- Email: [email protected]
Fibroblastic rheumatism: Immunosuppressive therapy is not always required
Joint Bone Spine 81 (2014) 178–179
Available online at www.sciencedirect.com
Case report
Fibroblastic rheumatism: Immunosuppressive therapy is not always required Alice Courties a , Sarah Guégan b , Anne Miquel c , Paul Duriez d , Francis Berenbaum a,∗ , Jérémie Sellam a Service de rhumatologie, université Paris 06, hôpital Saint-Antoine, AP–HP, DHU « inflammation, immunopathologie, biothérapie » I2B, Paris, France Service de dermatologie, université Paris 06, hôpital Tenon, AP–HP, Paris, France c Service d’imagerie médicale, université Paris 06, hôpital Saint-Antoine, AP–HP, Paris, France d Service d’anatomopathologie, université Paris 06, hôpital Tenon, AP–HP, Paris, France a
b
a r t i c l e
i n f o
Article history: Accepted 30 June 2013 Available online 7 October 2013 Keywords: Fibroblastic rheumatism Physical therapy Non-pharmacological treatment Immunosuppressive drugs
a b s t r a c t Fibroblastic rheumatism is a very rare cause of distal and bilateral polyarthritis characterized by cutaneous nodules, sclerodactylitis, thickened palmar fascia and Raynaud phenomenon. Physiopathology remains unknown and the diagnosis is histologic. Despite the use of immunosuppressive agents in some isolated cases with a variable efficacy, we report a case of typical fibroblastic rheumatism with severe digital retraction who dramatically improved after intensive physical therapy without immunosuppressive drugs prescription. Such a case illustrates that improvement may be spontaneous and that non pharmacological approach is a cornerstone in the management of this disease. © 2013 Société franc¸aise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved.
A 42-year-old caucasian patient, referred to our department with a 6-month history of progressive and increasing inflammatory pain and swelling of the wrists, metacarpophalangeal and proximal interphalangeal (PIP) joints, shoulders and knees. He experienced concomitantly, complained of a Raynaud phenomenon of hands without ulcerations, of diffuse myalgia and fatigue without weight loss nor anorexia. Two months after, pain spontaneously decreased while several tender and mobile skin nodules of 5 to 10 mm diameter size appeared on the backside of the fingers on both hands (Fig. 1A). Within 10 days, the patient noticed a rapidly increasing stiffness of the fingers and of the elbows impairing movement dexterity and grip. Clinical examination revealed several painful joints (wrists and PIP joints) and synovitis of the wrists. Fingers, especially the forefingers, were frankly retracted with restricted passive movements and palpable retracted flexor tendons. The patient displayed an induration of the finger pads, distal sclerodactylitis, several firm flesh-colored skin nodules of 5 mm diameter on the backside of the hands and fingers, in periarticular areas, as well as two nodules of 15 mm on the extension face of elbows. All laboratory investigations were normal (erythrocyte sedimentation rate, C reactive protein, hemogram, rheumatoid factor, anti-cyclic citrullinated peptide antibodies, antinuclear antibodies, anti-extractible nuclear antigen including anti-scl70, anti-DNA antibodies and antineutrophil cytoplasmic antibodies). Hand radiographs showed
no erosions or joint space narrowing. The ultrasound of the hands exhibited a hypoechogenic thickening of the superficial palmar fascia. The magnetic resonance imaging (MRI) of both hands showed a carpal synovitis and flexor tenosynovitis of II to V fingers without any erosion (Fig. 2A). No tumors were detected on a thoracic and abdominal CT scan. A skin nodule on one hand was removed: the histological analysis revealed a dermal fibrosis with an increased number of fibroblasts, and peri-vascular edema without any inflammatory cells, histiocytes, giant cells, granulomas, nor foreign bodies. Immunohistochemical stains found that the fibroblast-like cells were positive for smooth muscle actin (SMA), desmin and vimentin (Fig. 1B). Orcein staining displayed a lack of elastic fibers. Alcian blue staining showed extremely rare perivascular mucin deposits. Clinical and histological features were consistent with a diagnosis of fibroblastic rheumatism
∗ Corresponding author. Service de rhumatologie, hôpital Saint-Antoine, 184, rue du faubourg-Saint-Antoine, 75012 Paris, France. Tel.: +33 1 49 28 25 20; fax: +33 1 49 28 25 13. E-mail address: [email protected] (F. Berenbaum).
