Foot involvement in systemic sclerosis: A longitudinal study of 100 patients

doi:10.1053/sarh.2002.29493

Seminars in Arthritis and Rheumatism

Volume 31, Issue 4, February 2002, Pages 248-255

https://doi.org/10.1053/sarh.2002.29493 Get rights and content

Abstract

Objective: To investigate the clinical and radiologic features of foot involvement in systemic sclerosis (SSc). Patients: One hundred patients (91 women, 9 men; mean age, 51.9 ± 11 years) with SSc (mean disease duration, 17.4 ± 10.5 years) were retrospectively studied. Seventy-four subjects had limited scleroderma and 26 diffuse scleroderma. Methods: Radiologic changes of foot involvement were assessed at presentation (time of diagnosis) and follow-up ranging from 1 to 28 years (median range, 7 years) and were compared with changes detected in the hands of each patient at the same presentation and follow-up. Correlations with skin and internal organ involvement were assessed. Results: Ninety patients had foot involvement clinically. Forty-three had it at initial evaluation; 47 developed it during follow-up. Median time to clinical event occurrence was 10 years (95% CI, 6.7-13.3) with 44% censored case probability at this time. The onset of clinically evident foot involvement was later in limited SSc than in diffuse SSc. In comparison with hands with SSc, feet with SSc had lower rates of necrotizing Raynaud's phenomenon and tendon friction rubs and decreased skin thickening scores, whereas arthralgias occurred significantly more often. At presentation, 37 patients had radiologic abnormalities of their feet compared with 69 of their hands (P <.001); the hands had a significantly higher prevalence of acroosteolysis (P <.001). At the end of the follow-up, 35 of 50 SSc patients had radiographic foot involvement compared with 50 of 51 with hand involvement (P <.001). A significantly higher prevalence of acroosteolysis (P <.001), calcinosis (P <.05), and erosions (P <.05) of the hands were detected at that time. Conclusion: This study shows that compared with hand involvement in SSc, foot involvement in SSc has a later onset and is relatively less frequent but can be disabling. Semin Arthritis Rheum 31:248-255. Copyright 2002, Elsevier Science (USA). All rights reserved.

Section snippets

Patients and methods

We examined the clinical records of 100 SSc patients in whom hand and foot radiographs were available in the archives of our clinic in December 2000. All the patients had been admitted to our unit between 1979 and 2000. The baseline epidemiologic and clinical characteristics of this cohort of patients were not different from 223 other SSc patients admitted from January 1, 1965, to June 30, 1994 (our whole series consisting of 323 subjects on June 1994) nor from those of the 183 admitted during

Results

Ninety-six SSc patients satisfied the ACR criteria for the classification of SSc. In the remaining 4 cases, the diagnosis was established by the coexistence of anticentromere antibodies (ACA), sclerodactily, and esophageal involvement.

There were 74 cases with limited scleroderma (lcSSc), and 26 cases with diffuse scleroderma (dcSSc). Ninety-seven were ANA positive, 29 (of 100) were ACA positive, and 52 (of 85; 61%) were anti-Scl 70 positive. RF at a low titer (median, 1:80) was detected in 35

Discussion

Our study has several limitations, including its retrospective nature and the fact that patients had a long disease duration at presentation. Therefore, our conclusions apply only to long-surviving SSc patients. Two other points deserve to be addressed. The low prevalence of arthralgia may be a consequence of limiting our search to the feet and hands. The high prevalence of anti DNA-topoisomerase is a well-established feature of our series 13, 14 and may relate to unestablished ethnic or

