a “late” phase “scleroderma” type microangiopathy. In 69 images assessed as an “active” pattern, neoangiogenesis was found. In 43 out of 105 capillaroscopic pictures from DM patients, an “active” phase was detected; in 2 of the images, a “late” pattern was found, and in 60 capillaroscopic pictures, neoangiogenesis in combination with giant capillary loops was observed. Early microangiopathy was not found in this group. Among capillaroscopic images from SLE patients, “late” phase microangiopathy was not found. “Early” phase was present in 3 images, “active” phase in 29, neoangiogenesis in “active” phase in 4 pictures. Early microangiopathy was detected in 11 capillaroscopic pictures from RA patients (8 out of 9 patients), an “active” phase in 4 images (3 patients), and in 23 capillaroscopic images, neoangiogenesis with mild capillary derangement and capillary loss and single giant capillaries (“rheumatoid neoangiogenic pattern”) were observed. Classic “late” type microangiopathy was not found in RA patients as well as among patients with UCTD. The predominant capillaroscopic pattern in UCTD patients was early microangiopathy (n = 23). The rest images from UCTD exhibited features of the “active” phase.

Conclusion: In conclusion, early microangiopathy was observed in RA, SLE, and UCTD patients, but not in patients with DM. An “active” phase “scleroderma” type capillaroscopic pattern was detected in all patient groups other than SSc, i.e., DM, SLE, RA, and UCTD. “Late” phase “scleroderma” type microangiopathy was present in patients with scleroderma and DM and was not observed in SLE, RA, and UCTD. Despite the fact that in some cases, microangiopathy in scleroderma and other rheumatic diseases may be indistinguishable, the results of the current research have shown the presence of some differentiating features between “scleroderma” and ”scleroderma-like” microangiopathy that might be a morphological phenomenon associated with differences in the pathogenesis and the degree of microvascular pathology in various rheumatic diseases.

Keywords: Capillaroscopy, “scleroderma-like”, systemic sclerosis, microvascular damage, connective tissue diseases, UCTD.

Graphical Abstract
[1]
Brown G, O’Leary PA. Skin capillaries in scleroderma. Arch Intern Med 1925; 36(1): 73-88.
[http://dx.doi.org/10.1001/archinte.1925.00120130076008]
[2]
van den Hoogen F, Khanna D, Fransen J, et al. 2013 classification criteria for systemic sclerosis: An American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis 2013; 72(11): 1747-55.
[http://dx.doi.org/10.1136/annrheumdis-2013-204424 ] [PMID: 24092682]
[3]
Maricq HR, LeRoy EC, D’Angelo WA, et al. Diagnostic potential of in vivo capillary microscopy in scleroderma and related disorders. Arthritis Rheum 1980; 23(2): 183-9.
[http://dx.doi.org/10.1002/art.1780230208] [PMID: 7362667]
[4]
Maricq HR, Harper FE, Khan MM, Tan EM, LeRoy EC. Microvascular abnormalities as possible predictors of disease subsets in Raynaud phenomenon and early connective tissue disease. Clin Exp Rheumatol 1983; 1(3): 195-205.
[PMID: 6335855]
[5]
Bergman R, Sharony L, Schapira D, Nahir MA, Balbir-Gurman A. The handheld dermatoscope as a nail-fold capillaroscopic instrument. Arch Dermatol 2003; 139(8): 1027-30.
[http://dx.doi.org/10.1001/archderm.139.8.1027] [PMID: 12925391]
[6]
Lambova SN. The role of capillaroscopy in rheumatology 2011.
[7]
Lambova S, Hermann W, Müller-Ladner U. Capillaroscopic pattern at the toes of systemic sclerosis patients: Does it “tell” more than those of fingers? J Clin Rheumatol 2011; 17(6): 311-4.
[http://dx.doi.org/10.1097/RHU.0b013e31822be4e8 ] [PMID: 21869710]
[8]
Lambova S, Müller-Ladner U. Capillaroscopic findings in systemic sclerosis-are they associated with disease duration and presence of digital ulcers? Discov Med 2011; 12(66): 413-8.
