Rare complication of rheumatoid arthritis: Charcot Neuro-osteoarthropathy.

BACKGROUND: Rheumatoid arthritis (RA) is an autoimmune disease.However, there are few cases of Charcot Neuro-osteoarthropathy (CN) caused by rheumatoid diseases in clinical reports. It is not easy to pay attention to the diagnosis of CN in the complications of rheumatoid disease, which greatly increases the probability of misdiagnosis and missed diagnosis. This case reported a rare complication of rheumatoid arthritis, Charcot arthritis, and the molecular mechanism and diagnosis and treatment of CN caused by RA were systematically discussed. CASE PRESENTATION: The patient, a 79-year-old woman, was hospitalized due to bilateral shoulder pain, limited activity for half a year, aggravated for 4 months to the hospital. During this period, the symptoms did not improve after treatment with acupuncture and Chinese medicine. The patient was previously diagnosed with rheumatoid arthritis for more than 3 years and intermittent irregular use of methylprednisolone and methotrexate for 2 years. She had a history of osteoporosis. PHYSICAL EXAMINATION: symmetrical malformed swelling of the finger joints of both hands; Bilateral supraspinatus and deltoid muscle atrophy, tenderness at the acromion, and attachment of the long head tendon of the biceps brachii were observed. The left Dugas test and the right Dugas test were positive.Blood test: anti-cyclic citrullinated peptide antibody (A-CCP) 33.10U/ml (normal range: 0-5RU/ml); antinuclear antibody quantification (ANA) 47.40AU/ml (normal range: Negative or < 32); anti-double stranded DNA IgG antibody quantification (dsDNA) 31.00 IU/ml (normal range: 0-100 IU/ml); D-Dimer 6.43 µg/ml (normal range: 0-0.5 mg/L); erythrocyte sedimentation rate (ESR) was 27 mm/h (normal range: < 20 mm/60 min). C-reactive protein (CRP) 39.06 mg/L(0.068-8 mg/L).MRI 3.0 T enhancement of bilateral shoulder joints, cervical spine and thoracic spine showed: 1.Large bone destruction, cartilage injury, multiple effusion, synovitis, obvious on the right side. 2.Intervertebral disc degeneration, cervical 3/4, 4/5, 5/6, 6/7 disc herniation, with cervical 3/4 obvious, posterior central herniation; CONCLUSIONS: Rheumatoid arthritis complicated with Charcot's joint is rare. Clinically, patients with rheumatoid diseases should not ignore Charcot's joint complications because of rareness. Early blood inflammatory markers, neuro electrophysiology, and imaging MRI of rheumatoid CN are of great significance for the diagnosis of this mild or early neurovascular inflammation. Early diagnosis and treatment are helpful to prevent further joint injury. The clinical diagnosis, treatment, and molecular mechanism of osteolysis in RA and peripheral sensory nerve injury remain to be further revealed.

Camptodactyly-arthropathy-coxa vara-pericarditis syndrome and an unusual association with mitral stenosis.

BACKGROUND: Campotodactyly-artrhropathy-coxa vara-pericarditis (CACP) syndrome is a very rare autosomal recessive genetic disorder. It is characterized by flexion contracture of the fifth finger (camptodactyly); noninflammatory arthropathy; decreased angle between the shaft and the head of the femur (coxa vara) and pericarditis. Its association with mitral stenosis has not yet been reported. Hereby we report this unique association with CACP syndrome. CASE: An eleven-year-old girl presented with non-productive cough, dyspnea, and orthopnea. She was diagnosed CACP syndrome at the age of seven and a biallelic frameshift mutation in the PRG4 gene was determined. The physical examination revealed pectus excavatum, camptodactyly, genu valgum, tachypnea and orthopnea. The functional capacity was NYHA III-IV. She had 2/6 soft pansystolic murmur at 4th left intercostal space and a rumbling diastolic murmur at apex. Echocardiography revealed an enlarged left atrium, severe stenotic mitral valve with a mean diastolic transmitral gradient of 22.5 mmHg, mild mitral regurgitation and mild apical pericardial effusion. The patient had mitral comissurotomy and partial pericardiectomy operation. Her post-operative transmitral gradient decreased to 6.9 mmHg and the pulmonary pressure was 30 mmHg. Her functional capacity increased to NYHA I-II. CONCLUSIONS: The main defect is the proteoglycan 4 protein which acts like a lubricant in articular and visceral surfaces. Therefore, the leading clinical feature is arthropathy. Cardiac involvement other than clinically mild pericarditis is not usually expected. Three types of proteoglycans (decorin, biglycan, and versican) are present in the mitral valve. This could be the reason of mitral valve involvement in rare cases as like ours. It is important that these patients undergo echocardiographic examination regularly.

