- Email: [email protected]
Erosive hand osteoarthritis and systemic sclerosis: A casual association?
Joint Bone Spine 79 (2012) 507–509
Available online at
www.sciencedirect.com
Case report
Erosive hand osteoarthritis and systemic sclerosis: A casual association? Roberta Ramonda ∗ , Teresa Del Ross , Valentina Modesti , Mariagrazia Lorenzin , Carla Campana , Leonardo Punzi Rheumatology Unit, Department of Medicine, University of Padova, Via Giustiniani 2, 35128 Padova, Italy
a r t i c l e
i n f o
Article history: Accepted 15 June 2012 Available online 26 July 2012 Keywords: Erosive hand osteoarthritis Osteoarthritis Systemic sclerosis Autoimmune diseases
a b s t r a c t To describe an unexpected association between a patient affected with erosive hand osteoarthritis (EHOA) and systemic sclerosis (SSc). We report a case of SSc presenting typical radiological findings of EHOA in a 60-year-old woman referred to our outpatient Rheumatology Unit. Physical examination revealed puffy hands with sclerodactyly and concomitant adduction of the thumb and subluxation of the first carpometacarpal (CMC) and metacarpophalangeal (MCP) joints bilaterally and nodose deformities of the distal interphalangeal (DIP). Hand X-rays showed joint space narrowing, osteophytosis and bone sclerosis of the proximal interphalangeal (PIP) joints. The DIP joints showed central bone erosion, collapse of the subchondral bone plate and typical “gull-wing” type deformity. EHOA is a particularly aggressive subset of osteoarthritis (OA). In light of its still unclear pathogenesis, peculiarities in the disease characteristics may be useful to better define the EHOA patient profile. One of these is an unexpected association with some autoimmune diseases. EHOA and SSc in the same patient is not uncommon, and it could speculate that there may be a genetic and autoimmune involvement. © 2012 Published by Elsevier Masson SAS on behalf of the Société Française de Rhumatologie.
1. Introduction We report a case of systemic sclerosis (SSc) presenting typical radiological findings of erosive hand osteoarthritis (EHOA) in a 60year-old woman referred to our outpatient Rheumatology Unit. Erosive hand osteoarthritis (EHOA) is a particularly aggressive subset of osteoarthritis (OA) characterized by severe onset with pain and swelling involving the distal (DIP) and proximal interphalangeal (PIP) joints and radiographic findings of subchondral erosions in at least one interphalangeal joint (IP) [1–4]. In light of its still unclear pathogenesis, peculiarities in the disease characteristics may be useful to better define the EHOA patient profile. One of these is an unexpected association with some autoimmune diseases such as systemic sclerosis (SSc), Sjögren’s syndrome and autoimmune thyroiditis noted in the literature [1–7]. SSc is a multisystem connective tissue disease characterized by autoimmunity, microangiopathy and progressive interstitial and vascular fibrosis of the skin and internal organs [8]. Articular involvement, particularly of the hands and feet, is common in these patients and sometimes disabling [9]. Calcinosis and acro-osteolysis, both associated with vascular complications and highlighting a potential role of vascular injury in these lesions, are the main radiological features of hand involvement in patients with
∗ Corresponding author. Tel.: +39 049 8212199; fax: +39 049 8212191. E-mail address: [email protected] (R. Ramonda).
SSc [10,11]. Other aspects such as synovitis and bone erosions are nonetheless frequently underestimated [9–12].
