Cumulative Glucocorticoid Dose Research Articles

Efficacy and tolerance of methotrexate in a real-life monocentric cohort of patients with giant cell arteritis

ObjectivesTo assess the indications, efficiency and tolerance profiles of methotrexate (MTX) in patients with giant cell arteritis (GCA) in a real-life setting. MethodsFrom a monocentric database of >500 GCA patients, we retrospectively selected 49 patients who received MTX between 2010 and 2020. Cumulative glucocorticoid (GC) doses, the number of relapses and GC-related adverse events were recorded before, during and after MTX. We separately analyzed the 3 main indications of MTX, i.e., disease relapse, GC-sparing strategy, and GCA presentation. ResultsWith a median follow-up of 84 [10–255] months, 25 (51%) and 18/41 (44%) patients relapsed during MTX treatment and after its discontinuation, respectively. Among the 40 patients who relapsed before MTX, 26 (65%) experienced a new relapse after MTX introduction. Once MTX was introduced, 24 (49%) patients were able to discontinue GC after 20.5 [7–64] months. No significant difference in cumulative GC doses were noted before and after MTX introduction with a total GC dose of 14.7 [1.05–69.4] grams. At the last follow-up, MTX was discontinued in 41 patients, including 13 (32%) due to clinicobiological remission, 12 (30%) due to treatment failure and 15 (36%) due to side effects. ConclusionOur real-life study showed a modest beneficial effect of MTX on relapse in patients with GCA. However, we did not observe any GC-sparing effect in this study. Other studies are needed to assess the GC-sparing effect in patients in whom GC management is adapted from recent recommendations.

High incidence of clinical fragility fractures in postmenopausal women with rheumatoid arthritis. A case-control study.

To estimate the incidence of clinical fragility fractures in postmenopausal women with rheumatoid arthritis (RA) and analyze risk factors for fracture. Incidence of clinical fragility fractures in 330 postmenopausal women with RA was compared to that of a control population of 660 age-matched postmenopausal Spanish women. Clinical fractures during the previous five years were recorded. We analyzed associations with risk factors for fracture in both populations and with disease-related variables in RA patients. Median age of RA patients was 64years; median RA duration was eight years. Sixty-nine percent were in remission or on low activity. Eighty-five percent had received glucocorticoids (GCs); 85%, methotrexate; and 40%, ≥1 biologic DMARD. Fifty-four patients and 47 controls had ≥1 major osteoporotic fracture (MOF). Incidence of MOFs was 3.55 per 100 patient-year in patients and 0.72 in controls (HR: 2.6). Risk factors for MOFs in RA patients were age, previous fracture, parental hip fracture, years since menopause, BMD, erosions, disease activity and disability, and cumulative dose of GCs. Previous fracture in RA patients was a strong risk for MOFs (HR: 10.37). Of every 100 postmenopausal Spanish women with RA, 3-4 have a MOF per year. This is more than double that of the general population. A previous fracture poses a high risk for a new fracture. Other classic risk factors for fracture, RA disease activity and disability, and the cumulative dose of GCs are associated with fracture development.

Generic and disease-adapted cardiovascular risk scores as predictors of atherosclerosis progression in SLE

ObjectiveStudies show that generic cardiovascular risk (CVR) prediction tools may underestimate CVR in SLE. We examined, for the first time to our knowledge, whether generic and disease-adapted CVR scores may...

Association between the cumulative dose of glucocorticoids before the development of pneumonia and death in patients receiving long-term glucocorticoids: a secondary analysis based on a Chinese cohort study.