The Differentiation Between Infection and Acute Charcot.

The differentiation between acute Charcot neuroarthropathy and infection in the foot and ankle should be supported by multiple criteria. A detailed history and physical examination should always be completed. Plain radiographs should be performed, though advanced imaging, currently MRI, is more helpful in diagnosis. Scintigraphy and PET may become the standard imaging modalities once they are more clinically available due to their reported increased accuracy. Laboratory analysis can also act as a helpful diagnostic tool. Histopathology with culturing should be performed if osteomyelitis is suspected. The prompt diagnosis and initiation of treatment is vital to reducing patient morbidity and mortality.

Intraoperative Radiation Exposure During Midfoot Charcot Reconstruction.

While radiation exposure in foot and ankle surgery varies by procedure, attempts to minimize this hazard remain imperative to protect patients and surgeons. Hindfoot deformity correction employs significant radiation through intraoperative fluoroscopy, however, a paucity of data exists concerning Charcot reconstruction. This investigation describes and compares radiation exposure across varying Charcot pathology and fixation constructs. A retrospective chart review of patients undergoing midfoot Charcot reconstruction under large C-arm assistance from 2016-2022 was conducted. Demographics, pathology-specific, and intervention-specific variables were recorded and compared among midfoot reconstructions. The threshold for statistical significance was set at p ≤ .05. Among 40 patients, the average midfoot radiation exposure and fluoroscopy times were 9.5 ± 5.39 mGy and 256.64 ± 130.67 seconds, respectively. There existed no statistically significant difference in radiation exposure (p = .32) or fluoroscopy times (p = .71) among the different midfoot constructs. There existed a statistically significant relationship between radiation exposure with weight (p = .01) body mass index (p = .03) and number of stages (p = .04). Similarly, a relationship existed between fluoroscopy time with weight (p = .02), body mass index (p = .03), and number of beams/screws (p = .003). Due to the complexity of Charcot reconstruction coupled with multiple robust types of fixation, surgeons must remain cognizant of fluoroscopy usage. Moreover, providers who routinely perform Charcot reconstruction should wear personal protective equipment to protect against radiation.

Charcot neuroarthropathy in persons with diabetes: It's time for a paradigm shift in our thinking.

The aim of this paper is to review the recent literature regarding the epidemiology and surgical management of Charcot neuro-osteoarthropathy (CNO). We propose that a fundamental change in the approach and assumptions regarding the historical treatment of active CNO should be considered. Although the true incidence and prevalence of CNO in the US population with diabetes are not known, we estimated the incidence to be 27,602 per year and the prevalence to be 208,880 persons. In persons with diabetes, the incidence of CNO is higher than that of prostate, lung, kidney, and thyroid cancer, and in the entire US population, the incidence of CNO is higher than that of multiple myeloma, soft tissue sarcoma, and primary bone sarcoma. In persons with diabetes, the incidence of CNO is higher than fractures of the femoral shaft, distal femur, tibia, talus, calcaneus and Lisfranc ligament injuries. Surgical techniques have evolved over the past half century, and surgery is the standard for treating displaced fractures and intra-articular injuries. Since CNO is a fracture, dislocation, or fracture dislocation in patients with neuropathy, why do we treat CNO differently? Elsewhere in the skeleton displaced osseous and ligament injuries are treated surgically. Based on the information presented in this manuscript, we suggest that it is time for a paradigm shift in the treatment of persons with CNO. While uncommon, CNO in persons with diabetes is not rare. Given the advances in surgical techniques, surgical intervention should be considered earlier in persons with CNO who are at risk for developing deformity related foot ulceration.