2. Case report We report a case of SSc presenting typical radiological findings of EHOA in a 60-year-old woman referred to our outpatient Rheumatology Unit. The patient’s hands appeared puffy with sclerodactyly and concomitant adduction of the thumb and subluxation of the first carpometacarpal (CMC) and metacarpophalangeal (MCP) joints bilaterally and nodose deformities of the 2nd, 3rd and 5th DIP right joints and of the 3rd and 4th DIP left joints (Fig. 1). Disease onset, which was gradual, began 10 years earlier and was characterized by marked pain and functional impairment as well as inflammatory symptoms such as spontaneous pain, morning stiffness, swelling and tenderness of the IP joints [1,5]. Physical examination at the time of referral revealed cutaneous thickening of the face, forearms, and legs. The extent of skin thickening according to the mRodman total skin thickness scoring system was 19. A capillaroscopic examination highlighted an active scleroderma pattern. Laboratory tests resulted positive to antitopoisomerasi antibodies. A systemic assessment, chest X-ray, pulmonary function tests and carbon monoxide diffusion capacity divided by alveolar volume -DLCO/VA-, and Doppler echocardiography did not show any organ involvement. A functional scintigraphy, instead, revealed transit delay in the lower two thirds of the esophagus. Hand X-rays showed joint space narrowing,
1297-319X/$ – see front matter © 2012 Published by Elsevier Masson SAS on behalf of the Société Française de Rhumatologie. doi:10.1016/j.jbspin.2012.06.006
508
R. Ramonda et al. / Joint Bone Spine 79 (2012) 507–509
Fig. 1. Joint deformities in DIP “gull-wing” and “saw-tooth” (␣) alterations, “crumbling erosion” () of the central surface of the bone, “collapsed” (), hyperextension of MCP joint (␦) and “pencilling” ().
osteophytosis and bone sclerosis of the PIP joints (Fig. 1). The DIP joints (2nd, 3rd, 4th and 5th right and 2nd and 4th left) showed central bone erosion, collapse of the subchondral bone plate and typical “gull-wing” type deformity (Fig. 1). Both CMC joints also showed severe erosions of the trapezium and adjacent metacarpal bones with radial subluxation of the metacarpal base and hyperextension of the unstable MCP joint (Figs. 1 and 2). 3. Discussion In accordance with the European League Against Rheumatism (EULAR) definition, EHOA is generally defined as radiographic subchondral erosion, cortical destruction and subsequent reparative alterations which may include bony ankylosis [4]. The erosions considered characteristic of EHOA occur within the articulating surfaces of the DIP and PIP in the subchondral bone (central) typically showing the classic “seagull-wing” or “saw-tooth” pattern [1–4] (Figs. 1 and 2). Acro-osteolysis is, instead, one of the most characteristic radiographic findings in SSc patients in whom terminal digital tuft
resorption tends to occur. Bone resorption generally begins on the tuft, particularly on the palmar surface of the bone and leads to “pencilling” or sharpening of the phalanx (Fig. 1). In severe cases, much or all of the distal phalange may be destroyed, resulting in finger tapering. As suggested by Sharer and Smith, acro-osteolysis can be attributed to vascular injury possibly due to repeated vasospasm [10]. Recently, while investigating the frequency of hand radiographic changes with erosive characteristics in 46 SSc patients, Koutaissoff et al. observed that DIP joints were involved less frequently than other joint sites and affected with joint space narrowing in 4.3% and erosions in 2.1% [11]. Studying radiologic hand involvement in 120 patients with SSc, Avouac et al. observed that 25 (21%) had erosive changes, mainly in the DIP joints (18/25 = 72%) [12]. The aspects described by these investigators seem to be consistent with EHOA diagnosis [12]. Baron et al. reported typical radiological signs of EHOA involvement in 18.42% of the 38 SSc patients studied [13]. Both Baron and Avouac noted that the majority of their patients were female and postmenopausal, groups which tend to be prone to erosive OA and in which the presence of arthropathy could be unrelated to SSc. In the case of the woman described here, severe erosive changes were found in the CMC joints and although alterations in this location are frequently noted in EHOA patients it is not considered a characteristic feature of this disease [1]. Interestingly, Resnick observed a selective involvement of the CMC joint in four women affected with SSc [14]. Contracture of the thumb in adduction was a prominent feature in those patients in whom radiographic abnormalities were bilateral and consisted of distinctive resorption of the trapezium and adjacent metacarpal with varying degrees of radial subluxation of the metacarpal base [14]. The same radiographic findings were described by Baron in two patients [13] and noted in our patient (Fig. 2). In addition to radiographic changes in the DIP in our patient, there was a scalloped pattern of osseous erosion of the first metacarpal base and adjacent trapezium and subluxation. This is an important aspect as the CMC joint can be involved in a typical EHOA form, but it is not a frequent finding and is generally not so severe [1]. While EHOA clinically preceded the onset of SSc in our patient, her erosions were characterized by features of both diseases. The severe and destructive DIP lesions, the erosive and selective involvement of the CMC joints seem to confirm the association between the two pathologies in this patient. We conclude by pointing out that EHOA and SSc in the same patient is not uncommon, and hypothesizing that there may be a genetic and autoimmune involvement, as has already been postulated by ourselves and others [6,15]. Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. Acknowledgements The authors are grateful to Mrs. Linda Inverso Moretti for editing the English version of this manuscript. References
Fig. 2. Bilateral subluxation of the thumbs and “puffy hand” scleroderma pattern.