The present study aimed to evaluate the association between the cumulative dose of glucocorticoids (GCs) and case fatality in hospitalized patients who developed pneumonia while receiving glucocorticoid therapy. This retrospective cohort study included 625 patients receiving long-term GC treatment who were hospitalized with pneumonia (322 male and 303 female). Data were obtained from the Dryad Digital Repository and were used to perform secondary analysis. Multivariable Cox proportional hazard regression model and restricted cubic splines (RCS) were used to evaluate the association between the cumulative dose of GCs and case fatality. Sensitivity analyses and subgroup analyses were performed. The 30-day and 90-day death rates were 22.9 and 26.2%, respectively. After adjusting for potential confounders, compared with those in the lowest quintile (≤ 1.5 g), the Cox proportional hazard regression model analysis showed that patients with different cumulative doses of GCs (1.5 to 2.95, 2.95 to 5, 5 to 11.5, and > 11.5 g) had lower risks for 30-day death, with respective hazard ratios of 0.86 (95% CI, 0.52 to 1.42), 0.81 (0.49 to 1.33), 0.29 (0.15 to 0.55), and 0.42 (0.22 to 0.79). The multivariable-adjusted RCS analysis suggested a statistically significant N-shaped association between the cumulative dose of GCs and 30-day death. A higher cumulative dose of GC tended to first lead to an increase in 30-day death within 1.8 g, then to a statistically significant decrease until around 8 g [HR for 1 g = 0.82 (0.69 to 0.97)], and again to an increase afterward. Similar results were found in the subgroup analyses and sensitivity analyses. N-shaped association between the cumulative dose of GCs and case fatality was observed in patients receiving long-term GC treatment who were hospitalized with pneumonia. Our findings may help physicians manage these patients.

Development of a web tool to calculate the cumulative dose of glucocorticoids

IntroductionGlucocorticoids are associated with serious side effects related to dosing and time of use. Unfortunately, there is no standard method for determining glucocorticoid exposure, especially in patients undergoing long-term treatment. ObjectiveThe aim of this work was to create a free and easy-to-use web application to calculate, in a systematic way, the total cumulative dose of corticosteroids. MethodsThe total cumulative dose is calculated as the sum of all periods of treatment with different doses of corticosteroids, and is expressed as the equivalent dose of prednisone in mg. Glucocorticoid doses during periods in which the available information is missing or incomplete are estimated by systematic assumptions. ResultsA simulation exercise using standard patterns of steroid use in polymyalgia rheumatica, and giant cell arteritis showed that even when the period of no information reached 50% of the time, the accuracy of the calculator had a mean absolute percentage error (MAPE)<7%. ConclusionThis tool simplifies and standardizes the glucocorticoids cumulative dose calculation, thereby minimizing bias in the assessment of glucocorticoid cumulative dose.

Development of a web tool to calculate the cumulative dose of glucocorticoids

Glucocorticoids are associated with serious side effects related to dosing and time of use. Unfortunately, there is no standard method for determining glucocorticoid exposure, especially in patients undergoing long-term treatment. The aim of this work was to create a free and easy-to-use web application to calculate, in a systematic way, the total cumulative dose of corticosteroids. The total cumulative dose is calculated as the sum of all periods of treatment with different doses of corticosteroids, and is expressed as the equivalent dose of prednisone in mg. Glucocorticoid doses during periods in which the available information is missing or incomplete are estimated by systematic assumptions. A simulation exercise using standard patterns of steroid use in polymyalgia rheumatica, and giant cell arteritis showed that even when the period of no information reached 50% of the time, the accuracy of the calculator had a mean absolute percentage error (MAPE) < 7%. This tool simplifies and standardizes the glucocorticoids cumulative dose calculation, thereby minimizing bias in the assessment of glucocorticoid cumulative dose. Los glucocorticoides se asocian con efectos secundarios graves, relacionados con dosis y tiempo de uso. Desafortunadamente, no existe un método estándar disponible para determinar el nivel de exposición a glucocorticoides en tratamientos prolongados. Crear una aplicación web gratuita y fácil de usar para calcular, de forma sistematizada, la dosis acumulada de glucocorticoides. La dosis acumulada se calcula como la suma de todos los períodos de tratamiento con diferentes dosis, y se expresa como la dosis equivalente de prednisona en mg. La dosis durante los períodos en los que la información no está disponible o está incompleta se estima mediante asunciones sistematizadas. Un ejercicio de simulación utilizando patrones estándar de uso de esteroides en la polimialgia reumática y la arteritis de células gigantes demostró que, incluso cuando el período de ausencia de información alcanzaba el 50% del tiempo, la precisión de la calculadora tenía un porcentaje de error medio absoluto (MAPE) < 7%. Esta herramienta simplifica y estandariza el cálculo de la dosis acumulativa de glucocorticoides, minimizando el sesgo del cálculo.

