Case Report
Coexistence of Axial Spondyloarthritis, Systemic Lupus Erythematosus, Sjögren’s Syndrome and Secondary Antiphospholipid Syndrome: Case Report
Abstract
Hereby we report a 55-year-old female patient having the association of non-radiographic axial spondyloarthritis (nr-AxSpA), systemic lupus erythematosus (SLE), secondary antiphospholipid antibody syndrome (APS), and Sjögren's Syndrome (SjS). Diagnosis of nr-AxSpA was made based upon the presence inflammatory low-back pain, human leukocyte antigen B27 positivity, and presence of sacroiliitis only in magnetic resonance imaging (MRI). SLE was diagnosed with butterfly-shaped rash on her cheeks, inflammatory arthritis, photosensitivity, erythema involving dorsal inter-joint area of hand fingers, alopecia together with antinuclear antibody (ANA) and anti-dsDNA positivity, low serum complement levels, leucopenia and thrombocytopenia. Additional presence of sicca symptoms, low Schirmer I test, anti SSA/Ro and anti-SSB/La positivity, supported by positive labial salivary gland biopsy led to the diagnosis of SjS. Furthermore, this patient also had miscarriage at 16th week and cerebral vascular disease at 33 years. Besides, IgG and IgM anticardiolipin antibodies were found to be positive twice. Therefore, she was also diagnosed as secondary APS. She fulfilled the relevant classification criteria for AxSpA, SLE, SjS and APS. To our knowledge, this is the first case report showing the association of these four diseases, with different genetic, etiopathogenetic and clinical systemic inflammatory diseases.
Keywords
Antiphospholipid Antibody Syndrome Non-Radiographic Axial Spondyloarthritis Systemic Lupus Erythematosus Sjögren Syndrome
Supporting Institution
None
Project Number
None
References
- 1. Rudwaleit M, Landewé R, van der Heijde D, et al. The development of Assessment of Spondyloarthritis International Society classification criteria for axial spondyloarthritis (part I): Classification of paper patients by expert opinion including uncertainty appraisal. Ann Rheum Dis. 2009;68:770-6.
- 2. Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1997;40:1725.
- 3. Thorne I, Sutcliffe N. Sjögren's syndrome. Br J Hosp Med (Lond). 2017,78(8):438-42.
- 4. Miyakis S, Lockshin MD, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4:295-306.
- 5. Petri M, Orbai AM, Alarcón GS, et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum. 2012;64(8):2677-86.
- 6. Caroline H. Shiboski, Stephen C. Shiboski, et.al. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjogren’s Syndrome. Arthritis Rheumatol. 2017;69(1):35-45.
- 7. Miyakis S, Lockshin MD, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4:295-306.
- 8. Juanola X, Mateo L, Domenech P, et.al. Prevalence of antiphospholipid antibodies in patients with ankylosing spondylitis. J Rheumatol. 1995;22(10):1891-3.
- 9. Buchanan RR, Wardlaw JR, Riglar AG, et al. Antiphospholipid antibodies in the connective tissue diseases: their relation to the antiphospholipid syndrome and forme fruste disease. J Rheumatol. 1989;16(6):757-61.
- 10. Mateo L, Juanola X, Nolla JM, et al. Antiphospholipid syndrome in patients with ankylosing spondylitis. Presentation of 2 cases. Med Clin (Barc). 1992;14;98(10):381-3.
- 11. Karter Y, Ersin Y, Bilici A, et.al. Cutaneous vasculitis and antiphospholipid syndrome in a patient with ankylosing spondylitis: How may they be related? J Clin Rheumatol. 2002;8(3):183-5.
- 12. Blanchard B, Lazareth I, Brossel C, et al. Lupus anticoagulant and leg ulcers associated with ankylosing spondylitis. J Rheumatol. 1991;18(12):1922-3.
- 13. Brandt J, Rudwaleit M, Eggens U, et. al. Increased frequency of Sjögren's syndrome in patients with spondyloarthropathy. J Rheumatol. 1998;25(4):718-24.
- 14. Kobak S, Kobak AC, Kabasakal Y, et al. Sjögren's syndrome in patients with ankylosing spondylitis. Clin Rheumatol. 2007;26(2):173-5.
- 15. Tarhan F, Argın M, Can G, et al. Coexistence of systemic lupus erythematosus and ankylosing spondylitis: another case report and review of the literature. Eur J Rheumatol. 2014;1:39-43.