Diagnosis and treatment of active charcot neuro-osteoarthropathy in persons with diabetes mellitus: A systematic review.

BACKGROUND: There are uncertainties regarding the diagnostic criteria, optimal treatment methods, interventions, monitoring and determination of remission of Charcot neuro-osteoarthropathy (CNO) of the foot and ankle in people with diabetes mellitus (DM). The aims of this systematic review are to investigate the evidence for the diagnosis and subsequent treatment, to clarify the objective methods for determining remission and to evaluate the evidence for the prevention of re-activation in people with CNO, DM and intact skin. METHODS: We performed a systematic review based on clinical questions in the following categories: Diagnosis, Treatment, Identification of Remission and Prevention of Re-Activation in people with CNO, DM and intact skin. Included controlled studies were assessed for methodological quality and key data from all studies were extracted. RESULTS: We identified 37 studies for inclusion in this systematic review. Fourteen retrospective and observational studies relevant to the diagnosis of active CNO with respect to clinical examination, imaging and blood laboratory tests in patients with DM and intact skin were included. We identified 18 studies relevant to the treatment of active CNO. These studies included those focused on offloading (total contact cast, removable/non-removable knee high devices), medical treatment and surgical treatment in the setting of active CNO. Five observational studies were identified regarding the identification of remission in patients who had been treated for active CNO. We did not identify any studies that met our inclusion criteria for the prevention of re-activation in patients with DM and intact skin who had been previously treated for active CNO and were in remission. CONCLUSIONS: There is a paucity of high-quality data on the diagnosis, treatment, and prognosis of active CNO in people with DM and intact skin. Further research is warranted to address the issues surrounding this complex disease.

Guidelines on the diagnosis and treatment of active Charcot neuro-osteoarthropathy in persons with diabetes mellitus (IWGDF 2023).

The International Working Group on the Diabetic Foot (IWGDF) has published evidence-based guidelines on the prevention and management of diabetic foot disease since 1999. This is the first guideline on the diagnosis and treatment of active Charcot neuro-osteoarthropathy in persons with diabetes published by the IWGDF. We followed the GRADE Methodology to devise clinical questions in the PACO (Population, Assessment, Comparison, Outcome) and PICO (Population, Intervention, Comparison, Outcome) format, conducted a systematic review of the medical literature, and developed recommendations with the rationale. The recommendations are based on the evidence from our systematic review, expert opinion when evidence was not available, and also taking into account weighing of the benefits and harms, patient preferences, feasibility and applicability, and costs related to an intervention. We here present the 2023 Guidelines on the diagnosis and treatment of active Charcot neuro-osteoarthropathy in persons with diabetes mellitus and also suggest key future topics of research.

Prevalence of Ankle Charcot Neuroarthropathy Presenting in a Tertiary Care Center.

Charcot neuroarthropathy's (CN) anatomic classification was originally formulated by the Brodsky article and the Trepman et al modification, including midfoot (type 1), rearfoot (type 2), ankle (type 3a), calcaneus (type 3b), multiarticular (type 4), and forefoot (type 5). In these classic studies, ankle joint and multijoint CN are reported as 9% and 6% to 9%, respectively, but we believe ankle CN to be more common than that in a tertiary setting. We retrospectively reviewed patients presenting initially or as referral between 2004 and 2020. Initial presentation radiographs were reviewed and classified by 3 authors based on Brodsky's model with Trepman and colleagues' modification, and any discrepancies were reviewed by the fourth author. A total of 175 patients (205 feet) were assessed. This revealed 80 cases classified as type 1 (39.0%), 23 cases type 2 (11.2%), 17 cases type 3a (8.3%), 2 cases type 3b (1.0%), and 83 cases type 4 (40.5%). After subdividing type 4, total prevalence included 150 with type 1 anatomic location (73.2%), 103 type 2 (50.2%), 44 type 3a (21.5%), and still 2 type 3b (1.0%). This study revealed a similar prevalence of isolated ankle CN (8.5%) compared to the Trepman et al article (9%), however, in total, ankle CN (21.5%) occurred 2.4-times more than the original 9%. Our study also found there to be a higher prevalence of ankle CN in the setting of multiarticular CN, which has not been evaluated in past studies. The prevalence of multiarticular CN was found to be 4.5-fold greater than the Trepman article (6%-9%).