Herpes zoster in lupus nephritis: experience on 292 patients followed up for 15 years.

To evaluate the prevalence, incidence, and predictors of herpes zoster (HZ) development in lupus nephritis (LN). This retrospective study included 292 LN patients to determine HZ incidence during the last decades and its correlation with LN activity. LN patients with HZ were matched with LN patients without HZ in a 1:2 ratio based on sex, age, year of LN diagnosis, and LN histological class at kidney biopsy to assess HZ risk factors. Statistical tests included t-test, U-test, and Fisher's test. Univariate and multivariate logistic regression analyses were conducted to identify potential risk factors. HZ occurred after LN diagnosis in 66 patients (prevalence 22.6%) with an average of 8.7 years (range 0.2-28.4 years). Although with the potential limitations of the retrospective nature and the extensive duration of the study, the incidence of HZ was 15.6/1,000 person-years, increasing from 6.9 before 1980 to 16.0 in the 1990s and 43.9 after 2010. HZ onset was unrelated to LN activity. LN was active in 43% of cases and quiescent in the other 57% of cases at HZ diagnosis. The percentage of patients who developed lupus flares during the year after HZ (18.9%) was not different from that which occurred during the year before HZ (17.2%, p = 0.804). After excluding confounding factors through matching, the univariate analysis suggested that cyclosporin during induction therapy (p = 0.011) and higher cumulative doses of glucocorticoids (GCs; >50g, p = 0.004), cyclophosphamide (CYC; >5g, p = 0.001), and mycophenolate mofetil (MMF > 1,000 g, p = 0.007) predisposed patients to HZ. Univariate and multivariate analyses revealed a protective role of azathioprine (p = 0.008) and methylprednisolone pulses (p = 0.010) during induction therapy. HZ occurs unpredictably throughout the course of LN, underscoring the importance of continuous monitoring for these patients. In addition, the incidence of HZ seems to have increased in recent decades. Induction therapy with azathioprine and methylprednisolone pulses appears to provide protection, while higher cumulative doses of GCs, CYC, and MMF increase susceptibility.

Multicenter Case–Control Study of COVID-19–Associated Mucormycosis Outbreak, India

Abstract We performed a case–control study across 25 hospitals in India for the period of January–June 2021 to evaluate the reasons for an COVID-19–associated mucormycosis (CAM) outbreak. We investigated whether COVID-19 treatment practices (glucocorticoids, zinc, tocilizumab, and others) were associated with CAM. We included 1,733 cases of CAM and 3,911 age-matched COVID-19 controls. We found cumulative glucocorticoid dose (odds ratio [OR] 1.006, 95% CI 1.004–1.007) and zinc supplementation (OR 2.76, 95% CI 2.24–3.40), along with elevated C-reactive protein (OR 1.004, 95% CI 1.002–1.006), host factors (renal transplantation [OR 7.58, 95% CI 3.31–17.40], diabetes mellitus [OR 6.72, 95% CI 5.45–8.28], diabetic ketoacidosis during COVID-19 [OR 4.41, 95% CI 2.03–9.60]), and rural residence (OR 2.88, 95% CI 2.12–3.79), significantly associated with CAM. Mortality rate at 12 weeks was 32.2% (473/1,471). We emphasize the judicious use of COVID-19 therapies and optimal glycemic control to prevent CAM.