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Patients’ perspectives on systemic sclerosis-related Raynaud's phenomenon in the feet: A qualitative study from the OMERACT Foot and Ankle Working Group
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To explore, from patients’ perspectives, the symptoms and impact of Raynaud's phenomenon (RP) on the feet of patients with systemic sclerosis (SSc-RP), and to identify which foot-related domains are important to patients.
Forty participants (34 women) with SSc-RP took part in one of six focus groups held in the United Kingdom or United States. Participants were purposively sampled to ensure diversity in disease type, duration, and ethnicity. The topic guide included questions on RP impact, self-management, and treatment expectations. Qualitative content analysis was employed to identify key concepts in the data relating to foot-specific symptoms and their impact. Themes were organized by corresponding domains of potential importance.
Twenty-eight participants (70 %) reported experiencing RP in their feet. Five themes were identified corresponding to domains of potential importance: temperature changes, pain, cramping and stiffness, numbness, and color changes. These issues negatively affected participants’ lives, impairing walking, driving, and socializing, and causing issues with footwear and hosiery.
This large qualitative study exploring the experiences of patients with SSc-RP in the feet identified several key domains of high importance to patients. SSc-RP is common in the feet, presents in several patterns, and impacts multiple aspects of patients’ lives. These findings indicate where future foot-specific interventions for RP could be targeted. Findings from this study improve understanding of what domains are important to patients with SSc-RP affecting the feet and will contribute to the development of a core outcome set for foot and ankle disorders in rheumatic and musculoskeletal diseases.
Impact of foot functionality in patients with systemic sclerosis: Cross-sectional study
2024, Journal of Tissue Viability
Progressive systemic sclerosis or systemic scleroderma (SS) is a chronic and rare autoimmune disease that mainly affects the skin and various internal organs. Raynaud's phenomenon and digital ulcers are some of the symptoms that affect the foot, causing a decrease in the quality of life of patients. The objective of this study is to determine the functionality of the feet in patients with SS and determine the impact on their daily lives.
A sample of 165 patients (154 women, 11 men) diagnosed with SS with a mean age of 46.29 ± 11.36 years and a mean body mass index (BMI) of 24.90 ± 5.77 was recruited. Each participant completed the Foot Function Index (FFI) questionnaire and the Systemic Sclerosis Questionnaire (SySQ). A multivariate analysis was performed to determine which factors were related to a higher score in both questionnaires.
32.1% of the participants (n = 53) had claw toe deformities, 79.4% (n = 131) Raynaud's disease and 20% (n = 33) a history of foot ulcers. 51.5% of the participants (n = 85) presented symptoms in their nails, the most frequent sign being thickening, hardening and yellow coloration. The final score of the FFI questionnaire was 3.51 ± 2.41 (0–9.9), the pain subscale being the highest, with a score of 5.06 ± 2.75, followed by foot disability (3.26 ± 2.91) and difficulty performing activities (1.55 ± 2.22). The final score of the SySQ questionnaire was 0.95 ± 0.45 (0.18–2.45), and the subscales with the highest score were symptom frequency (1.30 ± 0.47), symptom intensity (1.11 ± 0.55), and general skill limitation (0.47 ± 0.51). A high correlation was observed between the final FFI score and the final SySQ score (r = 0.712; p=<0.001). Also, between foot activity limitation and general skill limitation (r = 0.658; p=<0.001). A moderate correlation was observed between foot pain score and overall symptom intensity (r = 0.482; p=<0.001). Also, between foot disability and overall symptom frequency (r = 0.556; p=<0.001). The multivariate analysis (R² 0.51) showed that the final FFI score had a significant relationship with the final SySQ score (p < 0.001). No significant correlation was found between age (p = 0.15), gender (p = 0.49), BMI (p = 0.74) or time of diagnosis (p = 0.57) and FFI.
SS is a disease that affects foot functionality in patients, with a greater impact on the pain scale. There is a correlation between the final FFI score and the final SySQ score, so improving foot functionality could help to improve the overall functionality of the patient with sclerosis.
Developing a core outcome set for foot and ankle disorders in rheumatic and musculoskeletal diseases: A scoping review and report from the OMERACT 2022 foot and ankle special interest group session
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Foot and ankle involvement is common in rheumatic and musculoskeletal diseases, yet high-quality evidence assessing the effectiveness of treatments for these disorders is lacking. The Outcome Measures in Rheumatology (OMERACT) Foot and Ankle Working Group is developing a core outcome set for use in clinical trials and longitudinal observational studies in this area.
A scoping review was performed to identify outcome domains in the existing literature. Clinical trials and observational studies comparing pharmacological, conservative or surgical interventions involving adult participants with any foot or ankle disorder in the following rheumatic and musculoskeletal diseases (RMDs) were eligible for inclusion: rheumatoid arthritis (RA), osteoarthritis (OA), spondyloarthropathies, crystal arthropathies and connective tissue diseases. Outcome domains were categorised according to the OMERACT Filter 2.1.
Outcome domains were extracted from 150 eligible studies. Most studies included participants with foot/ankle OA (63% of studies) or foot/ankle involvement in RA (29% of studies). Foot/ankle pain was the outcome domain most commonly measured (78% of studies), being the most frequently specified outcome domain across all RMDs. There was considerable heterogeneity in the other outcome domains measured, across core areas of manifestations (signs, symptoms, biomarkers), life impact, and societal/resource use. The group's progress to date, including findings from the scoping review, was presented and discussed during a virtual OMERACT Special Interest Group (SIG) in October 2022. During this meeting, feedback was sought amongst delegates regarding the scope of the core outcome set, and feedback was received on the next steps of the project, including focus group and Delphi methods.
Findings from the scoping review and feedback from the SIG will contribute to the development of a core outcome set for foot and ankle disorders in RMDs. The next steps are to determine which outcome domains are important to patients, followed by a Delphi exercise with key stakeholders to prioritise outcome domains.
Living with foot and ankle disorders in rheumatic and musculoskeletal diseases: A systematic review of qualitative studies to inform the work of the OMERACT Foot and Ankle Working Group
2023, Seminars in Arthritis and Rheumatism