Midfoot Fusion Using Superconstructs for the Charcot Foot: Current Techniques and Complications.

Neuroarthropathy of the foot and ankle presents a series of challenges. The treating physician faces a perfect storm of pathomechanics, deformity, and medical comorbidities. Successful treatment requires a systematic approach in diagnosis, nonsurgical management, surgical management, and long-term maintenance of the affected extremity. Nonsurgical care of the Charcot foot remains the mainstay of treatment and is successful in most cases. Surgery has become more accepted for patients with severe deformity. The concept of a superconstruct has been introduced to describe modern surgical techniques and implants that have been developed since the early 2000s where stability and durability are maximized. A superconstruct is defined by four factors: (1) fusion is extended beyond the zone of injury to bridge the area of bony dissolution; (2) aggressive bone resection is performed to allow for adequate reduction of deformity without undue tension on the soft-tissue envelope; (3) stronger implants are used than for nonneuropathic fusion procedures, including some specifically developed for fixation of the Charcot foot; and (4) the devices are applied in a position that maximizes mechanical stability to allow the implants to become load sharing. It is important to review the current techniques and implants used in fusion of the neuropathic midfoot and discuss the expected outcomes and complications based on the authors' experience.

Biomarkers for Early Detection of Charcot Arthropathy: A Prospective Study on Type 2 Diabetes Patients with Severe Neuropathy.

BACKGROUND: Charcot arthropathy (CA) is a progressive noninfectious inflammatory disease that causes irreversible destruction to pedal architecture in diabetic neuropathy (DN) patients. The debilitating prognosis demands early detection to prevent the development and progression of this disorder. Dysregulated and persistent production of inflammatory cytokines is reported as the key element in initiating osteoclastogenesis in CA. The study analyzed the potential association of markers of inflammation and bone turnover of prediagnostic serum samples on CA. METHODS: Seventy-one type 2 severe DN patients were selected based on inclusion-exclusion criteria. Serum samples of interleukin 6 (IL-6), osteoprotegerin (OPG), bone alkaline phosphatase (BALP), and C-reactive protein (CRP) were analyzed. These patients were followed for the development of symptoms of CA for 12 months. In the year of monitoring, 7 patients developed CA (group 1), whereas the remaining 64 patients did not develop CA (group 2). RESULTS: The rate of development of CA in patients with severe DN was 9.8%. In this group, significantly increased median values of HbA1c (group 2: 8.00 [7.00-9.00], group 1: 10.00 [9.25-11.50], P = .013); IL-6 (group 2: 1.21 [0.72-2.16], group 1: 11.08 [6.65-63.64], P = .008); and CRP (group 2: 1.25 [0.78-3.20], group 1: 3.31 [1.18-41.33], P = .041) were found. The receiver operating characteristic analysis showed that IL-6 was more strongly associated with the onset of CA (IL-6: area under the curve = 0.808; P = .008) than CRP. Cut-off values of ≥6.6 for IL-6 show potential to rule out CA in high-risk patients, with a positive predictive value of 26.1%, a negative predictive value of 97.9%, a sensitivity of 85.7%, and a specificity of 73.4%. CONCLUSION: In our study population, we found that an exacerbated inflammatory state, reflected by IL-6 values, generally occurred in DN patients before the clinical detection of CA. LEVEL OF EVIDENCE: Level II, prospective comparative study.

