ABSTRACT
OBJECTIVES: To evaluate if the timing of appearance with respect to disease onset may influence the arthritis presentation pattern in antisynthetase syndrome (ASSD). METHODS: The patients were selected from a retrospective large international cohort of ASSD patients regularly followed-up in centres referring to AENEAS collaborative group. Patients were eligible if they had an antisynthetase antibody testing positive in at least two determinations along with arthritis occurring either at ASSD onset (Group 1) or during the course of the disease (Group 2). RESULTS: 445 (70%; 334 females, 110 males, 1 transsexual) out of the 636 ASSD we collected had arthritis, in the majority of cases (367, 83%) from disease onset (Group 1). Patients belonging to Group 1 with respect to Group 2 had an arthritis more commonly polyarticular and symmetrical (p=0.015), IgM-Rheumatoid factor positive (p=0.035), erosions at hands and feet plain x-rays (p=0.036) and more commonly satisfying the 1987 revised classification criteria for rheumatoid arthritis (RA) (p=0.004). Features such as Raynaud's phenomenon, mechanic's hands and fever (e.g. accompanying findings) were more frequently reported in Group 2 (p=0.005). CONCLUSIONS: In ASSD, the timing of appearance with respect to disease onset influences arthritis characteristics. In particular, RA features are more common when arthritis occurs from ASSD onset, suggesting an overlap between RA and ASSD in these patients. When arthritis appears during the follow-up, it is very close to a connective tissue disease-related arthritis. Also, the different prevalence of accompanying features between these two groups is in line with this possibility.
Subject(s)
Arthritis/epidemiology , Myositis/epidemiology , Adult , Arthritis/diagnosis , Arthritis/immunology , Autoantibodies/blood , Biomarkers/blood , Europe/epidemiology , Female , Humans , Male , Mexico/epidemiology , Middle Aged , Myositis/diagnosis , Myositis/immunology , Phenotype , Prevalence , Prognosis , Retrospective Studies , Risk Factors , Time FactorsABSTRACT
To evaluate the efficacy of current treatments for the Raynaud phenomenon (RP) in patients with systemic sclerosis (SSc), a systematic literature search was performed using Medline, EMBASE, and Cochrane Central Register of Controlled Trials (from 1961 to October 2011). We included meta-analyses, systematic reviews, clinical trials, and high-quality cohort studies published in English or Spanish. Patient populations had to include adults diagnosed with limited cutaneous or diffuse SSc who had associated RP and/or digital ulcers under pharmacological treatment. Efficacy of treatments was evaluated based on: number of RP episodes, RP severity, episode-free time, ulcer improvement/healing, and appearance of new ulcers. We used the Jadad scale of methodological quality to evaluate the quality of randomized clinical trials, and the 2009 Oxford Centre for Evidence-Based Medicine classification for other studies. Of a total of 1617 studies identified, only 27 fulfilled inclusion criteria. Drugs received the following grade recommendations: Grade A for nifedipine, nicardipine, quinapril, IV iloprost, bosentan, tadalafil, and MQx-503; Grade B for beraprost, cicaprost, DMSO, cyclofenil, and atorvastatin; and Grade C for misoprostol, prazosin, OPC-2826, enalapril, sildenafil, antioxidant, and stanazolol. Calcium channel blockers, prostanoids, tadalafil, and bosentan received the highest recommendation level for their effectiveness. However, most systematic reviews reviewed just a handful of studies with small sample sizes and short follow-ups. Our review shows that the existing evidence on the efficacy of RP treatment in SSc patients is inconclusive which calls for further research, especially in the form of prospective studies of high quality with long-term follow-ups.
Subject(s)
Raynaud Disease/drug therapy , Scleroderma, Systemic/complications , Skin Ulcer/drug therapy , Vasodilator Agents/therapeutic use , Humans , Raynaud Disease/complications , Skin Ulcer/complications , Treatment OutcomeABSTRACT
Arthritis associated with Behçet's disease is typically nonerosive and nondeforming, and most patients respond to colchicine treatment. However, destructive arthritis and refractory arthritis have also been reported on occasion. Elevated white blood cell counts may occur in synovial fluid in BD, but pseudoseptic arthritis is a very rare event in this disease. We report a patient with BD and rapidly progressive deforming pseudoseptic arthritis refractory to colchicine, corticosteroids, and methotrexate, who entered remission after infliximab treatment.