This study aimed to determine outcome domains of importance to patients living with foot and ankle disorders in rheumatic and musculoskeletal diseases (RMDs), by exploring the symptoms and impact of these disorders reported in existing qualitative studies.
Six databases were searched from inception to March 2022. Studies were included if they used qualitative interview or focus group methods, were published in English, and involved participants living with RMDs (inflammatory arthritis, osteoarthritis, crystal arthropathies, connective tissue diseases, and musculoskeletal conditions in the absence of systemic disease) who had experienced foot and ankle problems. Quality was assessed using the Critical Appraisal Skills Programme qualitative tool and confidence in the findings was assessed using the Grading of Recommendations Assessment, Development and Evaluation Confidence in the Evidence from Reviews of Qualitative research (GRADE-CERQual) approach. All data from the results section of included studies were extracted, coded and synthesised to develop themes.
Of 1,443 records screened, 34 studies were included, with a total of 503 participants. Studies included participants with rheumatoid arthritis (n = 18), osteoarthritis (n = 5), gout (n = 3), psoriatic arthritis (n = 1), lupus (n = 1), posterior tibial tendon dysfunction (n = 1), plantar heel pain (n = 1), Achilles tendonitis (n = 1), and a mixed population (n = 3), who live with foot and ankle disorders. Seven descriptive themes were generated from the thematic synthesis: pain, change in appearance, activity limitations, social isolation, work disruption, financial burden and emotional impact. Descriptive themes were inductively analysed further to construct analytical themes relating to potential outcome domains of importance to patients. Foot or ankle pain was the predominant symptom experienced by patients across all RMDs explored in this review. Based on grading of the evidence, we had moderate confidence that most of the review findings represented the experiences of patients with foot and ankle disorders in RMDs.
Findings indicate that foot and ankle disorders impact on multiple areas of patients’ lives, and patients’ experiences are similar regardless of the RMD. This study will inform the development of a core domain set for future foot and ankle research and are also useful for clinicians, helping to focus clinical appointments and measurement of outcomes within clinical practice.
Characteristics of musculoskeletal ultrasound and its relationship with systemic inflammation in systemic sclerosis patients
2022, Egyptian Rheumatologist
Citation Excerpt :
In this study bone erosions were found in 36% of patients compared to other studies [15,16] that showed 40% of their patients had bone erosions. Avouac et al [22] and La Montagna et al [3], found that the prevalence of articular erosion in SSc had been estimated between 5 and 40%, however, Iagnocco et al [23], found that bone erosions were irrelevant abnormalities, while cortical irregularities may represent an interesting finding to be considered in the evaluation of SSc patients. The incidence of synovitis in this work was higher in diffuse than limited SSc which is in agreement with Avouac et al [22] who found that the frequency of synovitis was significantly higher in patients with the diffuse compared to the limited cutaneous subtype.
To describe musculoskeletal ultrasound (MSUS) findings in patients with systemic sclerosis (SSc) and to study their relation with cardiopulmonary and skin affection.
In 50 SSc patients, hands, wrists and six entheses sites were evaluated by MSUS. Entheses were scored using the Madrid Sonography Enthesitis Index (MASEI). The modified Rodnan skin score (mRSS) was assessed.
They were 42 females/8males with a mean age 36.2 ± 11.6 years, disease duration 3.5 ± 4.2 years, 27 had limited skin affection and 23 diffuse and the mRSS was 4.1 ± 15.3. 54% had arthritis, 10% tendon friction rub, 16% joint contractures and 10% calcinosis. 18% had left ventricular hypertrophy, 34% had pulmonary hypertension, 20% had restrictive pulmonary function test (PFT) and 24% had interstial lung disease (ILD). Synovitis was detected in 58% and was significantly related to mRSS, left ventricular hypertrophy, PFT, pulmonary hypertension and ILD (p = 0.023, p = 0.03, p = 0.006, p = 0.026 and p = 0.039 respectively). Common extensor tenosynovitis was detected in 82% and was significantly related to mRSS, PFT and ILD (p = 0.019, p = 0.043 and p = 0.032 respectively). Joint erosions were detected in 36% and were significantly related to age and C-reactive protein (CRP) (p = 0.01 and p = 0.034). Enthesopathy showed a significant relation with erythrocyte sedimentation rate (ESR), CRP and mRSS (p = 0.014, p = 0.013, p = 0.025, respectively).
Articular involvement is common in SSc and underestimated by clinical examination. Synovitis is associated with cardiovascular complication in SSc. Tenosynovitis is clinically related to restrictive pulmonary function. Enthesopathy should be kept in mind in symptomatic patients with systemic sclerosis.
Osteoarticular manifestations of systemic sclerosis: A systematic review of the literature
2020, Revista Colombiana de Reumatologia
Las manifestaciones osteoarticulares (MOA) están presentes de forma frecuente en pacientes con esclerosis sistémica (SSc). Generan gran discapacidad funcional con un impacto importante en la calidad de vida de los pacientes, por lo que requieren especial atención por parte de los clínicos.
Determinar la frecuencia de las MOA dentro de la SSc, su impacto en la condición general de los pacientes y las diferentes herramientas disponibles de diagnóstico y tratamiento.
Se realizó una revisión sistemática de la literatura disponible desde enero de 1970 hasta diciembre de 2018 en las bases de datos de Medline, Embase, Lilacs, Scielo, Cochrane y clinicaltrials.gov.
Se incluyeron un total de 116 artículos en esta revisión. Se determinaron el tipo y la prevalencia de las diferentes MOA reportadas en la literatura. Además de la evolución en los métodos diagnósticos de las mismas, cada vez existen más métodos validados para evaluar el impacto de las MOA en los pacientes con SSC. Se requieren ensayos clínicos aleatorizados que permitan establecer la mejor estrategia de tratamiento para estos pacientes.
Osteoarticular manifestations (OAM) are frequently present in patients with systemic sclerosis (SSc). These OAM are related to important functional disability and a severe impact on patient's life quality, therefore, they require special attention from clinicians.
Determining the frequency of reported OAM in SSc patients, the general impact of osteoarticular manifestations in patient's condition and the tools available for diagnosis and treatment.
A systematic review of the literature was performed for information published between January 1970 and December 2018 in the medical research databases of: Medline, Embase, Lilacs, Scielo, Cochrane and clinicaltrials.gov.
A total of 116 articles were included in this review. The type and prevalence of the different OAM reported in the literature were determined. In addition to the evolution of their diagnostic methods, there are more validated methods to assess the impact of OAM in patients with SSc. Randomized clinical trials are required to establish the best treatment strategy for these patients.

