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Journals / Cukurova Medical Journal / 2020 / Cilt: 45 - Sayı: 2

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

Sjögren sendromu ve şiddetli cilt tutulumu mevcut olan katastrofik antifosfolipid sendromu

Ebru KARAGÜN
Birgül ÖNEÇ
Türkay AKBAŞ
Mehmet GAMSIZKAN
Pages
746–751
Publication Date
2020-06-30
DOI
—
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

This report is presented to emphasize that Sjögren's syndrome should be considered in the etiology of catastrophic antiphospholipid syndrome.

📌 3 Key Takeaways

🎯
Objective & Hypothesis:Catastrophic antiphospholipid syndrome is a rare but rapidly progressing form of antiphospholipid antibody syndrome with high mortality.
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Key Findings & Data: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.
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Conclusion & Impact:This report is presented to emphasize that Sjögren's syndrome should be considered in the etiology of catastrophic antiphospholipid syndrome.
🔬Design:Research Article
📍Scope:Comprehensive Sample
⚙️Variables:Antiphospholipid syndrome · catastrophic · sjögren syndrome

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.

Özet

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.

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Cite This Article

KARAGÜN, E., ÖNEÇ, B., AKBAŞ, T., GAMSIZKAN, M. (2020). Sjögren’s syndrome and catastrophic antiphospholipid syndrome with severe skin involvement. Cukurova Medical Journal, Cilt: 45 - Sayı: 2, 746–751. https://ajindex.com/articles/111325

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References (14)

  1. Asherson RA, Cervera R, de Groot PG, Erkan D, Boffa MC, Piette JC et al. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus. 2003;12:530–34
  2. Nayer A, Ortega LM. Catastrophic antiphospholipid syndrome: a clinical review. J Nephropathol. 2014;3:9- 17
  3. Asherson RA. The catastrophic antiphospholipid syndrome. J Rheumatol. 1992;19:508–12
  4. 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
  5. Cervera R, Bucciarelli S, Plasín MA, Gómez-Puerta JA, Plaza J, Pons-Estel G et al. Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the “CAPS Registry”. J Autoimmun. 2009;32:240–45
  6. Kazzaz NM, McCune WJ, Knight JS. Treatment of catastrophic antiphospholipid syndrome. Curr Opin Rheumatol. 2016;28:218–27
  7. Rodriguez-Pintó I, Espinosa G, Cervera R. Catastrophic antiphospholipid syndrome: The current management approach. Best Pract Res Clin Rheumatol. 2016;30:239-49
  8. 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;18:863-68
  9. Cervera R, Font J, Gómez-Puerta JA, Espinosa G, Cucho M, Bucciarelli S et al. Validation of the preliminary criteria for the classification of catastrophic antiphospholipid syndrome. Ann Rheum Dis. 2005;64:1205–09
  10. Espinosa G, Rodríguez-Pintó I, Cervera R. Catastrophic antiphospholipid syndrome: an update. Panminerva Med. 2017;59:254-68
  11. Rodríguez-Pintó I, Moitinho M, Santacreu I, Shoenfeld Y, Erkan D, Espinosa G et al. Catastrophic antiphospholipid syndrome (CAPS): Descriptive analysis of 500 patients from the International CAPS Registry. Autoimmun Rev. 2016;15:1120-24
  12. Mendoza-Pinto C, García-Carrasco M, Cervera R. Role of infectious diseases in the antiphospholipid syndrome (including its catastrophic variant). Curr Rheumatol Rep. 2018;20:62
  13. Adhikari A, Chisti MM, Bastola S, KC O. Rare case of catastrophic antiphospholipid syndrome with spontaneous intracranial haemorrhage. BMJ Case Rep. 2019;12. e227171
  14. Ruffatti A, De Silvestro G, Marson P, Tonello M, Calligaro A, Favaro M et al. Catastrophic antiphospholipid syndrome: Lessons from 14 cases successfully treated in a single center. A narrative report. J Aut