[PMID: 22127112]
[9]
Kenik JG, Maricq HR, Bole GG. Blind evaluation of the diagnostic specificity of nailfold capillary microscopy in the connective tissue diseases. Arthritis Rheum 1981; 24(7): 885-91.
[http://dx.doi.org/10.1002/art.1780240704] [PMID: 7259800]
[10]
Cutolo M, Sulli A, Pizzorni C, Accardo S. Nailfold videocapillaroscopy assessment of microvascular damage in systemic sclerosis. J Rheumatol 2000; 27(1): 155-60.
[PMID: 10648032]
[11]
Manfredi A, Sebastiani M, Cassone G, et al. Nailfold capillaroscopic changes in dermatomyositis and polymyositis. Clin Rheumatol 2015 Feb; 34(2): 279-84.
[http://dx.doi.org/10.1007/s10067-014-2795-8]
[12]
Bertolazzi C, Cutolo M, Smith V, Gutierrez M. State of the art on nailfold capillaroscopy in dermatomyositis and polymyositis. Semin Arthritis Rheum 2017; 47(3): 432-44.
[http://dx.doi.org/10.1016/j.semarthrit.2017.06.001] [PMID: 28668440]
[13]
Nagy Z, Czirják L. Nailfold digital capillaroscopy in 447 patients with connective tissue disease and Raynaud’s disease. J Eur Acad Dermatol Venereol 2004; 18(1): 62-8.
[http://dx.doi.org/10.1111/j.1468-3083.2004.00853.x ] [PMID: 14678534]
[14]
Kabasakal Y, Elvins DM, Ring EF, McHugh NJ. Quantitative nailfold capillaroscopy findings in a population with connective tissue disease and in normal healthy controls. Ann Rheum Dis 1996; 55(8): 507-12.
[http://dx.doi.org/10.1136/ard.55.8.507] [PMID: 8774177]
[15]
Furtado RN, Pucinelli ML, Cristo VV, Andrade LE, Sato EI. Scleroderma-like nailfold capillaroscopic abnormalities are associated with anti-U1-RNP antibodies and Raynaud’s phenomenon in SLE patients. Lupus 2002; 11(1): 35-41.
[http://dx.doi.org/10.1191/0961203302lu144oa] [PMID: 11899953]
[16]
Lambova SN, Müller-Ladner U. Capillaroscopic pattern in systemic lupus erythematosus and undifferentiated connective tissue disease: What we still have to learn? Rheumatol Int 2013; 33(3): 689-95.
[http://dx.doi.org/10.1007/s00296-012-2434-0] [PMID: 22527142]
[17]
Lambova SN, Muller-Ladner U. Nailfold capillaroscopy within and beyond the scope of connective tissue diseases. Curr Rheumatol Rev 2018; 14(1): 12-21.
[http://dx.doi.org/10.2174/1573397113666170615093600 ] [PMID: 28641551]
[18]
Lambova SN, Müller-Ladner U. Capillaroscopic pattern in inflammatory arthritis. Microvasc Res 2012; 83(3): 318-22.
[http://dx.doi.org/10.1016/j.mvr.2012.03.002] [PMID: 22426123]
[19]
Rajaei A, Dehghan P, Amiri A. Nailfold capillaroscopy in 430 patients with rheumatoid arthritis. Caspian J Intern Med 2017; 8(4): 269-74.
[PMID: 29201317]
[20]
Lambova SN, Müller-Ladner U. Capillaroscopic features of microangiopathy in rheumatoid arthritis patients with peripheral vascular syndrome. Clin Rheumatol 2019; 38(9): 2339-41.
[http://dx.doi.org/10.1007/s10067-019-04561-x] [PMID: 31016582]
[21]
van Roon AM, Huisman CC, van Roon AM, et al. Abnormal nailfold capillaroscopy is common in patients with connective tissue disease and associated with abnormal pulmonary function tests. J Rheumatol 2019; 46(9): 1109-16.
[http://dx.doi.org/10.3899/jrheum.180615] [PMID: 30554151]
[22]
Lambova SN. Scleroderma-like pattern in various rheumatic diseases. J Rheumatol 2020; 47(6): 942.1-.