Subject(s)
Antibodies, Monoclonal/therapeutic use , Antirheumatic Agents/therapeutic use , Arthritis/drug therapy , Behcet Syndrome/complications , Adult , Arthritis/complications , Humans , Infliximab , Male , Treatment OutcomeABSTRACT
Objective: To compare organ involvement and disease severity between male and female patients with juvenile onset systemic sclerosis. Methods: Demographics, organ involvement, laboratory evaluation, patient-reported outcomes and physician assessment variables were compared between male and female juvenile onset systemic sclerosis patients enrolled in the prospective international juvenile systemic sclerosis cohort at their baseline visit and after 12 months. Results: One hundred and seventy-five juvenile onset systemic sclerosis patients were evaluated, 142 females and 33 males. Race, age of onset, disease duration, and disease subtypes (70% diffuse cutaneous) were similar between males and females. Active digital ulceration, very low body mass index, and tendon friction rubs were significantly more frequent in males. Physician global assessment of disease severity and digital ulcer activity was significantly higher in males. Composite pulmonary involvement was also more frequent in males, though not statistically significantly. After 12 months, they are the pattern of differences changed female patients had significantly more frequent pulmonary involvement. Conclusion: In this cohort, juvenile onset systemic sclerosis had a more severe course in males at baseline and but the pattern changed after 12 months. Some differences from adult findings persisted, there is no increased signal of pulmonary arterial hypertension or heart failure in male pediatric patients. While monitoring protocols of organ involvement in juvenile onset systemic sclerosis need to be identical for males and females.
ABSTRACT
OBJECTIVE: Utilizing data obtained from a prospective, international, juvenile systemic sclerosis (SSc) cohort, the present study was undertaken to determine if pulmonary screening with forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLco) is sufficient to assess the presence of interstitial lung disease (ILD) in comparison to high-resolution computed tomography (HRCT) in juvenile SSc. METHODS: The juvenile SSc cohort database was queried for patients enrolled from January 2008 to January 2020 with recorded pulmonary function tests (PFTs) parameters and HRCT to determine the discriminatory properties of PFT parameters, FVC, and DLco in detecting ILD. RESULTS: Eighty-six juvenile SSc patients had both computed tomography imaging and FVC values for direct comparison. Using findings on HRCT as the standard measure of ILD presence, the sensitivity of FVC in detecting ILD in juvenile SSc was only 40%, the specificity was 77%, and area under the curve (AUC) was 0.58. Fifty-eight juvenile SSc patients had both CT imaging and DLco values for comparison. The sensitivity of DLco in detecting ILD was 76%, the specificity was 70%, and AUC was 0.73. CONCLUSION: The performance of PFTs in juvenile SSc to detect underlying ILD was quite limited. Specifically, the FVC, which is one of the main clinical parameters in adult SSc to detect and monitor ILD, would miss ~60% of children who had ILD changes on their accompanying HRCT. The DLco was more sensitive in detecting potential abnormalities on HRCT, but with less specificity than the FVC. These results support the use of HRCT in tandem with PFTs for the screening of ILD in juvenile SSc.
Subject(s)
Lung Diseases, Interstitial/complications , Scleroderma, Systemic/complications , Adolescent , Child , Female , Humans , Lung Diseases, Interstitial/diagnostic imaging , Lung Diseases, Interstitial/pathology , Male , Missed Diagnosis , Prospective Studies , ROC Curve , Tomography, X-Ray Computed , Vital CapacityABSTRACT
OBJECTIVE: To evaluate the baseline clinical characteristics of juvenile systemic sclerosis (SSc) patients in the international juvenile SSc inception cohort, and to compare these characteristics between the classically defined juvenile diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc) subtypes and among those with overlap features. METHODS: A cross-sectional study was performed using baseline visit data. Information on demographic characteristics, organ system evaluation, treatment, and patient- and physician-reported outcomes was extracted and summary statistics applied. Comparisons between juvenile dcSSc and lcSSc subtypes and patients with and without overlap features were performed using chi-square and Mann-Whitney U tests. RESULTS: At data extraction, 150 juvenile SSc patients were enrolled across 42 centers; 83% were White, 80% were female, juvenile dcSSc predominated (72%), and 17% of the cohort had overlap features. Significant differences were found between juvenile dcSSc and juvenile lcSSc regarding modified Rodnan skin thickness score, the presence of Gottron's papules, digital tip ulceration, results of the 6-minute walk test, and composite pulmonary and cardiac involvement. All of these were more frequent in dcSSc except for cardiac involvement. Juvenile dcSSc patients had significantly worse scores for physician-rated disease activity and damage. A significantly higher occurrence of Gottron's papules and musculoskeletal and composite pulmonary involvement, and a significantly lower frequency of Raynaud's phenomenon, were seen in those with overlap features. CONCLUSION: Results from a large international juvenile SSc cohort demonstrate significant differences between juvenile dcSSc and juvenile lcSSc patients, including more globally severe disease and increased frequency of interstitial lung disease in juvenile dcSSc patients, while those with lcSSc have more frequent cardiac involvement. Those with overlap features had an unexpected higher frequency of interstitial lung disease.
