Case Report
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Sjögren’s syndrome and catastrophic antiphospholipid syndrome with severe skin involvement

Year 2020, Volume: 45 Issue: 2, 746 - 751, 30.06.2020
https://doi.org/10.17826/cumj.679449

Abstract

Catastrophic antiphospholipid syndrome is a rare but rapidly progressing form of antiphospholipid antibody syndrome with high mortality. The syndrome causes multiorgan failure associated with diffuse micro-thromboses. Necrotic-appearing ecchymotic lesions emerging from the distal aspect of both lower extremities and progressing to the upper leg region, and to the upper extremities developed in a 58-year-old woman. Histopathological examination of the biopsy specimen revealed intravascular microthrombi in the dermis. Laboratory findings for anti-cardiolipin antibodies IgM and lupus anticoagulant resulted positive. Lung and kidney involvement was observed. The clinical course progressed very quickly, and catastrophic antiphospholipid syndrome was diagnosed with these findings. Two-thirds of catastrophic antiphospholipid syndrome cases develop due to secondary causes, the most common being infections. Catastrophic antiphospholipid syndrome may also accompany autoimmune diseases, particularly systemic lupus erythematosus. This report is presented to emphasize that Sjögren's syndrome should be considered in the etiology of catastrophic antiphospholipid syndrome.

References

  • Referans1. Asherson RA, Cervera R, de Groot PG, et al. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus 2003;12(7):530–534. Referans2. Nayer A, Ortega LM. Catastrophic antiphospholipid syndrome: a clinical review. J Nephropathol 2014;3(1):9-17 Referans3. Asherson RA. The catastrophic antiphospholipid syndrome. J Rheumatol 1992;19:508–512. Referans4. Cervera R, Rodríguez-Pintó I, Espinosa G. The diagnosis and clinical management of the catastrophic antiphospholipid syndrome: A comprehensive review. J Autoimmun 2018;92:1-11. Referans5. Cervera R1, Bucciarelli S, Plasín MA, et al. Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the “CAPS Registry”. J Autoimmun 2009;32(3-4):240–245. Referans6. Kazzaz NM, McCune WJ, Knight JS. Treatment of catastrophic antiphospholipid syndrome. Curr Opin Rheumatol 2016;28(3):218–227. Referans7. Rodriguez-Pintó I, Espinosa G, Cervera R. Catastrophic antiphospholipid syndrome: The current management approach. Best Pract Res Clin Rheumatol 2016;30(2):239-249. Referans8. Cervera R, Tincani A. European working party on systemic lupus erythematosus and European Forum on antiphospholipid antibodies: two networks promoting European research on autoimmunity. Lupus 2009;18(10):863-868. Referans9. Cervera R, Font J, Gómez-Puerta JA, et al. Validation of the preliminary criteria for the classification of catastrophic antiphospholipid syndrome. Ann Rheum Dis 2005;64(8):1205–1209. Referans10. Espinosa G, Rodríguez-Pintó I, Cervera R. Catastrophic antiphospholipid syndrome: an update. Panminerva Med 2017;59(3):254-268. Referans11. Rodríguez-Pintó I, Moitinho M, Santacreu I, et al. Catastrophic antiphospholipid syndrome (CAPS): Descriptive analysis of 500 patients from the International CAPS Registry. Autoimmun Rev 2016;15(12):1120-1124. Referans12. Mendoza-Pinto C, García-Carrasco M, Cervera R. Role of Infectious Diseases in the Antiphospholipid Syndrome (Including Its Catastrophic Variant). Curr Rheumatol Rep 2018;20(10):62. Referans13. Adhikari A, Chisti MM, Bastola S, KC O. Rare case of catastrophic antiphospholipid syndrome with spontaneous intracranial haemorrhage. BMJ Case Rep 2019;12(3). e227171. Referans14. Ruffatti A, De Silvestro G, Marson P, et al. Catastrophic antiphospholipid syndrome: Lessons from 14 cases successfully treated in a single center. A narrative report. J Autoimmun 2018;93:124-130. Referans15. Carmi O, Berla M, Shoenfeld Y, Levy Y. Diagnosis and management of catastrophic antiphospholipid syndrome. Expert Rev Hematol 2017;10(4):365-374.

