G Model
ARTICLE IN PRESS
BONSOI-3990; No. of Pages 2
Joint Bone Spine xxx (2014) xxx–xxx
Available online at www.sciencedirect.com
Letter to the editor 2. Observation
Thrombosis of the left subclavian vein complicating SAPHO syndrome: A case report
a r t i c l e
We report the case of a 36-year-old woman with previous history of post-partum hypothyroidism and proven allergy to nonsteroidal anti-inflammatory drugs (NSAID). Since 2011, the patient complained of several episodes of inflammatory swelling of the left clavicle and recurrent pustulosis of the finger pulps (Supplementary material, Fig. S1). There were no other osteoarticular symptoms (spinal and sacroiliac involvement, peripheral synovitis, long bone involvement, enthesitis) nor other skin manifestations (severe acne, psoriasis). Magnetic resonance imaging revealed an inflammatory hyperostosis of the left clavicle (Supplementary material, Fig. S2); and a subsequent bone biopsy showed lesions of chronic aseptic osteitis. Bone scintigraphy ruled out any other infraclinical osteoarticular localizations. Test for the HLA-B27 antigen was negative. Altogether, the diagnosis of SAPHO syndrome was made; and due to several NSAIDs allergy, no specific treatment was given. In June 2013, the patient presented with warm swelling and painful tension of the left arm. Doppler imaging confirmed the diagnosis of left subclavian vein thrombosis. Thoracic CT-scan ruled out pulmonary embolism and revealed a compression of the left subclavian vein by the clavicular hyperostosis (Fig. 1), resulting in a thoracic outlet syndrome. Physical examination, arterial Doppler
i n f o
Keywords: SAPHO Sterno-costo-clavicular hyperostosis Deep vein thrombosis Subclavian vein thrombosis Superior vena cava syndrome Thoracic outlet syndrome
1. Introduction The SAPHO syndrome (synovitis, acne, palmo-plantar pustulosis, hyperostosis, osteitis) encompasses various conditions with common characteristics. Predominant features are cutaneous and osteoarticular manifestations, especially aseptic osteitis. Thromboembolic events have rarely been reported in patients with SAPHO syndrome. Here, we present an observation of this unusual association.
Table 1 Characteristic features of reported cases of deep vein thrombosis in SAPHO syndrome. Patients
Duration of SAPHO syndrome
Venous localization
Mechanisms
Female 36 yo Female 58 yo
2 years
Left subclavian DVT
Compression (hyperostosis)
3 years
Compression
Coloe et al., 2010 [2]
Male 15 yo
3 months
Right internal jugular, bilateral subclavian, and bilateral brachiocephalic DVT Bilateral iliofemoral DVT Pulmonary embolism (×7)
Kawabata et al., 2005 [3]
Female 42 yo
27 years
Compression (hyperostosis, soft tissue)
Legoupil et al., 2001 [4]
Male 44 yo N/A
3 years
Superior vena caval and left internal jugular DVT Bilateral subclavian stenosis Right iliac DVT
N/A
Hayem et al., 1999 [6]
N/A N/A
Bölükbas¸ et al., 1996 [7]
Male 33 yo Male 45 yo Male 46 yo Male 43 yo
Sanges 2013 Pernès et al., 2013 [1]
Lazzarin et al., 1999 [5]
Cunningham et al., 1993 [8] Van Holsbeeck 1989 et al., [9]
Thrombophilia? Immobilization?
Compression (soft tissue) Compression
N/A N/A
Right subclavian DVT Superior vena caval stenosis Subclavian stenosis Subclavian DVT
Compression (hyperostosis) Compression (hyperostosis)
N/A
Subclavian stenosis
Compression (hyperostosis?)
13 years
Superior vena caval DVT
Compression (soft tissue)
2 months
Subclavian, internal jugular, and right brachiocephalic DVT Bilateral subclavian DVT
Compression (hyperostosis, soft tissue)
10 years
Compression (hyperostosis)
yo: years-old; DVT: deep vein thrombosis; N/A: data not available. 1297-319X/$ – see front matter © 2014 Société franc¸aise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved. doi:10.1016/j.jbspin.2014.02.002
Please cite this article in press as: Sanges S, et al. Thrombosis of the left subclavian vein complicating SAPHO syndrome: A case report. Joint Bone Spine (2014), doi:10.1016/j.jbspin.2014.02.002
G Model BONSOI-3990; No. of Pages 2
ARTICLE IN PRESS Letter to the editor / Joint Bone Spine xxx (2014) xxx–xxx
2
References
Fig. 1. Thoracic CT-scan with contrast injection: hyperostosis of the left clavicle compressing adjacent soft tissues.