Avascular necrosis of the epiphyses of bones in patients who have had SARS-CoV-2 infection: Clinical observations and a narrative review of literature data

The SARS-CoV-2 coronavirus pandemic is a leading medical problem that is in the focus of attention of representatives of all medical specialties. In addition to fighting the COVID-19 infection itself, the task of preventing and treating a wide range of complications arising after the disease is becoming increasingly urgent. One of these complications is avascular necrosis (AN) of bone tissue – a severe pathology that leads to serious suffering, a decrease in the quality of life and disability of patients. For the period from 2020 to 2022 there are 9 reviews in the world literature devoted to the pathogenesis, clinical features and treatment possibilities of this complication. During the same period, 5 articles were published describing clinical observations of AN after suffering COVID-19.The purpose of this work is to demonstrate our own clinical observations, as well as to review the available literature data on the problem of AN after COVID-19 infection. Based on the analysis, it can be concluded that AN after SARS-CoV-2 infection most often develops in the femoral head (&gt;50% of cases), occurs regardless of the severity of the disease and the cumulative dose of glucocorticoids used in the acute period of the disease. It seems advisable to perform an MRI of the hip joints at least once every 3 months for all patients who have had COVID-19 in severe and moderate form during the first year after convalescence.

Individual patient data meta-analysis on continued use of glucocorticoids after their initiation as bridging therapy in patients with rheumatoid arthritis

ObjectivesTo investigate whether patients with rheumatoid arthritis (RA) can discontinue glucocorticoids (GC) after GC ‘bridging’ in the initial treatment step and to identify factors that may affect this.MethodsData from 7...

Performance of leflunomide as a steroid-sparing agent in giant cell arteritis: A single-center, open-label study.

The management of giant cell arteritis (GCA) remains challenging and many patients require prolonged glucocorticoid treatment due to high disease relapse rates. We aimed to evaluate the role of leflunomide as a steroid-sparing agent in GCA. This prospective open-label study included patients diagnosed with GCA between July 2014 and August 2020 and followed them for 96 weeks. At the time of diagnosis all patients received treatment following a predefined glucocorticoid regimen. At week 12 of follow-up, 10 mg of leflunomide per day was recommended as an adjunctive therapy. The decision to start with leflunomide treatment was patient-dependent. Follow-up visits were performed adhering to a predetermined protocol. The number of relapses, the cumulative glucocorticoid dose and treatment-related adverse events were recorded and compared between glucocorticoid-only and leflunomide groups. Of the 215 GCA patients [67.6% female, median (IQR) age 74 (66-79) years], 151 (70.2%) received leflunomide at week 12 (leflunomide group); the others continued with glucocorticoids (glucocorticoid-only group). During the study 64/215 (29.8%) patients relapsed. Of the 51 patients who relapsed after 12 weeks, 22/151 patients (14.6%) and 29/64 patients (45.3%) were in the leflunomide and glucocorticoid-only group, respectively (p = 0.001; NNT 3.3 for leflunomide). Furthermore, 80/151 patients in the leflunomide group managed to stop glucocorticoids at week 48 [with relapses in 6/80 patients (7.5%)]. The cumulative glucocorticoid dose was lower in the leflunomide group (p = 0.009). In our cohort, leflunomide safely and effectively reduced the GCA relapse rate and demonstrated a steroid-sparing effect in over three quarters of patients.

