Abstract
Arterial occlusion with subsequent amputation of extremities is a rare manifestation of systemic lupus erythematosus (SLE). It may be caused by local arteritis and/or thrombosis. We describe the clinical and laboratory manifestations and treatment administered to six SLE patients who developed peripheral arterial necrosis necessitating amputation of extremities secondary to the arterial occlusion. All patients were female, with ages ranging from 16 to 65 years. Arterial occlusion took place in the initial months of disease (median: 7 months). Only one of five patients tested for antiphospholipid antibodies had these antibodies who also had vasculitis and thrombosis in a histopathological study. Most patients presented a very benign outcome after the amputation of extremities and stayed in remission for several years. The satisfactory outcome of most patients after the vascular phenomenon allows us to consider the possibility that such a complication could be, for unknown reasons, a marker for good prognosis in SLE or, alternatively, that the aggressive therapy administrated for patients with this complication at the beginning of the disease could recover the balance of the immune system, avoiding future relapses.
Similar content being viewed by others
References
Jindal BK, Martin MFR, Gayner A (1983) Gangrene developing after minor surgery in a patient with undiagnosed systemic lupus erythematosus and anticoagulant. Ann Rheumatol Dis 42:347–349
Kaufman JL, Bancilla E, Slade J (1986) Lupus vasculitis with tibial artery thrombosis and gangrene (letter). Arthritis Rheum 19:1291–1292
Klumb EM, Lederman R, Guimarães SJ, Goldfarb M, Assad JEM, Leite N (1993) Large vessel vasculitis and occlusion associated to antiphospholipid antibodies in systemic lupus erythematosus. Rev Bras Rheumatol 33:42–45
Shaffer B, James GW, Scully JP, Pillsbury DM (1951) Systemic lupus erythematosus. Some cutaneous manifestations related to the diffuse collagenous diseases. Am J Med Sci 221:314–318
Tan EM, Cohen AS, Fries JF et al (1982) The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 25:1271–1277
Wilson WA, Gharavi AE, Koike T et al (1999) International consensus statement on preliminary classification for definite antiphospholipid syndrome. Arthritis Rheum 42:1309–1311
Bernabo JG, Zoruba D, Metta S, Salomon E, Juarez MA (1986) Gangrene de pie y occlusion de los grandes vasos de la pierna secundarias a lupus eritematoso sistemico con vasculitis. Medicina (B Aires) 46:209–212
Dubois EL, Tuffanelli DL (1964) Clinical manifestations of systemic lupus erythematosus. Computer analysis of 520 cases. JAMA 190:104–111
Goldberger E, Elder RC, Schwartz RA, Phillips PE (1992) Vasculitis in the antiphospholipid syndrome. Arthritis Rheum 35:569–572
Amital H, Levy Y, Langevitz P, Rubinow A, Shoenfeld Y (2002) Leg amputation–possible therapeutic role in catastrophic anti-phospholipid syndrome (CAPS)? Lupus 11:554
Shetty VB, Rao S, Krishnamurthy PN, Shenoy VU (2001) Peripheral gangrene during infancy: a rare presentation of systemic lupus erythematosus. Arch Dis Child 85:335–336
Yang SG, Kim KH, Park KC, Youn SW, Joh GY (1996) A case of systemic lupus erythematosus showing acute gangrenous change of fingertips (letter). Br J Dermatol 134:185–187
Vocks E, Welcker M, Ring J (2000) Digital gangrene: a rare skin symptom in systemic lupus erythematosus. J Eur Acad Dermatol Venereol 14:419–421
Gladstone DE, Prestrud AA, Styler MJ et al (2002) High dose cyclophosphamide for severe systemic lupus erythematosus. Lupus 11:405–410
Mittal G, Balakrishna C, Mangat G, Joshi VR (1999) ‘Sustained remission’ in a case of SLE following megadose cyclophosphamide. Lupus 8:77–80
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Rocha, M.C.B.T.d., Vilar, M.J.P., Freire, E.A.M. et al. Arterial occlusion in systemic lupus erythematosus: a good prognostic sign?. Clin Rheumatol 24, 602–605 (2005). https://doi.org/10.1007/s10067-005-1111-z
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10067-005-1111-z