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^*: Giovanni La Montagna, MD: Assistant Professor of Rheumatology, Antonietta Baruffo, MD: Clinical Assistant in Rheumatology, Rosella Tirri, MD: Investigator in Rheumatology, Giovanni Buono, MD: Fellow in Rheumatology, Gabriele Valentini, MD: Professor of Rheumatology and Chief of Rheumatology Unit; Dipartimento di Internistica Clinica e Sperimentale “F. Magrassi”–Seconda Università degli Studi di Napoli, Naples Italy.

^**: Address reprint requests to Dr. G. La Montagna, Divisione di Reumatologia, Seconda Università degli Studi di Napoli, Via S. Pansini, 5-80131 Napoli, Italy. E-mail: [email protected]

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Foot involvement in systemic sclerosis: A longitudinal study of 100 patients*,**

Abstract

Section snippets

Patients and methods

Results

Discussion

Rheum Dis Clin North Am

Systemic sclerosis

Scleroderma of the foot: orthopaedic treatment

Foot Ankle

A case of systemic sclerosis in childhood complicated by severe digital ulcers

Pediatr Med Chir

Preliminary criteria for classification of systemic sclerosis (scleroderma)

Arthritis Rheum

Scleroderma (systemic sclerosis): classification, subsets and pathogenesis

J Rheumatol

Gastrointestinal involvement in systemic sclerosis. A radiographic study of fifty-five cases

Ital J Med

La patologia interstiziale polmonare nella sclerosi sistemica: caratteristiche semeiologiche con la tomografia computerizzata con alta risoluzione e confronto con altre metodiche

La Radiologia Medica

Scleroderma renal crisis. Analysis of prevalence and outcome in a large Italian series

J Clin Rheumatol