Charcot Foot Beaming with Subsequent Titanium Allergy: A Case Report.

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Management of the Hindfoot and Ankle in Charcot Arthropathy.

Neuropathic destabilization of the hindfoot and/or ankle is a significant complication for the patient with diabetes/neuropathy. The loss of ligamentous integrity and bony destruction results in a limb that is not stable for weight bearing. This loss of independence adds significant health risks to the patient. Management of this disease process is both time consuming and technically demanding for both the practitioner and the patient. Attention to detail and aggressive decision making is often necessary to salvage the limb. The goal for treatment is to produce a stable, weight-bearing limb that is shoeable and free from soft-tissue ulceration.

Management of Infections and Osteomyelitis in Patients With Charcot Foot Arthropathy.

There is growing interest in performing reconstruction of deformities associated with Charcot foot arthropathy. At least half of the patients undergoing this reconstruction will have chronic wounds and osteomyelitis overlying the deformity. It is important to provide orthopaedic surgeons with tools for making the diagnosis of osteomyelitis in this patient population and creating a strategy for treatment.

Charcot Arthropathy of the Digit: A Report of 2 Cases.

CASE: Charcot arthropathy (CA) is a progressive degenerative joint disease typically affecting lower extremity weight-bearing joints, with only a few cases reported in the fingers. We present 2 cases of interphalangeal joint CA: the long finger distal interphalangeal joint in a 73-year-old man with severe carpal tunnel syndrome and the ring finger proximal interphalangeal joint of a 71-year-old woman with diabetic neuropathy. CONCLUSION: Two cases of CA of the digits were treated with splinting with resolution of symptoms and no wound complications.

Early Diagnosis of Late-Onset Below-Level Neuropathic Pain in an 83 Year-Old Incomplete Tetraplegic Patient: A Charcot Spinal Neuro-Arthropathy Case Report.

BACKGROUND Charcot spine (CS), also called neuropathic arthropathy, appears to be triggered by damage to the nervous system (either central or peripheral) impairing proprioception and pain/temperature sensation in the vertebral column. Therefore, the defense mechanisms of altered joints lead to a progressive degeneration of the vertebral joint and surrounding ligaments, which can provoke major spinal instability. Beyond the sensory aspects, mechanic factors are identified as risk factors. While its etiology and pathophysiology remain contested, CS represents a rare and difficult pathology to diagnose at an early stage, owing to its nonspecific clinical symptoms. The diagnosis of CS is probably still underestimated and often occurs only quite late in the disease course. CASE REPORT An 83-year-old male patient who had a history of a post-traumatic tetraplegia was diagnosed with CS after 3 years, after describing a recent progressive worsening of neuropathic pain. The diagnosis was earlier than the majority of cases described in the literature. Indeed, in a recent review, the mean time lag between the onset of neurological impairment and the diagnosis of CS was 17.3±10.8 years. CONCLUSIONS This case report demonstrates the benefits of early diagnosis of CS when confronted by the clinical and radiological criteria. Therefore, it seems important to be able to evoke this neuropathic spinal arthropathy sufficiently in time to prevent its disabling consequences in patients with spinal cord injury, in terms of quality of life and independence.

[Progress in clinical diagnosis and treatment of diabetic Charcot neuroarthropathy of foot and ankle].