Phenotypes of body composition in patients with systemic sclerosis

Background. Systemic sclerosis (SSc) is an autoimmune inflammatory disease of connective tissue with a polysyndromic clinical picture. One of the factors that significantly affect the quality of life of such patients is the involvement of the musculoskeletal system. A small number of studies were aimed at studying osteoporosis and body composition in patients with SSc, however, the clinical heterogeneity of the disease, a small number of patients included in the studies do not allow to draw unambiguous conclusions about the frequency of various phenotypes of body composition, their relationship and factors affecting their occurrence.Aim. To study the body composition and to establish the frequency of isolated and combined pathological phenotypes in women with SSс and to determine the factors associated with the muscle mass.Materials and methods. 85 postmenopausal women (46 with SSc, 39 without rheumatic pathology) were included. Questionnaires, anthropometric measurements, assessment of nutritional status by MNA, dual-energy X-ray absorptiometry of the whole body, lumbar spine, femoral neck and total hip were carried out. Correlation and univariate linear regression analyses were performed to identify factors related to appendicular muscle mass.Results. Normal body composition was found in 13.0% of patients with SSс and 25.6% — in the control, isolated and combined pathological phenotypes were detected in 34.8% and 52.2% women with SSс and in 61.5% and 12.8% in the group without rheumatic diseases, respectively. Combined pathological phenotypes, phenotypes with the presence of sarcopenia and/or osteoporosis were significantly more often in SSc patients (p&lt; 0.001), but the frequency of obesity did not differ (69.6% and 64.1%, respectively). Negative correlations were revealed between the value of appendicular muscle mass and the presence of osteolysis of the nail phalanges (Rs=-0.23), the cumulative dose of glucocorticoids (GC) (Rs=-0.43) and direct — with the value of the femoral neck BMD (Rs=0.47), nutritional status by MNA (Rs=0.51), BMI (Rs=0.70), the circumference of the shoulder of the non-dominant arm (Rs=0.68), waist (Rs=0.66), hips (Rs=0.72) and lower leg of the non-dominant leg (Rs=0.81), p&lt; 0.05. Univariate linear analysis confirmed the presence of positive associations between the appendicular muscle mass and proximal hip BMD, BMI, shoulder circumferences, waist, hips and lower leg (p&lt; 0.001), and negative associations with the Rodnan skin score (p=0.012) and the cumulative dose of GC (p=0.001).Conclusion. 87% of patients with SSc had pathological phenotypes of body composition, among them combined phenotypes were significantly more common than in people without rheumatic diseases. BMD in the proximal hip, BMI, circumference of the shoulder, waist, hips and lower leg were positively, and the Rodnan skin score and cumulative dose of GC were negatively associated with the appendicular muscle mass.

Off-label use of mycophenolate mofetil in immune-mediated diseases

Mycophenolate mofetil (MMF) is a largely used immunosuppressive agent in the prevention of transplant rejection and lupus nephritis. Its use has been extended to other immune-mediated diseases (ID). To assess the off-label use of MMF, its performance as a glucocorticoid sparing agent, the therapeutic response, and its adverse effects. A retrospective study was performed. One hundred-seven patients aged 58 ± 16 years (83% females) who received MMF for ID in off label uses between 2016 and 2018 were included. The study variables were cause of MMF indication, sex, age, use as a first- or second-line treatment and maintenance dosing. The cumulative doses of glucocorticoids six months before and after MMF indication were compared. MMF was used as a second-line therapy in 66 patients (62%). The mean maintenance dose of MMF was 1,500 ± 540 mg/day. Prednisone cumulative doses were 3,908 ± 2,173 and 1,672 ± 1,083 milligrams six months before and six months after starting MMF, respectively (p < 0.01). Adverse effects were identified in 21 (20%) cases, none of them serious. Mycophenolate has a favorable response profile as a second line immunosuppressive agent. It is effective as a glucocorticoid sparing drug. The safety profile is also favorable as adverse effects were scanty and mild.

Advantages of an alternate-day glucocorticoid treatment strategy for the treatment of IgG4-related disease: A preliminary retrospective cohort study.