[1] Punzi L, Ramonda R, Sfriso P. Erosive osteoarthritis. Best Pract Res Clin Rheumatol 2004;18:739–58. [2] Punzi L, Frigato M, Frallonardo P, et al. Inflammatory osteoarthritis of the hand. Best Pract Res Clin Rheumatol 2010;24:301–12. [3] Belhorn LR, Hess EV. Erosive osteoarthritis. Semin Arthritis Rheum 1993;22:298–306. [4] Zhang W, Doherty M, Leeb BF, et al. EULAR evidence based recommendations for the diagnosis of hand osteoarthritis – report of a task force of the EULAR
R. Ramonda et al. / Joint Bone Spine 79 (2012) 507–509
[5]
[6] [7] [8] [9]
standing committee for international clinical studies including therapeutics (ESCISIT). Ann Rheum Dis 2009;68:8–17. Punzi L, Michelotto M, Pianon M, et al. Clinical, laboratory and immunogenetic aspects of arthritis associated with chronic lymphocytic thyroiditis. Clin Exp Rheumatol 1997;15:373–80. Ramonda R, Lo Nigro A, Campana C, et al. An unusual association between erosive hand osteoarthritis and morphea. Joint Bone Spine 2011;78:532–3. Shuckett R, Russel ML, Gladman DD, et al. Atypical erosive osteoarthritis and Sjögren’s syndrome. Ann Rheum Dis 1986;45:281–5. Denton CP, Black CM, Abraham DJ. Mechanisms and consequences of fibrosis in systemic sclerosis. Nat Clin Pract Rheumatol 2006;2:134–44. La Montagna G, Sodano A, Capurro V, et al. The arthropathy of systemic sclerosis: a 12-month prospective clinical and imaging study. Skeletal Radiol 2005;34:35–41.
509
[10] Scharer L, Smith DW. Resorption of the terminal phalanges in scleroderma. Arthritis Rheum 1969;12:51–63. [11] Koutaissoff S, Vanthuyne M, Smith V, et al. Hand radiological damage in systemic sclerosis: comparison with a control group and clinical and functional correlations. Semin Arthritis Rheum 2011;40:455–60. [12] Avouac J, Guerini H, Wipff J, et al. Radiological hand involvement in systemic sclerosis. Ann Rheum Dis 2006;65:1088–92. [13] Baron M, Lee P, Keyston EC. The articular manifestations of progressive systemic sclerosis (scleroderma). Ann Rheum Dis 1982;41:147–52. [14] Resnick D, Greenway G, Vint VC, et al. Selective involvement of the first carpometacarpal joint in scleroderma. Am J Roentgenol 1978;131:283–6. [15] Ramonda R, Musacchio E, Campana C, et al. Immunogenetic aspects of erosive osteoarthritis of the hand in patients from northern Italy. Scand J Rheumatol 2011;40:139–44.