[http://dx.doi.org/10.3899/jrheum.200020] [PMID: 32295850]
[23]
Lambova S. Capillaroscopic findings in systemic lupus erythematosus with cutaneous digital lesions. Lupus 2021; 30(10): 1696-7.
[http://dx.doi.org/10.1177/09612033211027935] [PMID: 34192955]
[24]
Piotto DGP, Len CA, Hilário MOE, Terreri MTRA. Nailfold capillaroscopy in children and adolescents with rheumatic diseases. Rev Bras Reumatol 2012; 52(5): 722-32.
[PMID: 23090372]
[25]
Redisch W, Messina EJ, Hughes G, McEwen C. Capillaroscopic observations in rheumatic diseases. Ann Rheum Dis 1970; 29(3): 244-53.
[http://dx.doi.org/10.1136/ard.29.3.244] [PMID: 5432591]
[26]
De Angelis R, Cerioni A, Del Medico P, Blasetti P. Raynaud’s phenomenon in undifferentiated connective tissue disease (UCTD). Clin Rheumatol 2005; 24(2): 145-51.
[http://dx.doi.org/10.1007/s10067-004-0988-2] [PMID: 15351873]
[27]
Cutolo M, Paolino S, Smith V. Nailfold capillaroscopy in rheumatology: Ready for the daily use but with care in terminology. Clin Rheumatol 2019; 38(9): 2293-7.
[http://dx.doi.org/10.1007/s10067-019-04716-w] [PMID: 31396833]
[28]
Boulon C, Devos S, Mangin M, et al. Original article Reproducibility of capillaroscopic classifications of systemic sclerosis: Results from the SCLEROCAP study. Rheumatology 2017; 56(10): 1713-20.
[29]
Smith V, Vanhaecke A, Herrick AL, et al. Fast track algorithm: How to differentiate a “scleroderma pattern” from a “non-scleroderma pattern”. Autoimmun Rev 2019; 18(11): 102394.
[http://dx.doi.org/10.1016/j.autrev.2019.102394] [PMID: 31520797]
[30]
Masi AT, Rodnan GP, Medsger TA Jr, et al. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 1980; 23(5): 581-90.
[http://dx.doi.org/10.1002/art.1780230510] [PMID: 7378088]
[31]
Bohan A, Peter JB. Polymyositis and dermatomyositis. N Engl J Med 1975; 292(7): 344-7.
[http://dx.doi.org/10.1056/NEJM197502132920706 ] [PMID: 1090839]
[32]
Arnett FC, Edworthy SM, Bloch DA, et al. The american rheumatism association 1987 revised criteria for the classification of rheumatoid arthritis. Arthritis Rheum 1988; 31(3): 315-24.
[http://dx.doi.org/10.1002/art.1780310302] [PMID: 3358796]
[33]
Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1982; 25(11): 1271-7.
[http://dx.doi.org/10.1002/art.1780251101] [PMID: 7138600]
[34]
Mosca M, Neri R, Bombardieri S. Undifferentiated connective tissue diseases (UCTD): A review of the literature and a proposal for preliminary classification criteria. Clin Exp Rheumatol 1999; 17(5): 615-20.
[PMID: 10544849]
[35]
Schmidt JA, Caspary L, von Bierbrauer A, et al. Standardization of nailfold capillary microscopy in routine diagnostics. Vasa 1997; 26(1): 5-10.
[http://dx.doi.org/10.1024/0301-1526.35.1.5] [PMID: 9163237]
[36]
Lambova SN, Müller-Ladner U. Inhomogeneity of capillaroscopic findings in systemic sclerosis. Int J Rheum Dis 2020; 23(2): 207-15.
[http://dx.doi.org/10.1111/1756-185X.13760] [PMID: 31808306]
[37]
Lambova S, Müller -Ladner U. Nailfold capillaroscopy of fingers and toes - variations of normal. Curr Rheumatol Rev 2017; 13: 28-35.
[38]
Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet 2003; 362(9388): 971-82.
[http://dx.doi.org/10.1016/S0140-6736(03)14368-1] [PMID: 14511932]

Rights & Permissions Print Cite
© 2024 Bentham Science Publishers | Privacy Policy