Fig. 1. A. Initial clinical presentation with subcutaneous skin’s nodules on the hands and fingers retraction at the beginning of the disease. The black arrows point the skin nodules. B. Haematoxylin and eosin staining of a skin nodule dispaying an inscreased number of fibroblasts and a marked dermal fibrosis (× 100).
1297-319X/$ – see front matter © 2013 Société franc¸aise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved. doi:10.1016/j.jbspin.2013.06.012
A. Courties et al. / Joint Bone Spine 81 (2014) 178–179
Fig. 2. A. Initial magnetic resonance imaging (T1 FAT-SAT plus gadolinium sequence) showing midcarpal synovitis and tenosynovitis of the right hand. B. Magnetic resonance imaging after 2 years follow-up: disappearance of the synovitis and absence of erosion.
179
evidence of systemic sclerosis and multicentric reticulohistiocytosis according to the histological analysis of the skin biopsy. Moreover, the patient did not fulfil the classification criteria for rheumatoid arthritis [3]. We used several imaging methods including MRI and have observed that FR may involve synovitis, tenosynovitis as well as fasciitis, which may be objectively evaluated. Such an imaging examination may be highly recommended for diagnosis as well as for an objective follow-up. Therapeutic options are limited and primarily derived from observational data in isolated cases. Multiple pharmacological treatments have been tested such as corticosteroid [4] hydroxychloroquine [5], interferon alpha [6] methotrexate [7], and infliximab [8]. Their efficacy seems variable according to each case, and there is no proof of a structural benefit in case of erosive arthropathy. The course of FR is unpredictable, and a spontaneous improvement may occur as observed in our case. However, residual flexion deformities may be observed as well as clinical relapses. In our case, we did not introduce immunosupressive drugs for several reasons: • pain and skin nodules resolved spontaneously; • no erosive arthropathy was observed even on MRI and; • the patient was reluctant to such treatment.
Fig. 3. Improvement of digital retraction between the initial presentation (A) and 2 years after (B) after intensive physical therapy.
(FR). Intensive physical therapy (3 times/week) without any use of pharmacological treatment led to a dramatic improvement of the retraction (Fig. 3). The pain progressively resolved as well as the skin nodules. After a 2-year follow-up with weekly regular physical therapy, the functional impairment continuously improved and some delicacy in the fingers movements was recovered. Furthermore, the MRI showed neither erosions nor synovitis confirming the dramatic improvement without the use of any immunosuppressive agents (Fig. 2B). 1. Discussion FR, first described in 1980 by Chaouat et al. is a rare disorder of unknown cause characterized by firm, usually periarticular, cutaneous nodules, polyarthritis, sclerodactylitis, thickened palmar fascia and Raynaud phenomenon [1]. An erosive arthropathy may occur in some cases [2]. In our case, the diagnosis was based on: • the presence of all these symptoms; • characteristic histological features and; • the absence of any other defined diseases. The typical histopathological presentation was observed here with a dermal proliferation of randomly arranged fibroblasts in a densely collageous stroma, SMA, desmin and vimentin positive stainings consistent with previous studies supporting a fibroblast/myofibroblast cellular origin of the disease. There was no