Subject(s)
Lung Diseases, Interstitial , Scleroderma, Diffuse , Scleroderma, Systemic , Skin Ulcer , Cross-Sectional Studies , Female , Humans , Male , Scleroderma, Diffuse/diagnosis , Scleroderma, Localized , Scleroderma, Systemic/diagnosis , Scleroderma, Systemic/epidemiologyABSTRACT
BACKGROUND: Fluoroquinolones have been associated with increased risk of tendinopathy and Achilles tendon rupture (ATR), especially in patients over 60 years of age. METHODS: A retrospective study was carried out including patients over 60 years of age with ATR attended in our centre over the period 2000-2017. RESULTS: We identified 44 patients with RTA, of whom 18% (8/44) had been previously treated with fluoroquinolones, with a mean age at diagnosis of ATR of 77.37 years and concomitant corticotherapy in 4 of them. In 7patients, the rupture was spontaneous and all required surgical management. A significantly higher frequency of smoking, concomitant corticotherapy and spontaneous ruptures were found in the group treated with fluoroquinolones. CONCLUSIONS: ATR is an adverse event that can occur in patients over 60 years of age treated with fluoroquinolones, so an adequate risk-benefit assessment should be carried out in this population, especially in the presence of associated risk factors.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/adverse effects , Carboplatin/adverse effects , Glutamates/adverse effects , Guanine/analogs & derivatives , Lupus Erythematosus, Cutaneous/chemically induced , Neoplasms, Multiple Primary/drug therapy , Adenocarcinoma/diagnosis , Adenocarcinoma/drug therapy , Aged , Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Biopsy, Needle , Carboplatin/administration & dosage , Carcinoma, Renal Cell/diagnosis , Carcinoma, Renal Cell/drug therapy , Female , Follow-Up Studies , Glutamates/administration & dosage , Guanine/administration & dosage , Guanine/adverse effects , Humans , Immunohistochemistry , Kidney Neoplasms/diagnosis , Kidney Neoplasms/drug therapy , Lung Neoplasms/diagnosis , Lung Neoplasms/drug therapy , Lupus Erythematosus, Cutaneous/pathology , Neoplasms, Multiple Primary/pathology , Pemetrexed , Risk AssessmentSubject(s)
Hyperpigmentation/diagnosis , Nail Diseases/diagnosis , Nails/pathology , Scleroderma, Systemic/diagnosis , Skin Diseases/diagnosis , Biopsy , Cell Proliferation , Comorbidity , Female , Fingers , Humans , Hyperpigmentation/pathology , Hyperpigmentation/physiopathology , Melanocytes/pathology , Middle Aged , Nail Diseases/pathology , Nail Diseases/physiopathology , Scleroderma, Systemic/pathology , Scleroderma, Systemic/physiopathology , Skin Diseases/pathology , Skin Diseases/physiopathologyABSTRACT
Paraneoplastic polyarthritis is an inflammatory arthritis, is usually seronegative, and has a temporal and pathophysiological relationship with an underlying malignancy. Although head and neck tumors may be a cause of paraneoplastic polyarthritis, its association with tongue carcinoma has not been previously reported. We present the case of a 69-year-old man who was a former smoker and presented with polyarthritis since 2 months in the wrists, proximal interphalangeal joints, knees, and elbows, with increased levels of acute-phase reactants; negativity for rheumatoid factor, anticitrullinated cyclic peptide antibody, and antinuclear antibody; and negative results for crystals and microorganisms in the synovial fluid. Cervical computed tomography and posterior rhinoscopy were performed, which detected an asymptomatic lesion on the base of the tongue, whose biopsy was compatible with nonkeratinizing squamous cell carcinoma. Polyarthritis did not respond to glucocorticoids at medium doses (oral prednisone 20 mg/day) but progressively resolved after the initiation of antineoplastic therapy.
