Prevalence and incidence of systemic lupus erythematosus in Tucumán, Argentina

Lupus ◽  
2020 ◽  
Vol 29 (13) ◽  
pp. 1815-1820
Author(s):  
Luciana Gonzalez Lucero ◽  
Ana L Barbaglia ◽  
Verónica I Bellomio ◽  
Maria C Bertolaccini ◽  
Maximiliano A Machado Escobar ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Private Practice ◽  
Annual Incidence ◽  
Public Hospitals ◽  
Age At Diagnosis ◽  
Systemic Lupus ◽  
The Mean

The aim of this study was to determine the prevalence and incidence of Systemic Lupus Erythematosus (SLE) in Tucumán, Argentina. Methods: The study included inpatient and outpatient charts from four public hospitals and private practice rheumatology clinics, all of them members of the Tucumán Rheumatology Society. Patients older than 16 years with diagnosis of SLE between January 2005 and December 2012 were included. Prevalence and annual incidence were calculated as the number of cases per 100.000 inhabitants during the period 2005 to 2012. Results: Three hundred fifty-three patients were identified. The mean age at diagnosis was 30.5 ± 11.7 years, 93.5% women, 83% mestizos. Prevalence was 24.3 cases/100.000 inhabitants (CI 95% 22.6–28.8) and age-adjusted (≥16 years) of 34.9 cases/100.000 inhabitants (CI 95% 32.8–41.1). The annual incidence in 2005 was 1.8 cases/100.000 inhabitants (95% CI 1–2.9) and 2012 of 4.2 cases/100.000 inhabitants (95% CI 2.9–5.8). Mortality was 9.1%, with infections being the most frequent cause (14/32). Conclusion: The prevalence of SLE in the province of Tucumán was 34.9 cases/100.000 inhabitants.

Health-Related Quality of Life and fatigue are associated with a higher work productivity impairment in systemic lupus erythematosus patients: Data from the Almenara Lupus Cohort

Lupus ◽  
2021 ◽  
pp. 096120332110524
Author(s):  
Cristina Reategui-Sokolova ◽  
Rocío Violeta Gamboa-Cárdenas ◽  
Mariela Medina ◽  
Francisco Zevallos-Miranda ◽  
Paola Alejandra Zeña-Huancas ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Emotional Health ◽  
Damage Index ◽  
Work Productivity ◽  
Age At Diagnosis ◽  
Systemic Lupus ◽  
Work Impairment ◽  
Factors Associated ◽  
The Mean

Objectives: This study aims to determine the factors associated with absenteeism, presenteeism, and overall work impairment in patients with systemic lupus erythematosus (SLE). Methods: A total of 133 consecutive working patients with SLE were assessed between October 2017 and December 2018, using a standardized data collection form. Sociodemographic, disease, and work-related variables were collected. Work productivity and activity impairment (WPAI) was assessed with the respective questionnaire; absenteeism and presenteeism due to overall health and symptoms during the past 7 days were scored. Linear regression models were performed to determine the factors associated with absenteeism, presenteeism, and overall work impairment. Potential factors included were age at diagnosis, gender, socioeconomic status, educational level, SLEDAI, SLICC/ACR damage index (SDI), FACIT-Fatigue, and the domains of the LupusQoL Results: The mean age at diagnosis was 32.2 years (11.8); 121 (91.7%) were female. Nearly all patients were Mestizo. The mean percent of time for absenteeism was 5.0 (12.9), it was 28.5 (26.4) for presenteeism, and it was 31.3 (27.2) for overall work impairment. In the multiple regression analysis, factors associated with absenteeism were disease duration (B = −0.34; SE = 0.12; p = 0.007), pain (B = −0.14; SE = 0.06; p = 0.046), intimate relationship (B = −0.07; SE = 0.03; p = 0.046), and emotional health (B = 0.16; SE = 0.06; p = 0.006); factors associated with presenteeism were physical health (B = −0.43; SE = 0.14; p = 0.002) and FACIT (B = −0.87; SE = 0.30; p = 0.005); and factors associated with overall work impairment were pain (B = −0.40; SE = 0.11; p = 0.001) and FACIT-Fatigue (B = −0.74; SE = 0.28; p = 0.010). Conclusion: A poor HRQoL and higher levels of fatigue were associated with a higher percentage of absenteeism, presenteeism, and overall work impairment in SLE patients.

scholarly journals Unique Characteristics of Prepubertal Onset Systemic Lupus Erythematosus

2019 ◽  
Vol 2019 ◽  
pp. 1-7 ◽  
Author(s):  
R. Abdwani ◽  
E. Abdalla ◽  
I. Al-Zakwani
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Statistical Tests ◽  
Disease Onset ◽  
Age At Diagnosis ◽  
Systemic Lupus ◽  
Cutaneous Manifestations ◽  
Pubertal Onset ◽  
The Mean