On the other hand, we insisted on intensive and regular physical therapy (i.e., range joint exercises and ergotherapy), which allowed an efficient functional improvement for the daily life. The use of non-pharmacological therapies has been rarely reported previously [9,10]. Our case illustrates that physical therapy certainly represents the cornerstone of the management of joint retractions in RF and that immunosuppressive drugs prescription should be carefully discussed in absence of erosive arthropathy taking into account the possibility of spontaneous improvement. Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. References [1] Chaouat Y, Aron-Brunetiere R, Faures B. Une nouvelle entité : le rhumatisme fibroblastique. À propos d’une observation. Rev Rhum Mal Osteoartic 1980;47:345–51. [2] Romas E, Finlay M, Woodruff T. The arthropathy of fibroblastic rheumatism. Arthritis Rheum 1997;40:183–7. [3] Aletaha D, Neogi T, Silman AJ, et al. 2010 rheumatoid arthritis classification criteria: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Ann Rheum Dis 2010;69: 1580–8. [4] Lacour JP, Maquart FX, Bellon G, et al. Fibroblastic rheumatism: clinical, histological, immunohistological, ultrastructural and biochemical study of a case. Br J Dermatol 1993;128:194–202. [5] Schiavon F, Punzi L, Todesco S. Fibroblastic rheumatism a fifteen-year follow-up study. Arthritis Rheum 1998;41:759–60. [6] Ostlere LS, Stevens HP, Jarmulowicz M, et al. Fibroblastic rheumatism. Clin Exp dermatol 1994;19:268–70. [7] Vittecoq O, Mejjad O, Da silva F, et al. Preliminary experience with low dose of methotrexate in fibroblastic rheumatism. Arthritis Rheum 1996;39:2070–3. [8] Romiti R, Levy Neto M, Menta Simonsen Nico M. Response of fibroblastic rheumatism to infliximab. Dermatol Res Pract 2009;2009:715–29. [9] Pedersen JK, Poulsen T, Hørslev-Petersen K. Fibroblastic rheumatism: a Scandinavian case report. Ann Rheum Dis 2005;64:156–7. [10] Watanabe S, Kamada K, Imai H, et al. An Asian case of fibroblastic rheumatism: clinical, radiological, and histological features. Mod Rheumatol 2010;20:423–6.
Available online at www.sciencedirect.com
Case report
Fibroblastic rheumatism: Immunosuppressive therapy is not always required Alice Courties a , Sarah Guégan b , Anne Miquel c , Paul Duriez d , Francis Berenbaum a,∗ , Jérémie Sellam a Service de rhumatologie, université Paris 06, hôpital Saint-Antoine, AP–HP, DHU « inflammation, immunopathologie, biothérapie » I2B, Paris, France Service de dermatologie, université Paris 06, hôpital Tenon, AP–HP, Paris, France c Service d’imagerie médicale, université Paris 06, hôpital Saint-Antoine, AP–HP, Paris, France d Service d’anatomopathologie, université Paris 06, hôpital Tenon, AP–HP, Paris, France a
b
a r t i c l e
i n f o
Article history: Accepted 30 June 2013 Available online 7 October 2013 Keywords: Fibroblastic rheumatism Physical therapy Non-pharmacological treatment Immunosuppressive drugs
a b s t r a c t Fibroblastic rheumatism is a very rare cause of distal and bilateral polyarthritis characterized by cutaneous nodules, sclerodactylitis, thickened palmar fascia and Raynaud phenomenon. Physiopathology remains unknown and the diagnosis is histologic. Despite the use of immunosuppressive agents in some isolated cases with a variable efficacy, we report a case of typical fibroblastic rheumatism with severe digital retraction who dramatically improved after intensive physical therapy without immunosuppressive drugs prescription. Such a case illustrates that improvement may be spontaneous and that non pharmacological approach is a cornerstone in the management of this disease. © 2013 Société franc¸aise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved.