Aksiyel Spondiloartrit, Sistemik Lupus Eritematozus, Sjögren Sendromu ve Sekonder Antifosfolipid Sendromunun Birlikte Görüldüğü Olgu Sunumu
Abstract
Non-Radyografik aksiyel spondiloartrit (nr-axSpA), sistemik lupus eritematozus (SLE), sekonder antifosfolipid antikor sendromu (APS) ve Sjögren Sendromu (SjS) birlikteliğine sahip 55 yaşında bir kadın hastayı sunuyoruz. Nr-AxSpA tanısı enflamatuar bel ağrısı, insan lökosit antijeni B27 pozitifliği ve manyetik rezonans görüntülemede sakroileit varlığına dayanılarak yapıldı. SLE tanısı yanaklarında kelebek şeklinde döküntü, enflamatuar artrit, fotosensitivite, el parmaklarının dorsal eklemler arası alanı tutan eritem, antinükleer antikor (ANA) ve anti-dsDNA pozitifliği, alopesi, düşük serum kompleman seviyeleri, lökopeni ve trombositopeni varlığı ile konuldu. Pozitif labiyal tükürük bezi biyopsisi ile desteklenen sicca semptomları, düşük Schirmer testi, Anti SSA/Ro ve anti-SSB/La pozitifliği ile SjS tanısı konuldu. Ayrıca bu hastada 16. haftada düşük ve 33 yaşında serebrovasküler olay öyküsü vardı. IgG ve IgM antikardiolipin antikorlarının iki kez pozitif olduğu bulundu. Bu nedenle, kendisine sekonder APS tanısı konuldu. Hasta Ax-SpA, SLE, SjS ve APS için sınıflandırma kriterleri doldurmaktadır. Bilindiği üzere farklı genetik, etiyopatogenetik ve klinik özellikleri olan bu dört hastalığın bir arada bulunduğu ilk vaka sunumudur.
Keywords
Antifosfolipit Antikor Sendromu Radyolojik Olmayan Aksiyel Spondilartrit Sistemik Lupus Eritamatozus Sjögren Sendromu
Project Number
None
References
- 1. Rudwaleit M, Landewé R, van der Heijde D, et al. The development of Assessment of Spondyloarthritis International Society classification criteria for axial spondyloarthritis (part I): Classification of paper patients by expert opinion including uncertainty appraisal. Ann Rheum Dis. 2009;68:770-6.
- 2. Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1997;40:1725.
- 3. Thorne I, Sutcliffe N. Sjögren's syndrome. Br J Hosp Med (Lond). 2017,78(8):438-42.
- 4. Miyakis S, Lockshin MD, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4:295-306.
- 5. Petri M, Orbai AM, Alarcón GS, et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum. 2012;64(8):2677-86.
- 6. Caroline H. Shiboski, Stephen C. Shiboski, et.al. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjogren’s Syndrome. Arthritis Rheumatol. 2017;69(1):35-45.
- 7. Miyakis S, Lockshin MD, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4:295-306.
- 8. Juanola X, Mateo L, Domenech P, et.al. Prevalence of antiphospholipid antibodies in patients with ankylosing spondylitis. J Rheumatol. 1995;22(10):1891-3.
- 9. Buchanan RR, Wardlaw JR, Riglar AG, et al. Antiphospholipid antibodies in the connective tissue diseases: their relation to the antiphospholipid syndrome and forme fruste disease. J Rheumatol. 1989;16(6):757-61.
- 10. Mateo L, Juanola X, Nolla JM, et al. Antiphospholipid syndrome in patients with ankylosing spondylitis. Presentation of 2 cases. Med Clin (Barc). 1992;14;98(10):381-3.
- 11. Karter Y, Ersin Y, Bilici A, et.al. Cutaneous vasculitis and antiphospholipid syndrome in a patient with ankylosing spondylitis: How may they be related? J Clin Rheumatol. 2002;8(3):183-5.
- 12. Blanchard B, Lazareth I, Brossel C, et al. Lupus anticoagulant and leg ulcers associated with ankylosing spondylitis. J Rheumatol. 1991;18(12):1922-3.
- 13. Brandt J, Rudwaleit M, Eggens U, et. al. Increased frequency of Sjögren's syndrome in patients with spondyloarthropathy. J Rheumatol. 1998;25(4):718-24.
- 14. Kobak S, Kobak AC, Kabasakal Y, et al. Sjögren's syndrome in patients with ankylosing spondylitis. Clin Rheumatol. 2007;26(2):173-5.
- 15. Tarhan F, Argın M, Can G, et al. Coexistence of systemic lupus erythematosus and ankylosing spondylitis: another case report and review of the literature. Eur J Rheumatol. 2014;1:39-43.
There are 15 citations in total.
Details
| Primary Language | English |
|---|---|
| Subjects | Internal Diseases |
| Journal Section | Case Report |
| Authors | |
| Project Number | None |
| Publication Date | April 29, 2022 |
| Submission Date | March 18, 2021 |
| Published in Issue | Year 2022 Volume: 9 Issue: 1 |
