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On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.85 no.3 Rio de Janeiro June 2010
Study about the association between anticardiolipin antibodies and peripheral vascular phenomena in patients suffering from systemic scleroderma*
Ana Paula Torres LiberatiI; Camila Ferrari RibeiroII; Thelma SkareIII
IIntern, General Practice Clinic Service,
Evangelical University Hospital of Curitiba (HUEC) - Curitiba (PR), Brazil
IIIntern, General Practice Clinic Service, Evangelical University Hospital of Curitiba (HUEC) - Curitiba (PR), Brazil
IIIHead of the Rheumatological Service of the Evangelical University Hospital of Curitiba (HUEC), Rheumatology Professor at the Medical School of the Evangelical Faculty of Paraná (FEPAR) - Curitiba (PR), Brazil
Ischemia is common in systemic scleroderma and it is caused by vasospasm and thrombosis. In the present study we analyzed the association of peripheral vascular events and anticardiolipin (aCl) antibodies in 54 patients suffering from systemic scleroderma. The results showed that 100% of the patients presented Raynaud; 59.2% presented digital micro scars; 43.3%, presented teleangiectasies and 14.8%, presented peripheral thromboembolism. ACl IgG were positive in 9.2% and IgM, in 7.4%. Peripheral tromboembolic phenomena had a positive association with aCl IgG (p=0.03). No other associations were found.
Keywords: Scleroderma, diffuse; Skin ulcer; Scleroderma, limited
Systemic scleroderma (ES) is an autoimmune disease of unknown etiology. Endothelial disfunction plays a fundamental etiopathogenetic role1 in it. It is characterized by an increase in the synthesis and deposition of collagen fibers on the skin and internal organs causing structural and functional damage to small arteries and capillaries apart from perivascular infiltration by inflammatory mononuclear cells.2 The excess of Endothelin-1(ET-1) is associated with the fisiopathologic mechanism of esclerodermic. The ET- 1, a potent endogenous vasoconstrictor, is mitogen for fibroblasts, smooth muscle cells and endothelial cells.3
Various auto-antibodies have been found in ES, among them, the antiphospholipids (AAF). Marie and cols2, studying 69 patients suffering from ES, found positiviness for these auto-antibodies in 19% of them. Assous and cols,4 in 108 pacients, found anticardiolipin antibodies (aCl) in 14% of the cases.
The AAF are responsible for trombotic phenomena of macro and microcirculation5 that can superpose the vascular damage of scleroderma, aggravating ischemic lesions.6 In some studies the presence of aCLs was associated with the appearing of digital micro scars.2,3
The present analysis was carried out to verify the prevailance of aCLs in the local population with ES and such auto-antibodies are associated with peripheral vascular disorders such as ulcers and digital necrosis, digital micro scars, teleangiectasies and peripheral thrombosis.
After being approved by the Ethics in Research Committee and the consent agreements were signed it was carried out a transversal study with 54 patients with ES (5 men and 49 women) with an average age of 51,5Â±11,7 (maximum of 79 and minimum of 26 years of age ) and an average length of time of the disease of 12,3Â±8,9 years (maximum of 40 years; minimum of 2 years). All them fulfiled the criteria of the American College of. Rheumatology for such disease.7 This sample was formed by patients with ES that agreed in taking part in the study and that had attended the service in the last 12 months of the research. All them presented the systemic form of the disease being divided according to the cutaneous expressions of the disease in 11 (20,3%) with the diffuse form, one (1,8%) with the sine scleroderma form and 33 (61,1%) with the limited or CREST form. Nine (16,6%) presented the overjet form with other collagenosis. It was sought the presence of digital ulcers, digital necrosis, micro digital scars, Raynaud, phenomenon, teleangiectasies, peripheral trombosis, of aCl IgG and IgM. The clinical data indexed are part of a list rated regularly in patients of the service with ES. From an immunological point of view, 50/54 (92,5%) patients were FAN positive, 17/51 (33,3%) presented anticentromere and 6/50 (12%) had Scl-70 positive. Around 10/54 (18,5%) were using cyclophosphamide and 5/54 (9,2%), methotrexate. For control, 150 patients with osteoarthritis and without any other comorbidities were includedin the study. Neither of the patients or controls were taking drugs that would imply positiviness for aCLs such as propranolol, chlorpromazine, γ interferon, amoxacillin, procainamide, anti TNF-α and cocaine in the moment of the dosage.8 ACls were surveyed by the ELISA technic (Euroimmun®, Lübeck, Germany) being considered positive values above 15 UI/ml. Data was collected in frequency and contingency charts and the Fisher test was calculated for associations with the support of the Graph Pad Prism software, version 4. The adopted significance was 5%.