ABSTRACT
Introduction: Juvenile systemic sclerosis is an orphan disease. Currently, the majority of juvenile systemic sclerosis cohort studies are retrospective in design without standardized assessment. This study was conducted prospectively to investigate the difference in manifestations of limited cutaneous juvenile systemic sclerosis and diffuse cutaneous juvenile systemic sclerosis subtypes. An additional aim was to compare these data to other juvenile systemic sclerosis cohorts and a large adult systemic sclerosis cohort. Methods: Patients fulfilling the Paediatric Rheumatology European Society juvenile systemic sclerosis classification criteria were included. Clinical characteristics and patient-related outcomes were assessed. Results: In all, 88 patients with a mean disease duration of 3.5 years were enrolled, 72.5% with diffuse cutaneous juvenile systemic sclerosis with a mean modified Rodnan Skin score of 18 and 27.5% with limited cutaneous juvenile systemic sclerosis with mean modified Rodnan Skin score of 9. The mean age at the onset of Raynaud's and first non-Raynaud's symptoms was similar in both groups, approximately 9 and 10.5 years. Active digital tip ulcerations were present in 29% diffuse cutaneous juvenile systemic sclerosis and none in the limited cutaneous juvenile systemic sclerosis subjects (p = 0.005). Of those with cardiopulmonary testing, 3% of diffuse cutaneous juvenile systemic sclerosis and 23% of limited cutaneous juvenile systemic sclerosis group had cardiac involvement (p = 0.015), and 41% diffuse cutaneous juvenile systemic sclerosis and 22% of the limited cutaneous juvenile systemic sclerosis group had pulmonary involvement (p = 0.009). Physician global disease damage assessment was higher in the diffuse cutaneous juvenile systemic sclerosis group compared to the limited cutaneous juvenile systemic sclerosis group: 35 and 15 (p = 0.021). Discussion: The majority of this international juvenile systemic sclerosis cohort had diffuse cutaneous juvenile systemic sclerosis (72.5%) with more frequent vascular and pulmonary involvement compared to the limited cutaneous group, who had increased cardiac involvement. Our cohort reflects prior findings of published juvenile systemic sclerosis cohorts and emphasizes a difference in the presentation compared to adult-onset systemic sclerosis.
ABSTRACT
BACKGROUND: Biologic therapy has changed the prognosis of patients with juvenile idiopathic arthritis (JIA). The aim of this study was to examine the pattern of use, drug survival, and adverse events of biologics in patients with JIA during the period from diagnosis to adulthood. METHODS: All patients included in BIOBADASER (Spanish Registry for Adverse Events of Biological Therapy in Rheumatic Diseases), a multicenter prospective registry, diagnosed with JIA between 2000 and 2015 were analyzed. Proportions, means, and SDs were used to describe the population. Incidence rates and 95% CIs were calculated to assess adverse events. Kaplan-Meier analysis was used to compare the drug survival rates. RESULTS: A total of 469 patients (46.1% women) were included. Their mean age at diagnosis was 9.4 ± 5.3 years. Their mean age at biologic treatment initiation was 23.9 ± 13.9 years. The pattern of use of biologics during their pediatric years showed a linear increase from 24% in 2000 to 65% in 2014. Biologic withdrawal for disease remission was higher in patients who initiated use biologics prior to 16 years of age than in those who were older (25.7% vs 7.9%, p < 0.0001). Serious adverse events had a total incidence rate of 41.4 (35.2-48.7) of 1000 patient-years. Patients younger than 16 years old showed significantly increased infections (p < 0.001). CONCLUSIONS: Survival and suspension by remission of biologics were higher when these compounds were initiated in patients with JIA who had not yet reached 16 years of age. The incidence rate of serious adverse events in pediatric vs adult patients with JIA treated with biologics was similar; however, a significant increase of infection was observed in patients under 16 years old.