Alternate-day glucocorticoid (GC) therapy is a treatment option that can reduce GC-associated adverse events. We investigated the safety and efficacy of alternate-day GC therapy in patients with immunoglobulin G4-related disease (IgG4-RD).Medical records of patients with IgG4-RD who were followed for at least one year at St. Luke’s International Hospital, Tokyo, Japan, from 2004 to 2020 were reviewed. Patients who fulfilled comprehensive IgG4-RD diagnostic criteria were divided into alternate-day or daily GC treatment groups based on their treatment protocol. The effect of alternate-day GC therapy on glucocorticoid toxicity index (GTI) score was evaluated using multilinear analysis with adjustments for cumulative GC doses until each assessment point and propensity scores (PS) for alternate-day GC therapy. Kaplan–Meier curves and Cox proportional hazard models were used to assess the efficacy of alternate-day GC therapy for disease control.Among the 67 patients with IgG4-RD, patients with alternate-day (n = 13) and daily (n = 31) GC treatments were analyzed after excluding 23 ineligible patients. The median (interquartile range) age was 64 (60–70) years, 29 (65.9%) were male patients, 26 (59.1%) patients had positive biopsy results, and the median follow-up period was 1643 days. Significantly more patients with alternate-day GC treatment used concomitant immunosuppressants (11 [84.6%] vs 11 [35.5%]; P = .007). The alternate-day strategy significantly lowered the GTI score after adjusting for cumulative GC dose until the assessment and PS (adjusted coefficient: −29.5 [−54.3, −4.8], P = .021 at 12 months; −20.0 [−39.8, −0.1], P = .049 at 24 months). Serious infections were numerically less frequent in the alternate-day group (incidence ratio [95% confidence interval [CI]: 0.45 [0.05, 3.63], P = .45). Most patients (92.3%) in the alternate-day GC treatment group and all patients in the daily GC treatment group showed treatment responses in the remission induction therapy. The PS-adjusted hazard ratio of alternate-day GC treatment for disease flares was not significant (1.55 [0.53, 4.51]; P = .43).The alternate-day treatment strategy significantly reduced GC-related adverse events regardless of the cumulative GC dose. Alternate-day GC treatment is a feasible option for patients with IgG4-RD, without a significant increase in disease flares particularly when combined with immunosuppressants.

Bone remodeling serum markers in children with systemic lupus erythematosus

IndroductionSLE is an autoimmune multisystem disease. Glucocorticoid is an irreplaceable medication for SLE. Glucocorticoid and inflammatory mediators impact bone remodeling by OPG/RANKL/RANK signal system, which could lead to osteoporosis. Our aim is to detect the expression of RANKL/OPG in children with SLE, and to preliminarily explore the changes of bone remodeling serum markers in children with SLE.MethodsSerum RANKL and OPG of 40 children with SLE and healthy children were detected by ELISA, while 25(OH)VitD3 was detected routinely. Clinical data of children with SLE were recorded, including gender, age, height, weight, BMI, SLEDAI, duration of the disease, cumulative dose of glucocorticoid, and correlation analysis was conducted with RANKL, OPG and 25(OH)VitD3.ResultsSerum RANKL concentrations in SLE group were significantly higher than health group (9.82 ± 7.20 vs. 6.80 ± 4.35 pg/ml and 0.081 ± 0.072 vs. 0.042 ± 0.034, P < 0.05) respectively, and the concentrations of OPG and 25(OH)VitD3 in serum were significantly lower than health group (156.34 ± 57.33 vs. 189.16 ± 68.70 pg/ml and 43.66 ± 31.27 vs. 59.04 ± 21.56 mmol/L, P < 0.05). Serum RANKL in children with SLE was positively correlated with the duration of SLE, cumulative dose of GC(r = 0.593, 0.727, P < 0.05). And it was negatively correlated with serum OPG and 25(OH)VitD3 (r = -0.601, -0.469, P < 0.05). In addition, serum OPG and 25(OH)VitD3 concentrations were inversely correlated with cumulative dose of GC (r = -0.66, -0.508, P < 0.05).ConclusionLow levels of vitamin D3 and bone metabolic abnormalities still persist in children with SLE even if the disease is in remission, while serum RANKL level was elevated, OPG expression was reduced. In the case of disease remission, GC is involved in the occurrence and development of abnormal bone remodeling through RANKL/OPG.