A 42-year-old caucasian patient, referred to our department with a 6-month history of progressive and increasing inflammatory pain and swelling of the wrists, metacarpophalangeal and proximal interphalangeal (PIP) joints, shoulders and knees. He experienced concomitantly, complained of a Raynaud phenomenon of hands without ulcerations, of diffuse myalgia and fatigue without weight loss nor anorexia. Two months after, pain spontaneously decreased while several tender and mobile skin nodules of 5 to 10 mm diameter size appeared on the backside of the fingers on both hands (Fig. 1A). Within 10 days, the patient noticed a rapidly increasing stiffness of the fingers and of the elbows impairing movement dexterity and grip. Clinical examination revealed several painful joints (wrists and PIP joints) and synovitis of the wrists. Fingers, especially the forefingers, were frankly retracted with restricted passive movements and palpable retracted flexor tendons. The patient displayed an induration of the finger pads, distal sclerodactylitis, several firm flesh-colored skin nodules of 5 mm diameter on the backside of the hands and fingers, in periarticular areas, as well as two nodules of 15 mm on the extension face of elbows. All laboratory investigations were normal (erythrocyte sedimentation rate, C reactive protein, hemogram, rheumatoid factor, anti-cyclic citrullinated peptide antibodies, antinuclear antibodies, anti-extractible nuclear antigen including anti-scl70, anti-DNA antibodies and antineutrophil cytoplasmic antibodies). Hand radiographs showed
no erosions or joint space narrowing. The ultrasound of the hands exhibited a hypoechogenic thickening of the superficial palmar fascia. The magnetic resonance imaging (MRI) of both hands showed a carpal synovitis and flexor tenosynovitis of II to V fingers without any erosion (Fig. 2A). No tumors were detected on a thoracic and abdominal CT scan. A skin nodule on one hand was removed: the histological analysis revealed a dermal fibrosis with an increased number of fibroblasts, and peri-vascular edema without any inflammatory cells, histiocytes, giant cells, granulomas, nor foreign bodies. Immunohistochemical stains found that the fibroblast-like cells were positive for smooth muscle actin (SMA), desmin and vimentin (Fig. 1B). Orcein staining displayed a lack of elastic fibers. Alcian blue staining showed extremely rare perivascular mucin deposits. Clinical and histological features were consistent with a diagnosis of fibroblastic rheumatism
∗ Corresponding author. Service de rhumatologie, hôpital Saint-Antoine, 184, rue du faubourg-Saint-Antoine, 75012 Paris, France. Tel.: +33 1 49 28 25 20; fax: +33 1 49 28 25 13. E-mail address: [email protected] (F. Berenbaum).
Fig. 1. A. Initial clinical presentation with subcutaneous skin’s nodules on the hands and fingers retraction at the beginning of the disease. The black arrows point the skin nodules. B. Haematoxylin and eosin staining of a skin nodule dispaying an inscreased number of fibroblasts and a marked dermal fibrosis (× 100).
1297-319X/$ – see front matter © 2013 Société franc¸aise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved. doi:10.1016/j.jbspin.2013.06.012
A. Courties et al. / Joint Bone Spine 81 (2014) 178–179
Fig. 2. A. Initial magnetic resonance imaging (T1 FAT-SAT plus gadolinium sequence) showing midcarpal synovitis and tenosynovitis of the right hand. B. Magnetic resonance imaging after 2 years follow-up: disappearance of the synovitis and absence of erosion.
179
evidence of systemic sclerosis and multicentric reticulohistiocytosis according to the histological analysis of the skin biopsy. Moreover, the patient did not fulfil the classification criteria for rheumatoid arthritis [3]. We used several imaging methods including MRI and have observed that FR may involve synovitis, tenosynovitis as well as fasciitis, which may be objectively evaluated. Such an imaging examination may be highly recommended for diagnosis as well as for an objective follow-up. Therapeutic options are limited and primarily derived from observational data in isolated cases. Multiple pharmacological treatments have been tested such as corticosteroid [4] hydroxychloroquine [5], interferon alpha [6] methotrexate [7], and infliximab [8]. Their efficacy seems variable according to each case, and there is no proof of a structural benefit in case of erosive arthropathy. The course of FR is unpredictable, and a spontaneous improvement may occur as observed in our case. However, residual flexion deformities may be observed as well as clinical relapses. In our case, we did not introduce immunosupressive drugs for several reasons: • pain and skin nodules resolved spontaneously; • no erosive arthropathy was observed even on MRI and; • the patient was reluctant to such treatment.