Subject(s)
Antirheumatic Agents/adverse effects , Arthritis, Juvenile/drug therapy , Arthritis, Juvenile/epidemiology , Biological Therapy/adverse effects , Drug-Related Side Effects and Adverse Reactions/epidemiology , Registries , Adolescent , Adult , Antirheumatic Agents/therapeutic use , Arthritis, Juvenile/diagnosis , Biological Therapy/methods , Child , Drug-Related Side Effects and Adverse Reactions/diagnosis , Humans , Middle Aged , Prospective Studies , Spain/epidemiology , Treatment OutcomeABSTRACT
OBJECTIVE: Arthritis, myositis and interstitial lung disease (ILD) constitute the classic clinical triad of anti-synthetase syndrome (ASSD). These patients experience other accompanying features, such as Raynaud's phenomenon, fever or mechanic's hands. Most ASSD patients develop the complete triad during the follow-up. In the present study we aimed to determine whether the subsequent appearance of accompanying features may suggest the development of triad findings lacking at the onset in anti-Jo1 positive ASSD patients. METHODS: Anti-Jo1 positive patients presenting with incomplete ASSD (no >2 classic triad features) were assessed. Clinical characteristics and clusters of disease manifestations were retrospectively collected and analyzed in a large international multicenter cohort of ASSD patients. RESULTS: 165 patients (123 women) with incomplete ASSD were identified. Ninety-five patients (57.5%) developed new classic triad manifestations after 15months median (IQR 9-51) and 40 (24%) developed new accompanying features after 19months median (IQR 6-56) from disease onset. During the follow-up, the ex-novo occurrence of triad features was observed in 32 out of 40 patients (80%) with new accompanying findings and in 63 out of 125 patients (50.5%) without new accompanying findings (p=0.002). In patients with at least one new accompanying feature the odds ratio for the occurrence of new triad manifestations was 3.94 with respect to patients not developing ex-novo accompanying findings (95% CI 1.68-9.21, p=0.002). CONCLUSION: Anti-Jo1 ASSD patients with incomplete forms at disease onset are at high risk for the subsequent occurrence of lacking classic triad findings. Although all ASSD patients should be carefully assessed for the occurrence of new triad features, a closer follow-up should be considered in the subgroup of patients developing ex novo accompanying findings. These patients, indeed, have near four-fold increased risk for new classic triad manifestation occurrence with respect to patients not presenting ex novo accompanying findings.
Subject(s)
Ligases/antagonists & inhibitors , Raynaud Disease/metabolism , Autoantibodies , Female , Follow-Up Studies , Humans , Male , Middle Aged , Retrospective Studies , SyndromeABSTRACT
Anti-Jo-1 is the most frequently detectable antibody in the antisynthetase syndrome (ASSD), an autoimmune disease characterized by the occurrence of arthritis, myositis, and interstitial lung disease (ILD). Recently, we organized an international collaborative group called American and European NEtwork of Antisynthetase Syndrome (AENEAS) for the study of this rare and fascinating disease. The group collected and published one of the largest series of ASSD patients ever described and with one of the longer follow-up ever reported. The number of participating centers is steadily increasing, as well as the available cohort. In the first paper, we showed that arthritis, myositis, and ILD may be frequently the only feature at disease onset, raising problems to reach a correct diagnosis of this syndrome. Nevertheless, we first observed that the ex novo appearance of further manifestations is common during the follow-up, strengthening the importance of a correct diagnosis. In our cohort, the 24 % of the 243 patients up to now collected had isolated arthritis as a presenting feature. These patients represent the most intriguing group in terms of differential diagnosis and clinical time course. Furthermore, data on this aspect are scanty, the reason that lead us to evaluate these aspects in our cohort of patients, reviewing also available literature. In fact, the most relevant aspect is that ASSD is rarely suspected in this setting of patients, in particular in case of poliarticular involvement, positive rheumatoid factor (RF), or anti-cyclic citrullinated peptide antibodies (ACPA) or evidence of joint erosions at plain radiographs. These findings were not rare in our cohort, and they have been also described in other series. Furthermore, manifestations such as Raynaud's phenomenon, mechanic's hands, and fever that may lead to the suspect of ASSD are observed only in a third of cases. If we consider the high rate of clinical picture progression in these patients, we feel that ASSD should be carefully considered in all patients presenting with isolated arthritis, even in those with erosive, RF, and ACPA-positive arthritis.