Available online at
www.sciencedirect.com
Case report
Erosive hand osteoarthritis and systemic sclerosis: A casual association? Roberta Ramonda ∗ , Teresa Del Ross , Valentina Modesti , Mariagrazia Lorenzin , Carla Campana , Leonardo Punzi Rheumatology Unit, Department of Medicine, University of Padova, Via Giustiniani 2, 35128 Padova, Italy
a r t i c l e
i n f o
Article history: Accepted 15 June 2012 Available online 26 July 2012 Keywords: Erosive hand osteoarthritis Osteoarthritis Systemic sclerosis Autoimmune diseases
a b s t r a c t To describe an unexpected association between a patient affected with erosive hand osteoarthritis (EHOA) and systemic sclerosis (SSc). We report a case of SSc presenting typical radiological findings of EHOA in a 60-year-old woman referred to our outpatient Rheumatology Unit. Physical examination revealed puffy hands with sclerodactyly and concomitant adduction of the thumb and subluxation of the first carpometacarpal (CMC) and metacarpophalangeal (MCP) joints bilaterally and nodose deformities of the distal interphalangeal (DIP). Hand X-rays showed joint space narrowing, osteophytosis and bone sclerosis of the proximal interphalangeal (PIP) joints. The DIP joints showed central bone erosion, collapse of the subchondral bone plate and typical “gull-wing” type deformity. EHOA is a particularly aggressive subset of osteoarthritis (OA). In light of its still unclear pathogenesis, peculiarities in the disease characteristics may be useful to better define the EHOA patient profile. One of these is an unexpected association with some autoimmune diseases. EHOA and SSc in the same patient is not uncommon, and it could speculate that there may be a genetic and autoimmune involvement. © 2012 Published by Elsevier Masson SAS on behalf of the Société Française de Rhumatologie.
1. Introduction We report a case of systemic sclerosis (SSc) presenting typical radiological findings of erosive hand osteoarthritis (EHOA) in a 60year-old woman referred to our outpatient Rheumatology Unit. Erosive hand osteoarthritis (EHOA) is a particularly aggressive subset of osteoarthritis (OA) characterized by severe onset with pain and swelling involving the distal (DIP) and proximal interphalangeal (PIP) joints and radiographic findings of subchondral erosions in at least one interphalangeal joint (IP) [1–4]. In light of its still unclear pathogenesis, peculiarities in the disease characteristics may be useful to better define the EHOA patient profile. One of these is an unexpected association with some autoimmune diseases such as systemic sclerosis (SSc), Sjögren’s syndrome and autoimmune thyroiditis noted in the literature [1–7]. SSc is a multisystem connective tissue disease characterized by autoimmunity, microangiopathy and progressive interstitial and vascular fibrosis of the skin and internal organs [8]. Articular involvement, particularly of the hands and feet, is common in these patients and sometimes disabling [9]. Calcinosis and acro-osteolysis, both associated with vascular complications and highlighting a potential role of vascular injury in these lesions, are the main radiological features of hand involvement in patients with
∗ Corresponding author. Tel.: +39 049 8212199; fax: +39 049 8212191. E-mail address: [email protected] (R. Ramonda).
SSc [10,11]. Other aspects such as synovitis and bone erosions are nonetheless frequently underestimated [9–12].