Objective: To summarize the progress of clinical diagnosis and treatment of diabetic Charcot neuroarthropathy (CNO) of foot and ankle to provide reference for clinical treatment. Methods: The research literature on diabetic CNO of foot and ankle at home and abroad was widely reviewed, and the stages and classification criteria of CNO were summarized, and the treatment methods at different stages of the disease course were summarized. Results: CNO is a rapidly destructive disease of bone and joint caused by peripheral neuropathy, which leads to the formation of local deformities and stress ulcers due to bone and joint destruction and protective sensory loss, which eventually leads to disability and even life-threatening. At present, the modified Eichenholtz stage is a commonly used staging criteria for CNO of foot and ankle, which is divided into 4 stages by clinical and imaging manifestations. The classification mainly adopts the modified Brodsky classification, which is divided into 6 types according to the anatomical structure. The treatment of diabetic CNO of foot and ankle needs to be considered in combination with disease stage, blood glucose, comorbidities, local soft tissue conditions, degree of bone and joint destruction, and whether ulcers and infections are present. Conservative treatment is mainly used in the active phase and surgery in the stable phase. Conclusion: The formulation of individualized and stepped treatment regimens can help improve the effectiveness of diabetic CNO of foot and ankle. However, there is still a lack of definitive clinical evidence to guide the treatment of active and stable phases, and further research is needed.

[Functional evaluation and quality of life in the follow-up of patients with Charcot arthropathy treated by ankle arthrodesis with a locked retrograde nail]. / Evaluación funcional y de calidad de vida en el seguimiento de pacientes con artropatía de Charcot tratados mediante artrodesis de tobillo con clavo centromedular retrógrado bloqueado.

INTRODUCTION: Charcot's arthropathy is a disabling non-infectious, progressive condition characterized by bony and articular destruction in patients with sensory neuropathy. In advanced cases with deformities and ankle instability, it requires a more invasive treatment as an ankle fusion with a retrograde locked intramedullary nail. It is unknown if, in these patients, the functional results of AOFAS (American Orthopaedic Foot and Ankle Society) correlate with the quality of life ones from the EuroQol-5D test and the possible postoperative complications. MATERIAL AND METHODS: the design is experimental, longitudinal prospective with ambispective data analysis (retrospective and prospective) to evaluate the functional results and life quality with a year of following patients with Charcot's arthropathy diagnosis treated with a retrograde locked nail from January 1, 2010, to November 1, 2018. RESULTS: this study resulted in complete consolidation from nine out of 11 cases, with a success rate of 81.2%, and only two cases (18.2%) developed nonunion. AOFAS and EuroQol-5D tests correlate positively in agreement with the Pearson correlation. CONCLUSIONS: AOFAS and EuroQol-5D correlate positively, having a 45 and 63% of acceptable and satisfactory results, respectively, for both tests.

INTRODUCCIÓN: la artropatía de Charcot es una condición incapacitante, no infecciosa, progresiva, que se caracteriza por destrucción ósea y articular en pacientes con neuropatía sensorial. En casos avanzados, en los que se tiene deformidad severa e inestabilidad de tobillo, se requieren procedimientos más invasivos como la artrodesis de tobillo con clavo centromedular retrógrado bloqueado. Se desconoce si en estos pacientes las puntuaciones de la valoración funcional postquirúrgica con la escala de AOFAS (American Orthopaedic Foot and Ankle Society) correlaciona con las puntuaciones de la escala de calidad de vida medida con el test EuroQol-5D y las posibles complicaciones postquirúrgicas. MATERIAL Y MÉTODOS: se trata de un ensayo autocontrolado de práctica clínica habitual, cuasiexperimental, longitudinal y prospectivo con recolección ambispectiva (retrospectiva y prospectiva) de datos para evaluar los resultados funcionales y de calidad de vida a un año de seguimiento de los pacientes con artropatía de Charcot tratados mediante artrodesis de tobillo con clavo centromedular retrógrado bloqueado del 1 de Enero de 2010 al 1 de Noviembre de 2018. RESULTADOS: la consolidación se logró en nueve casos de un total de 11 pacientes para una tasa de éxitos de 81.8% y únicamente dos casos (18.2%) en los cuales no se observó consolidación. Las escalas de AOFAS y EuroQol-5D se correlacionaron positivamente de acuerdo con la correlación de Pearson. CONCLUSIONES: las escalas de AOFAS y EuroQol-5D se correlacionan positivamente, obteniéndose 45 y 63% con resultados aceptables y satisfactorios respectivamente en ambas escalas.