Fig. 3. Improvement of digital retraction between the initial presentation (A) and 2 years after (B) after intensive physical therapy.
(FR). Intensive physical therapy (3 times/week) without any use of pharmacological treatment led to a dramatic improvement of the retraction (Fig. 3). The pain progressively resolved as well as the skin nodules. After a 2-year follow-up with weekly regular physical therapy, the functional impairment continuously improved and some delicacy in the fingers movements was recovered. Furthermore, the MRI showed neither erosions nor synovitis confirming the dramatic improvement without the use of any immunosuppressive agents (Fig. 2B). 1. Discussion FR, first described in 1980 by Chaouat et al. is a rare disorder of unknown cause characterized by firm, usually periarticular, cutaneous nodules, polyarthritis, sclerodactylitis, thickened palmar fascia and Raynaud phenomenon [1]. An erosive arthropathy may occur in some cases [2]. In our case, the diagnosis was based on: • the presence of all these symptoms; • characteristic histological features and; • the absence of any other defined diseases. The typical histopathological presentation was observed here with a dermal proliferation of randomly arranged fibroblasts in a densely collageous stroma, SMA, desmin and vimentin positive stainings consistent with previous studies supporting a fibroblast/myofibroblast cellular origin of the disease. There was no
On the other hand, we insisted on intensive and regular physical therapy (i.e., range joint exercises and ergotherapy), which allowed an efficient functional improvement for the daily life. The use of non-pharmacological therapies has been rarely reported previously [9,10]. Our case illustrates that physical therapy certainly represents the cornerstone of the management of joint retractions in RF and that immunosuppressive drugs prescription should be carefully discussed in absence of erosive arthropathy taking into account the possibility of spontaneous improvement. Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. References [1] Chaouat Y, Aron-Brunetiere R, Faures B. Une nouvelle entité : le rhumatisme fibroblastique. À propos d’une observation. Rev Rhum Mal Osteoartic 1980;47:345–51. [2] Romas E, Finlay M, Woodruff T. The arthropathy of fibroblastic rheumatism. Arthritis Rheum 1997;40:183–7. [3] Aletaha D, Neogi T, Silman AJ, et al. 2010 rheumatoid arthritis classification criteria: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Ann Rheum Dis 2010;69: 1580–8. [4] Lacour JP, Maquart FX, Bellon G, et al. Fibroblastic rheumatism: clinical, histological, immunohistological, ultrastructural and biochemical study of a case. Br J Dermatol 1993;128:194–202. [5] Schiavon F, Punzi L, Todesco S. Fibroblastic rheumatism a fifteen-year follow-up study. Arthritis Rheum 1998;41:759–60. [6] Ostlere LS, Stevens HP, Jarmulowicz M, et al. Fibroblastic rheumatism. Clin Exp dermatol 1994;19:268–70. [7] Vittecoq O, Mejjad O, Da silva F, et al. Preliminary experience with low dose of methotrexate in fibroblastic rheumatism. Arthritis Rheum 1996;39:2070–3. [8] Romiti R, Levy Neto M, Menta Simonsen Nico M. Response of fibroblastic rheumatism to infliximab. Dermatol Res Pract 2009;2009:715–29. [9] Pedersen JK, Poulsen T, Hørslev-Petersen K. Fibroblastic rheumatism: a Scandinavian case report. Ann Rheum Dis 2005;64:156–7. [10] Watanabe S, Kamada K, Imai H, et al. An Asian case of fibroblastic rheumatism: clinical, radiological, and histological features. Mod Rheumatol 2010;20:423–6.