2. Case report We report a case of SSc presenting typical radiological findings of EHOA in a 60-year-old woman referred to our outpatient Rheumatology Unit. The patient’s hands appeared puffy with sclerodactyly and concomitant adduction of the thumb and subluxation of the first carpometacarpal (CMC) and metacarpophalangeal (MCP) joints bilaterally and nodose deformities of the 2nd, 3rd and 5th DIP right joints and of the 3rd and 4th DIP left joints (Fig. 1). Disease onset, which was gradual, began 10 years earlier and was characterized by marked pain and functional impairment as well as inflammatory symptoms such as spontaneous pain, morning stiffness, swelling and tenderness of the IP joints [1,5]. Physical examination at the time of referral revealed cutaneous thickening of the face, forearms, and legs. The extent of skin thickening according to the mRodman total skin thickness scoring system was 19. A capillaroscopic examination highlighted an active scleroderma pattern. Laboratory tests resulted positive to antitopoisomerasi antibodies. A systemic assessment, chest X-ray, pulmonary function tests and carbon monoxide diffusion capacity divided by alveolar volume -DLCO/VA-, and Doppler echocardiography did not show any organ involvement. A functional scintigraphy, instead, revealed transit delay in the lower two thirds of the esophagus. Hand X-rays showed joint space narrowing,
1297-319X/$ – see front matter © 2012 Published by Elsevier Masson SAS on behalf of the Société Française de Rhumatologie. doi:10.1016/j.jbspin.2012.06.006
508
R. Ramonda et al. / Joint Bone Spine 79 (2012) 507–509
Fig. 1. Joint deformities in DIP “gull-wing” and “saw-tooth” (␣) alterations, “crumbling erosion” () of the central surface of the bone, “collapsed” (), hyperextension of MCP joint (␦) and “pencilling” ().
osteophytosis and bone sclerosis of the PIP joints (Fig. 1). The DIP joints (2nd, 3rd, 4th and 5th right and 2nd and 4th left) showed central bone erosion, collapse of the subchondral bone plate and typical “gull-wing” type deformity (Fig. 1). Both CMC joints also showed severe erosions of the trapezium and adjacent metacarpal bones with radial subluxation of the metacarpal base and hyperextension of the unstable MCP joint (Figs. 1 and 2). 3. Discussion In accordance with the European League Against Rheumatism (EULAR) definition, EHOA is generally defined as radiographic subchondral erosion, cortical destruction and subsequent reparative alterations which may include bony ankylosis [4]. The erosions considered characteristic of EHOA occur within the articulating surfaces of the DIP and PIP in the subchondral bone (central) typically showing the classic “seagull-wing” or “saw-tooth” pattern [1–4] (Figs. 1 and 2). Acro-osteolysis is, instead, one of the most characteristic radiographic findings in SSc patients in whom terminal digital tuft
resorption tends to occur. Bone resorption generally begins on the tuft, particularly on the palmar surface of the bone and leads to “pencilling” or sharpening of the phalanx (Fig. 1). In severe cases, much or all of the distal phalange may be destroyed, resulting in finger tapering. As suggested by Sharer and Smith, acro-osteolysis can be attributed to vascular injury possibly due to repeated vasospasm [10]. Recently, while investigating the frequency of hand radiographic changes with erosive characteristics in 46 SSc patients, Koutaissoff et al. observed that DIP joints were involved less frequently than other joint sites and affected with joint space narrowing in 4.3% and erosions in 2.1% [11]. Studying radiologic hand involvement in 120 patients with SSc, Avouac et al. observed that 25 (21%) had erosive changes, mainly in the DIP joints (18/25 = 72%) [12]. The aspects described by these investigators seem to be consistent with EHOA diagnosis [12]. Baron et al. reported typical radiological signs of EHOA involvement in 18.42% of the 38 SSc patients studied [13]. Both Baron and Avouac noted that the majority of their patients were female and postmenopausal, groups which tend to be prone to erosive OA and in which the presence of arthropathy could be unrelated to SSc. In the case of the woman described here, severe erosive changes were found in the CMC joints and although alterations in this location are frequently noted in EHOA patients it is not considered a characteristic feature of this disease [1]. Interestingly, Resnick observed a selective involvement of the CMC joint in four women affected with SSc [14]. Contracture of the thumb in adduction was a prominent feature in those patients in whom radiographic abnormalities were bilateral and consisted of distinctive resorption of the trapezium and adjacent metacarpal with varying degrees of radial subluxation of the metacarpal base [14]. The same radiographic findings were described by Baron in two patients [13] and noted in our patient (Fig. 2). In addition to radiographic changes in the DIP in our patient, there was a scalloped pattern of osseous erosion of the first metacarpal base and adjacent trapezium and subluxation. This is an important aspect as the CMC joint can be involved in a typical EHOA form, but it is not a frequent finding and is generally not so severe [1]. While EHOA clinically preceded the onset of SSc in our patient, her erosions were characterized by features of both diseases. The severe and destructive DIP lesions, the erosive and selective involvement of the CMC joints seem to confirm the association between the two pathologies in this patient. We conclude by pointing out that EHOA and SSc in the same patient is not uncommon, and hypothesizing that there may be a genetic and autoimmune involvement, as has already been postulated by ourselves and others [6,15]. Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. Acknowledgements The authors are grateful to Mrs. Linda Inverso Moretti for editing the English version of this manuscript. References
Fig. 2. Bilateral subluxation of the thumbs and “puffy hand” scleroderma pattern.