Association of nesfatin-1 levels and bone remodeling markers in patients with rheumatoid arthritis

Aim. To evaluate the association of nesfatin-1 (NF-1) levels and bone remodeling markers in patients with rheumatoid arthritis (RA).Materials and methods. 110 patients (105 women and 5 men) with RA, classified by ACR/EULAR 2010 criteria were enrolled in our study. All patients undergone complete clinical and laboratory examination. NF-1 serum levels were determined using a commercial test-systems (RayBio® Nesfatin Enzyme Immunoassay [EIA] Kit). Statistical analysis was performed using a software package Statistica 12.0. The results were considered statistically significant at p &lt; 0.050.Results. Significant positive correlation was determined between serum NF-1 levels and Intact N-Terminal Propeptide of Type 1 Procollagen (P1NP) levels (r = 0.218; p = 0.022). Serum NF-1 levels were not associated with bone mineral density in lumbar spine and femur. Serum NF-1 levels didn't correlate with body composition indices and Beta-CrossLaps levels. Patients with osteoporosis (n = 53) and osteoporotic fractures (n = 25) had statistically significant higher levels of NF-1 (Z = –2.060; p = 0.040 и Z = –2.370; p = 0.017 respectively). However, serum NF-1 levels didn’t correlate with cumulative dose of glucocorticoids (ρ = 0.090; p = 0.368) and the duration of glucocorticoids intake (ρ = 0.070; p = 0.462).Conclusions. In our study, we determined the significant positive correlation between NF-1 and P1NP levels, supposingly due to NF-1 possible effect on the osteoblasts differentiation and function. Patients with osteoporosis and osteoporotic fractures had higher median blood serum levels of NF-1.

The frequency of sarcopenia and factors affecting appendicular muscle mass in patients with systemic sclerosis

Aim. To determine the frequency of sarcopenia (SP) and to identify factors associated with the muscle mass in women with systemic sclerosis (SSc).Materials and methods. The study included 64 women with SSc aged 40–70 years. Questionnaires, clinical, instrumental, laboratory examinations and absorptiometry. Linear regression analysis was performed to identify factors associat ed with appendicular muscle mass (AMM).Results. Probable SP was detected in 35 (54.7 %), and confirmed SP – 17 (26.5 %) women with SSc. The frequency of SP did not differ depending on the form of the disease. Univariate linear analysis revealed the relationship between the AMM and BMI, nutritional status; mid-upper arm, waist, hip and calf circumferences, skin Rodnan score, cumulative dose of glucocorticoids (GC) and BMD of the proximal hip. Multivariate linear analysis confirmed the presence of associations between the AMM index and BMI (b = 0.65; p &lt;0.001), the Rodnan skin score (b = –0.19; p = 0.047), the cumulative dose of GC (b = –0.22; p = 0.021).Conclusion. The study demonstrated that more than a quarter of patients with SSc had a confirmed SP. Although age is the main risk factor for SP in the general population, in our study it did not differ between patients with low and normal AMM. The cumulative dose of GC and the Rodnan skin score were negatively, and BMI was positively associated with the value of AMМ.

Clinical Study on Systemic Lupus Erythematosus Complicated with Knee Bone Infarction.