It was found Raynaud in 100% of the cases, (n=54); digital micro scars in 59,2% (n=32); teleangiectasies in 44,4% (n=24); digital pulp ulcers in 18,5% (n=10), peripheral tromboembolic phenomena in 14,8% (n=8); digital necrosis in 7,4% (n=4), other digital ulcers in 3,7% (n=2), other sites ulcers in 1,8% (n=1).
The aCls in patients with ES were positive in 5 (9,25%) for IgG and in 4 (7,4%) for IgM. Among the control patients only 2 (1,3%) were positive for IgG. Comparing the prevalence of aCls among patients and controls it was found p=0,05 for aCls IgG and p=0,01 for IgM (Fisher).
The study of the association between aCls and peripheral vascular manifestations in ES can be found in Table 1.
Results show that the aCls are not associated with vascular phenomena in the extremities of patients with ES, except for cases of thrombosis.
1. Mayes M. Systemic sclerosis: clinical features. In: Klipel J, Stone J, Crofford L, White P, eds. Primer on rheumatic diseases. New York: Springer; 2008. p.343-58. [ Links ]
2. Marie I, Jouen F, Hellot M, Levesque H. Anticardiolipin and anti-beta-2 glycoprotein 1 antibodies and lupus like anticoagulant: prevalence and significance in systemic sclerosis. Br J Dermatol. 2008;158:141-4. [ Links ]
3. Xu S, Denton C, Holmes A, Dashwood M, Abraham D, Black C. Endothelins: effect on matrix biosynthesis and proliferation in normal and scleroderma fibroblasts. J Cardiovasc Pharmacol. 1998;31Suppl 1:S360-3. [ Links ]
4. Assous N, Allanore Y, Batteux F, Meune C, Toulon P, Weill B, et al. Prevalence of antiphospholipid antibodies in systemic sclerosis and association with primitive pulmonary arterial hypertension and endothelial injury. Clin Exp Rheumatol. 2005;23:199-204. [ Links ]
5. Santamaria JR, Badziak D, de Barros MF, Manderlli FL, Cavalin LC, Sato MS. Síndrome antifosfolipide. An Bras Dermatol 2005;80:225-39. [ Links ]
6. Petri M. Clinical and management aspects of antiphospholipid antibody syndrome. In: Wallace D, Hahn B, eds. Dubois' Lupus erythematosus. Philadelphia: Willians & Wilkins; 2007. p.1262-97. [ Links ]
7. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Arthritis Rheum. 1980;23:281-90. [ Links ]
8. Goldberg SN, Conti-Kelly AM, Greco TP. A family study of anticardiolipin antibodies and associated clinical conditions. Am J Med. 1995;98:473-9. [ Links ]
Mailing Address: Received on 17.12.2008. * Study carried
out in the Rheumatological Service of the Evangelical University Hospital of
Curitiba (HUEC) - Curitiba (PR), Brazil
Rua João Alencar Guimarães, 796
80310 420 Curitiba - PR
Approved by the Advisory Board and accepted for publication on 27.11.2009.
Conflict of interest: None
Financial funding: None
Received on 17.12.2008.
* Study carried out in the Rheumatological Service of the Evangelical University Hospital of Curitiba (HUEC) - Curitiba (PR), Brazil