Sjögren sendromu ve şiddetli cilt tutulumu mevcut olan katastrofik antifosfolipid sendromu

Year 2020, Volume: 45 Issue: 2, 746 - 751, 30.06.2020
https://doi.org/10.17826/cumj.679449

Abstract

Katastrofik antifosfolipid sendromu; nadir görülen hızlı seyirli mortalitesi yüksek antifosfolipid antikor sendromunun ciddi bir formudur. Sendrom, yaygın mikro trombozlara bağlı olarak multiorgan yetmezliğine neden olur. Bu olguda; 58 yaşında kadın hasta da alt extremite distal bölgeden başlayan ekimozlar üst bacak bölgesine ve üst extremiteye yayılan lezyonlar hızlı seyir göstererek nekrotik alanlara dönüştü. Histopatolojik incelemede dermiste intravasküler trombüsler tespit edilen hastanın laboratuvar bulgularında IgM anti-kardiyolipin antikor ve Lupus antikoagülan’ı pozitifti. Klinik seyir çok hızlı ilerleyen hastada akciğer ve böbrek tutulumu da eşlik etti. Mevcut bulgularla hastaya katastrofik antifosfolipid sendromu tanısı konuldu. Katastrofik antifosfolipid sendromu vakalarının üçte ikisin de, en sık enfeksiyonların tetiklemesine sekonder olarak gelişmektedir. Katastrofik antifosfolipid sendromu’na otoimmün hastalıklar özellikle sistemik lupus eritematozus eşlik etmektedir. Olgumuz; Sjögren sendromunun katastrofik APS etyolojisinde düşünülmesini gerektiğini vurgulamak amacıyla sunulmaktadır.

References

  • Referans1. Asherson RA, Cervera R, de Groot PG, et al. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus 2003;12(7):530–534. Referans2. Nayer A, Ortega LM. Catastrophic antiphospholipid syndrome: a clinical review. J Nephropathol 2014;3(1):9-17 Referans3. Asherson RA. The catastrophic antiphospholipid syndrome. J Rheumatol 1992;19:508–512. Referans4. Cervera R, Rodríguez-Pintó I, Espinosa G. The diagnosis and clinical management of the catastrophic antiphospholipid syndrome: A comprehensive review. J Autoimmun 2018;92:1-11. Referans5. Cervera R1, Bucciarelli S, Plasín MA, et al. Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the “CAPS Registry”. J Autoimmun 2009;32(3-4):240–245. Referans6. Kazzaz NM, McCune WJ, Knight JS. Treatment of catastrophic antiphospholipid syndrome. Curr Opin Rheumatol 2016;28(3):218–227. Referans7. Rodriguez-Pintó I, Espinosa G, Cervera R. Catastrophic antiphospholipid syndrome: The current management approach. Best Pract Res Clin Rheumatol 2016;30(2):239-249. Referans8. Cervera R, Tincani A. European working party on systemic lupus erythematosus and European Forum on antiphospholipid antibodies: two networks promoting European research on autoimmunity. Lupus 2009;18(10):863-868. Referans9. Cervera R, Font J, Gómez-Puerta JA, et al. Validation of the preliminary criteria for the classification of catastrophic antiphospholipid syndrome. Ann Rheum Dis 2005;64(8):1205–1209. Referans10. Espinosa G, Rodríguez-Pintó I, Cervera R. Catastrophic antiphospholipid syndrome: an update. Panminerva Med 2017;59(3):254-268. Referans11. Rodríguez-Pintó I, Moitinho M, Santacreu I, et al. Catastrophic antiphospholipid syndrome (CAPS): Descriptive analysis of 500 patients from the International CAPS Registry. Autoimmun Rev 2016;15(12):1120-1124. Referans12. Mendoza-Pinto C, García-Carrasco M, Cervera R. Role of Infectious Diseases in the Antiphospholipid Syndrome (Including Its Catastrophic Variant). Curr Rheumatol Rep 2018;20(10):62. Referans13. Adhikari A, Chisti MM, Bastola S, KC O. Rare case of catastrophic antiphospholipid syndrome with spontaneous intracranial haemorrhage. BMJ Case Rep 2019;12(3). e227171. Referans14. Ruffatti A, De Silvestro G, Marson P, et al. Catastrophic antiphospholipid syndrome: Lessons from 14 cases successfully treated in a single center. A narrative report. J Autoimmun 2018;93:124-130. Referans15. Carmi O, Berla M, Shoenfeld Y, Levy Y. Diagnosis and management of catastrophic antiphospholipid syndrome. Expert Rev Hematol 2017;10(4):365-374.
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Details

Primary Language English
Subjects Haematology
Journal Section Case Report
Authors

Ebru Karagün 0000-0002-5032-7429

Birgül Öneç 0000-0003-2824-1044

Türkay Akbaş 0000-0002-2150-6866

Mehmet Gamsızkan 0000-0001-9942-4898

Publication Date June 30, 2020
Acceptance Date April 8, 2020
Published in Issue Year 2020 Volume: 45 Issue: 2

Cite

MLA Karagün, Ebru et al. “Sjögren’s Syndrome and Catastrophic Antiphospholipid Syndrome With Severe Skin Involvement”. Cukurova Medical Journal, vol. 45, no. 2, 2020, pp. 746-51, doi:10.17826/cumj.679449.