Objective The present study aims to (1) analyze the clinical characteristics and related influencing factors of knee bone infarction in systemic lupus erythematosus (SLE) and (2) improve the understanding of SLE complicated with knee bone infarction. Methods The data of patients with SLE complicated with knee bone infarction were retrospectively analysed; patients with SLE during the same period who matched in age, gender, and disease duration were selected as control subjects, with a 1 : 1 ratio with the SLE group. The clinical data were collected to analyze the risk factors for SLE complicated with knee bone infarction. Results In a total of 36 (6.4%) of 563 patients aged 19–33 (25.8 ± 4.8) years who had SLE during the same period, the disease was complicated with knee bone infarction. The diagnosis of knee bone infarction was made at an SLE duration of 7–65 (26.2 ± 15.7) months. During the SLE course, knee bone infarction occurred within 1 year in 6 cases (16.7%), within 1–5 years in 28 cases (77.8%), and in >5 years in 2 cases (5.6%). Raynaud's phenomenon incidence and anti-nRNP antibody positivity were significantly higher in the knee bone infarction group than in the control group (P < 0.01 and P < 0.05, respectively). The cumulative glucocorticoid dose at 1, 3, and 6 months was significantly higher in the knee bone infarction group than in the control group (P < 0.05). SLE complicated with knee necrosis had a statistically significant rank correlation with Raynaud's phenomenon (r = 0.445, P < 0.001), anti-nRNP antibody (r = 0.309, P=0.008), and renal injury (r = 0.252, P=0.032). The multivariate analysis of SLE complicated with knee bone infarction showed that Raynaud's phenomenon was an independent influencing factor for the complicated knee bone infarction in SLE patients (OR = 4.938, P=0.004), and the probability of SLE complicated with knee bone infarction in Raynaud's phenomenon positive patients was 4.938 times that of Raynaud's phenomenon negative patients. Conclusions The risk of knee bone infarction was relatively high in patients with SLE within a 5-year disease course and in young patients. The risk factors were Raynaud's phenomenon, anti-nRNP antibody positivity, and early high-dose glucocorticoid therapy.

Factors affecting bone mineral density in children and adolescents with secondary osteoporosis.

This study aimed to investigate the clinical factors associated with bone mineral density (BMD) among children and adolescents with osteoporosis secondary to treatment for underlying clinical conditions. We retrospectively reviewed the medical records of patients aged 10-18 years and evaluated them for lumbar spine BMD (LSBMD) after treatment for underlying diseases, including hemato-oncologic, rheumatologic system, and inflammator y bowel diseases. LSBMD measured by dual-energy x-ray absorptiometry (DXA) performed from March 2019 to March 2021 was evaluated. We analyzed 117 patients who underwent initial DXA after treatment for underlying diseases. Subjects in this study had multiple underlying diseases: hemato-oncologic (78.6%), rheumatologic (11.1%), and inflammatory bowel diseases (10.3%). There was no significant association between the z-score and bone metabolic markers (P&gt;0.05). However, higher cumulative glucocorticoid (GC) dose significantly reduced LSBMD z-score (P=0.029). Moreover, the association between cumulative dose of GC and initial z-score of LSBMD was significant in logarithmic regression analysis (P=0.003, R2=0.149). GC accumulation was a significant risk factor for vertebral fracture when the initial BMD was evaluated after treatment (P=0.043). Bone metabolic markers did not significantly influence the risk of vertebral fracture. Initial bone mass density of the lumbar spine evaluated after long-term GC use for underlying diseases is a predictor of further vertebral fractures.

Retroperitoneal fibrosis - diagnosis and treatment.

Idiopathic retroperitoneal fibrosis (IRF) is a rare condition characterized by the development of a peri-aortic and peri-iliac tissue showing chronic inflammatory infiltrates and pronounced fibrosis. Ureteral entrapment with consequent obstructive uropathy is one of the most common complications which can lead to acute renal failure and, in the long term, to varying degrees of chronic kidney disease. Common symptoms at onset include lower back, abdominal or flank pain. Pain is frequently referred to the hip, to the groin and to the lateral regions of the leg, often with nocturnal exacerbations and not responding to position changes. The disease is commonly associated with signs of systemic inflammatory response (malaise, fever, and anorexia and weight loss). Glucocorticoids are considered the cornerstone of the therapy. The use of other immunosuppressive agents, including cyclophosphamide, azathioprine, methotrexate, mycophenolate mofetil and biological agents such as rituximab, tocilizumab and infliximab have been reported as a valuable option mostly in case reports, cases series and small studies. These agents allowed to reduce cumulative dose of glucocorticoids and their adverse effects. Combined therapy is preferable for all patients who suffer from significant glucocorticoid- related toxicity or in cases where glucocorticoids alone are insufficient to treat the condition.