Subject(s)
Antibodies, Antinuclear/blood , Arthritis/immunology , Autoantibodies/immunology , Myositis/immunology , Antibodies, Antinuclear/immunology , Autoantibodies/blood , Histidine-tRNA Ligase/immunology , Humans , Myositis/blood , Myositis/complicationsABSTRACT
Introducción: Las fluoroquinolonas han sido asociadas con aumento del riesgo de tendinopatía y rotura del tendón de Aquiles (RTA), especialmente en pacientes mayores de 60 años. Métodos: Se llevó a cabo un estudio retrospectivo en el que se incluyó a los pacientes mayores de 60 años con RTA atendidos en nuestro centro durante el período 2000-2017. Resultados: Se identificó a 44 pacientes con RTA, de los cuales 18% (8/44) habían sido tratados previamente con fluoroquinolonas, con una media de edad al diagnóstico de RTA de 77,37 años y corticoterapia concomitante en 4 de ellos. En 7 pacientes la rotura fue espontánea y todas requirieron tratamiento quirúrgico. Se encontró una frecuencia significativamente mayor de tabaquismo, corticoterapia concomitante y roturas espontáneas en el grupo tratado con fluoroquinolonas. Conclusiones: La RTA es un evento adverso que puede ocurrir en pacientes mayores de 60 años tratados con fluoroquinolonas, por lo que debería realizarse una adecuada evaluación relación riesgo-beneficio en esta población, especialmente en presencia de factores de riesgo asociados.(AU)
Background: Fluoroquinolones have been associated with increased risk of tendinopathy and Achilles tendon rupture (ATR), especially in patients over 60 years of age.Methods: A retrospective study was carried out including patients over 60 years of age with ATR attended in our centre over the period 2000-2017. Results: We identified 44 patients with RTA, of whom 18% (8/44) had been previously treated with fluoroquinolones, with a mean age at diagnosis of ATR of 77.37 years and concomitant corticotherapy in 4 of them. In 7patients, the rupture was spontaneous and all required surgical management. A significantly higher frequency of smoking, concomitant corticotherapy and spontaneous ruptures were found in the group treated with fluoroquinolones. Conclusions: ATR is an adverse event that can occur in patients over 60 years of age treated with fluoroquinolones, so an adequate risk-benefit assessment should be carried out in this population, especially in the presence of associated risk factors.(AU)
Subject(s)
Humans , Male , Female , Middle Aged , Achilles Tendon , Rupture , Fluoroquinolones , Tendinopathy , Levofloxacin , Ciprofloxacin , Retrospective Studies , Rheumatology , Rheumatic DiseasesABSTRACT
RESUMEN La osteomalacia oncogénica es un síndrome metabólico paraneoplásico caracterizado por hipofosfatemia debida a la pérdida renal de fosfato, con nivel bajo de vitamina D. Este trastorno está asociado con la liberación de factores fosfatúricos por células tumorales, especialmente el factor de crecimiento fibrolástico 23 (FGF23). Las neoplasias relacionadas con la osteomalacia oncogénica suelen ser tumores pequeños de linaje mesenquimatoso y pueden ser difíciles de localizar en algunos casos debido a su tamaño y ubicación poco accesible al examen físico. Presentamos a un paciente que desarrolló fracturas vertebrales y de cadera debido a osteomalacia oncogénica asociada con un tumor mesenquimatoso fosfatúrico del tejido graso profundo de la planta del pie, que finalmente se diagnosticó después de 3 años del inicio de los síntomas, cuando el tumor pudo ser localizado por el rastreo gammagráfico óseo con pentatreótido marcado con indio-111 y por las imágenes de resonancia magnética nuclear.
ABSTRACT Oncogenic osteomalacia is a paraneoplastic metabolic syndrome characterised by a low phosphates in the blood due to renal phosphate losses with inadequately normal or low vitamin D levels. This disorder is associated with the release of tumour cell-secreted phosphaturic factor, most notably fibroblast growth factor 23 (FGF-23). The neoplasms related to oncogenic osteomalacia are usually small tumours of mesenchymal lineage, and they may be difficult to locate in the physical examination in some cases, due to their size and inaccessible location. The case is presented of a patient who developed vertebral and hip fractures due to oncogenic osteomalacia associated with a phosphaturic mesenchymal tumour of the deep fat tissue in the sole of the foot. This was finally diagnosed after 3 years of the onset of symptoms after being located by bone scintigraphy with Indium-111 labelled pentetreotide and magnetic resonance imaging.
Subject(s)
Humans , Male , Middle Aged , Osteomalacia , Neoplasms , Vitamin D , Hypophosphatemia , Fractures, BoneSubject(s)
Lunate Bone , Osteonecrosis/complications , Scleroderma, Systemic/complications , Aged , Female , Humans , Middle Aged , Retrospective StudiesABSTRACT
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