imaging and electromyography showed no sign of associated arterial or nervous compression. Thombophilia testing did not reveal any biologic predisposing factor for deep vein thrombosis. The patient was started on anticoagulants and short-term corticosteroids, leading to a favorable outcome. 3. Discussion This observation describes a case of subclavian vein thrombosis in a patient with typical SAPHO syndrome. The underlying mechanism leading to the thromboembolic event was an extrinsic compression of the vein by clavicular hyperostosis, resulting in a thoracic outlet syndrome. In the literature, there are only rare reports of venous thromboembolic events in patients with SAPHO syndrome (Table 1) [1–9]. In almost all cases, venous complications affected the upper limb (mainly the subclavian vein and the superior vena cava) and were explained by compression resulting from adjacent hyperostosis and/or an inflammatory or fibrotic pseudotumor surrounding the vein. Some cases required vascular surgery to release the compressed vessel [5,9]. Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. Appendix A. Supplementary data Supplementary data (Figs. S1–S2) associated with this article can be found, in the online version, at http://www. sciencedirect.com and doi:10.1016/j.jbspin.2014.02.002.
[1] Pernès JM, Carcone B, Becherel PA, et al. Syndrome cave supérieur compliquant un syndrome SAPHO. Sang Thromb Vaiss 2013;25:253–6. [2] Coloe J, Diamantis S, Henderson F, et al. Synovitis-acne-pustulosis-hyperostosisosteitis (SAPHO) syndrome complicated by seven pulmonary emboli in a 15-year old patient. J Am Acad Dermatol 2010;62:333–6. [3] Kawabata T, Morita Y, Nakatsuka A, et al. Multiple venous thrombosis in SAPHO syndrome. Ann Rheum Dis 2005;64:505–6. [4] Legoupil N, Révelon G, Allain J, et al. Iliac vein thrombosis complicating SAPHO syndrome: MRI and histologic features of soft tissue lesions. Joint Bone Spine 2001;68:79–83. [5] Lazzarin P, Punzi L, Cesaro G, et al. Thrombosis of the subclavian vein in SAPHO syndrome. A case-report. Rev Rhum Engl Ed 1999;66:173–6. [6] Hayem G, Bouchaud-Chabot A, Benali K, et al. SAPHO syndrome: a long-term follow-up study of 120 cases. Semin Arthritis Rheum 1999;29:159–71. [7] Bölükbas¸ N, Bölükbas¸ S, Erel A, et al. Sapho syndrome associated with acne fulminans and prominent acromioclavicular joint involvement. Scand J Rheumatol 1996;25:180–2. [8] Cunningham T, Farrell J, Veale D, et al. Anterior mediastinal fibrosis with superior vena caval obstruction complicating the synovitis-acnepustulosis-hyperostosis-osteomyelitis syndrome. Br J Rheumatol 1993;32: 408–10. [9] Van Holsbeeck M, Martel W, Dequeker J, et al. Soft tissue involvement, mediastinal pseudotumor, and venous thrombosis in pustulotic arthro-osteitis. A study of eight new cases. Skeletal Radiol 1989;18:1–8.