[1] Punzi L, Ramonda R, Sfriso P. Erosive osteoarthritis. Best Pract Res Clin Rheumatol 2004;18:739–58. [2] Punzi L, Frigato M, Frallonardo P, et al. Inflammatory osteoarthritis of the hand. Best Pract Res Clin Rheumatol 2010;24:301–12. [3] Belhorn LR, Hess EV. Erosive osteoarthritis. Semin Arthritis Rheum 1993;22:298–306. [4] Zhang W, Doherty M, Leeb BF, et al. EULAR evidence based recommendations for the diagnosis of hand osteoarthritis – report of a task force of the EULAR
R. Ramonda et al. / Joint Bone Spine 79 (2012) 507–509
[5]
[6] [7] [8] [9]
standing committee for international clinical studies including therapeutics (ESCISIT). Ann Rheum Dis 2009;68:8–17. Punzi L, Michelotto M, Pianon M, et al. Clinical, laboratory and immunogenetic aspects of arthritis associated with chronic lymphocytic thyroiditis. Clin Exp Rheumatol 1997;15:373–80. Ramonda R, Lo Nigro A, Campana C, et al. An unusual association between erosive hand osteoarthritis and morphea. Joint Bone Spine 2011;78:532–3. Shuckett R, Russel ML, Gladman DD, et al. Atypical erosive osteoarthritis and Sjögren’s syndrome. Ann Rheum Dis 1986;45:281–5. Denton CP, Black CM, Abraham DJ. Mechanisms and consequences of fibrosis in systemic sclerosis. Nat Clin Pract Rheumatol 2006;2:134–44. La Montagna G, Sodano A, Capurro V, et al. The arthropathy of systemic sclerosis: a 12-month prospective clinical and imaging study. Skeletal Radiol 2005;34:35–41.
509
[10] Scharer L, Smith DW. Resorption of the terminal phalanges in scleroderma. Arthritis Rheum 1969;12:51–63. [11] Koutaissoff S, Vanthuyne M, Smith V, et al. Hand radiological damage in systemic sclerosis: comparison with a control group and clinical and functional correlations. Semin Arthritis Rheum 2011;40:455–60. [12] Avouac J, Guerini H, Wipff J, et al. Radiological hand involvement in systemic sclerosis. Ann Rheum Dis 2006;65:1088–92. [13] Baron M, Lee P, Keyston EC. The articular manifestations of progressive systemic sclerosis (scleroderma). Ann Rheum Dis 1982;41:147–52. [14] Resnick D, Greenway G, Vint VC, et al. Selective involvement of the first carpometacarpal joint in scleroderma. Am J Roentgenol 1978;131:283–6. [15] Ramonda R, Musacchio E, Campana C, et al. Immunogenetic aspects of erosive osteoarthritis of the hand in patients from northern Italy. Scand J Rheumatol 2011;40:139–44.