Sébastien Sanges a,∗ Vincent Ducoulombier a Bruno Sivery b Nicolas Delhaye c Clarisse Haffner a Eric Houvenagel a a Department of Rheumatology, Saint-Philibert Hospital, 115, rue du Grand-But, 59160 Lomme, France b Department of Medicine, Wattrelos Hospital, rue du Dr-Alexander-Fleming, 59393 Wattrelos, France c Department of Vascular Surgery, Saint-Philibert Hospital, 115, rue du Grand-But, 59160 Lomme, France ∗ Corresponding
author. Tel.: +33 3 20 22 50 59; fax: +33 3 20 22 38 76. E-mail address: sebastien [email protected] (S. Sanges) Accepted 28 January 2014 Available online xxx
Please cite this article in press as: Sanges S, et al. Thrombosis of the left subclavian vein complicating SAPHO syndrome: A case report. Joint Bone Spine (2014), doi:10.1016/j.jbspin.2014.02.002
ARTICLE IN PRESS
BONSOI-3990; No. of Pages 2
Joint Bone Spine xxx (2014) xxx–xxx
Available online at www.sciencedirect.com
Letter to the editor 2. Observation
Thrombosis of the left subclavian vein complicating SAPHO syndrome: A case report
a r t i c l e
We report the case of a 36-year-old woman with previous history of post-partum hypothyroidism and proven allergy to nonsteroidal anti-inflammatory drugs (NSAID). Since 2011, the patient complained of several episodes of inflammatory swelling of the left clavicle and recurrent pustulosis of the finger pulps (Supplementary material, Fig. S1). There were no other osteoarticular symptoms (spinal and sacroiliac involvement, peripheral synovitis, long bone involvement, enthesitis) nor other skin manifestations (severe acne, psoriasis). Magnetic resonance imaging revealed an inflammatory hyperostosis of the left clavicle (Supplementary material, Fig. S2); and a subsequent bone biopsy showed lesions of chronic aseptic osteitis. Bone scintigraphy ruled out any other infraclinical osteoarticular localizations. Test for the HLA-B27 antigen was negative. Altogether, the diagnosis of SAPHO syndrome was made; and due to several NSAIDs allergy, no specific treatment was given. In June 2013, the patient presented with warm swelling and painful tension of the left arm. Doppler imaging confirmed the diagnosis of left subclavian vein thrombosis. Thoracic CT-scan ruled out pulmonary embolism and revealed a compression of the left subclavian vein by the clavicular hyperostosis (Fig. 1), resulting in a thoracic outlet syndrome. Physical examination, arterial Doppler
i n f o
Keywords: SAPHO Sterno-costo-clavicular hyperostosis Deep vein thrombosis Subclavian vein thrombosis Superior vena cava syndrome Thoracic outlet syndrome
1. Introduction The SAPHO syndrome (synovitis, acne, palmo-plantar pustulosis, hyperostosis, osteitis) encompasses various conditions with common characteristics. Predominant features are cutaneous and osteoarticular manifestations, especially aseptic osteitis. Thromboembolic events have rarely been reported in patients with SAPHO syndrome. Here, we present an observation of this unusual association.
Table 1 Characteristic features of reported cases of deep vein thrombosis in SAPHO syndrome. Patients
Duration of SAPHO syndrome
Venous localization
Mechanisms
Female 36 yo Female 58 yo
2 years
Left subclavian DVT
Compression (hyperostosis)
3 years
Compression
Coloe et al., 2010 [2]
Male 15 yo
3 months
Right internal jugular, bilateral subclavian, and bilateral brachiocephalic DVT Bilateral iliofemoral DVT Pulmonary embolism (×7)
Kawabata et al., 2005 [3]
Female 42 yo
27 years
Compression (hyperostosis, soft tissue)
Legoupil et al., 2001 [4]
Male 44 yo N/A
3 years
Superior vena caval and left internal jugular DVT Bilateral subclavian stenosis Right iliac DVT
N/A
Hayem et al., 1999 [6]
N/A N/A
Bölükbas¸ et al., 1996 [7]
Male 33 yo Male 45 yo Male 46 yo Male 43 yo
Sanges 2013 Pernès et al., 2013 [1]
Lazzarin et al., 1999 [5]
Cunningham et al., 1993 [8] Van Holsbeeck 1989 et al., [9]
Thrombophilia? Immobilization?
Compression (soft tissue) Compression
N/A N/A
Right subclavian DVT Superior vena caval stenosis Subclavian stenosis Subclavian DVT
Compression (hyperostosis) Compression (hyperostosis)
N/A
Subclavian stenosis
Compression (hyperostosis?)
13 years
Superior vena caval DVT
Compression (soft tissue)
2 months
Subclavian, internal jugular, and right brachiocephalic DVT Bilateral subclavian DVT
Compression (hyperostosis, soft tissue)
10 years
Compression (hyperostosis)
yo: years-old; DVT: deep vein thrombosis; N/A: data not available. 1297-319X/$ – see front matter © 2014 Société franc¸aise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved. doi:10.1016/j.jbspin.2014.02.002
Please cite this article in press as: Sanges S, et al. Thrombosis of the left subclavian vein complicating SAPHO syndrome: A case report. Joint Bone Spine (2014), doi:10.1016/j.jbspin.2014.02.002
G Model BONSOI-3990; No. of Pages 2
ARTICLE IN PRESS Letter to the editor / Joint Bone Spine xxx (2014) xxx–xxx
2
References
Fig. 1. Thoracic CT-scan with contrast injection: hyperostosis of the left clavicle compressing adjacent soft tissues.
imaging and electromyography showed no sign of associated arterial or nervous compression. Thombophilia testing did not reveal any biologic predisposing factor for deep vein thrombosis. The patient was started on anticoagulants and short-term corticosteroids, leading to a favorable outcome. 3. Discussion This observation describes a case of subclavian vein thrombosis in a patient with typical SAPHO syndrome. The underlying mechanism leading to the thromboembolic event was an extrinsic compression of the vein by clavicular hyperostosis, resulting in a thoracic outlet syndrome. In the literature, there are only rare reports of venous thromboembolic events in patients with SAPHO syndrome (Table 1) [1–9]. In almost all cases, venous complications affected the upper limb (mainly the subclavian vein and the superior vena cava) and were explained by compression resulting from adjacent hyperostosis and/or an inflammatory or fibrotic pseudotumor surrounding the vein. Some cases required vascular surgery to release the compressed vessel [5,9]. Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. Appendix A. Supplementary data Supplementary data (Figs. S1–S2) associated with this article can be found, in the online version, at http://www. sciencedirect.com and doi:10.1016/j.jbspin.2014.02.002.
[1] Pernès JM, Carcone B, Becherel PA, et al. Syndrome cave supérieur compliquant un syndrome SAPHO. Sang Thromb Vaiss 2013;25:253–6. [2] Coloe J, Diamantis S, Henderson F, et al. Synovitis-acne-pustulosis-hyperostosisosteitis (SAPHO) syndrome complicated by seven pulmonary emboli in a 15-year old patient. J Am Acad Dermatol 2010;62:333–6. [3] Kawabata T, Morita Y, Nakatsuka A, et al. Multiple venous thrombosis in SAPHO syndrome. Ann Rheum Dis 2005;64:505–6. [4] Legoupil N, Révelon G, Allain J, et al. Iliac vein thrombosis complicating SAPHO syndrome: MRI and histologic features of soft tissue lesions. Joint Bone Spine 2001;68:79–83. [5] Lazzarin P, Punzi L, Cesaro G, et al. Thrombosis of the subclavian vein in SAPHO syndrome. A case-report. Rev Rhum Engl Ed 1999;66:173–6. [6] Hayem G, Bouchaud-Chabot A, Benali K, et al. SAPHO syndrome: a long-term follow-up study of 120 cases. Semin Arthritis Rheum 1999;29:159–71. [7] Bölükbas¸ N, Bölükbas¸ S, Erel A, et al. Sapho syndrome associated with acne fulminans and prominent acromioclavicular joint involvement. Scand J Rheumatol 1996;25:180–2. [8] Cunningham T, Farrell J, Veale D, et al. Anterior mediastinal fibrosis with superior vena caval obstruction complicating the synovitis-acnepustulosis-hyperostosis-osteomyelitis syndrome. Br J Rheumatol 1993;32: 408–10. [9] Van Holsbeeck M, Martel W, Dequeker J, et al. Soft tissue involvement, mediastinal pseudotumor, and venous thrombosis in pustulotic arthro-osteitis. A study of eight new cases. Skeletal Radiol 1989;18:1–8.
Sébastien Sanges a,∗ Vincent Ducoulombier a Bruno Sivery b Nicolas Delhaye c Clarisse Haffner a Eric Houvenagel a a Department of Rheumatology, Saint-Philibert Hospital, 115, rue du Grand-But, 59160 Lomme, France b Department of Medicine, Wattrelos Hospital, rue du Dr-Alexander-Fleming, 59393 Wattrelos, France c Department of Vascular Surgery, Saint-Philibert Hospital, 115, rue du Grand-But, 59160 Lomme, France ∗ Corresponding
author. Tel.: +33 3 20 22 50 59; fax: +33 3 20 22 38 76. E-mail address: sebastien [email protected] (S. Sanges) Accepted 28 January 2014 Available online xxx
Please cite this article in press as: Sanges S, et al. Thrombosis of the left subclavian vein complicating SAPHO syndrome: A case report. Joint Bone Spine (2014), doi:10.1016/j.jbspin.2014.02.002