Objectives. The aim of this study was to investigate the influence of age at disease onset on disease expression and outcomes of pediatric systemic lupus erythematosus SLE (pSLE). Methods. A total of 103 patients with pSLE from Sultan Qaboos University Hospital, Oman, were retrospectively studied. Epidemiological, clinical phenotype, disease severity, serology, treatment, and outcome were compared among the three groups using univariate statistical tests. Results. The mean disease duration of the cohort was 9.8 ± 4.7 years. The patients were divided into three groups: prepubertal onset (n=39) with mean age at diagnosis of 5.1 ± 2.0 years and pubertal disease onset (n=29) with mean age at diagnosis of 10.8 ± 1.0 years as well as postpubertal disease onset (n=35) group with mean age at diagnosis of 15.3 ± 1.6 years. The prepubertal pSLE cohort demonstrates unique characteristics with increased frequency of familial SLE (61%) of which 49% were from first-degree relatives. Similarly, this group had distinctive clinical features, which included increased renal disease in pubertal and postpubertal groups, respectively (51% vs 23% vs 20%; p=0.039). Prepubertal, similar to pubertal group, had a higher incidence of cutaneous manifestations than in the postpubertal group (74% vs 69% vs 46%; p=0.029). Laboratory features in prepubertal group were distinct with increased frequency of positive anti-cardiolipin antibodies (47%), anti-glycoprotein antibodies (42%), ANCA (62%), and low complement levels (97%) compared to pubertal and postpubertal group. The prepubertal group also has the lowest frequency of positive SSA antibodies (18%) and SSB antibodies (5.1%). The overall mean SLEDAI score in pSLE cohort was 15.6 ± 18.5. The mean SLEDAI scores among the groups showed no significance difference (p=0.110). The overall SLICC DI ≥1 was 36% with a mean damage score of 0.76 ± 1.38. No significant differences in damage index (SLICC DI ≥1) were noted among the groups. Conclusions. Distinct clinical features were identified in prepubertal onset pSLE population of Arab ethnicity. Given the high rate of consanguineous marriage and high frequency of familial SLE in this cohort, these manifestations could be explained by higher frequency of genetic factors that influence the disease pathogenesis.

Age at diagnosis and health-related quality of life are associated with fatigue in systemic lupus erythematosus patients: Data from the Almenara Lupus Cohort

Lupus ◽  
2020 ◽  
Vol 29 (12) ◽  
pp. 1644-1649
Author(s):  
Claudia Elera-Fitzcarrald ◽  
Cristina Reátegui-Sokolova ◽  
Rocío V Gamboa-Cárdenas ◽  
Mariela Medina ◽  
Francisco Zevallos ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Emotional Health ◽  
Age At Diagnosis ◽  
Systemic Lupus ◽  
Related Quality ◽  
Health Related ◽  
Multivariable Analyses ◽  
The Mean

Objective To define the factors associated with fatigue in Mestizo patients with Systemic Lupus Erythematosus (SLE). Methods This is a cross-sectional study of SLE patients from a single center cohort. Visits were performed every six months. For these analyses, the first visit between October 2017 and December 2018 was included. Demographic and clinical characteristics as well as treatment were recorded at every visit. Fatigue was ascertained with the Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-FT), Health-Related Quality of Life (HRQoL) with the LupusQoL, disease activity with the Systemic Lupus Erythematosus Disease Activity Index –2 K (SLEDAI-2K), and damage with the Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology damage index (SDI). Prednisone use was recorded as current daily dose. Immunosuppressive drugs and antimalarial use were recorded as current, past or never. Univariable and multivariable analyses were performed using linear regression models. For the multivariable analyses, model selection followed a backward elimination procedure. Results Two hundred and twenty-six patients were evaluated. The mean (SD) age at diagnosis was 35.6 (13.1) years, 211 (93.4%) were female; and disease duration was 11.0 (7.3) years. The mean SLEDAI and SDI were 2.4 (3.5) and 1.3 (1.5), respectively. The mean FACIT-FT was 33.1 (10.8). On the multivariable analysis, age at diagnosis and some domains of HRQoL (physical health, emotional health and fatigue) remained associated. Conclusions Age at diagnosis is negatively associated with fatigue whereas HRQoL domains like physical health, emotional health and fatigue are positively associated with fatigue.

scholarly journals Methyl donor micronutrients, CD40-ligand methylation and disease activity in systemic lupus erythematosus: A cross-sectional association study

Lupus ◽  
2021 ◽  
pp. 096120332110345
Author(s):  
Stefan Vordenbäumen ◽  
Alexander Sokolowski ◽  
Anna Rosenbaum ◽  
Claudia Gebhard ◽  
Johanna Raithel ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Dna Methylation ◽  
T Cells ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Cd40 Ligand ◽  
Systemic Lupus ◽  
Methyl Donors ◽  
Methyl Donor ◽  
The Mean

Objective Hypomethylation of CD40-ligand (CD40L) in T-cells is associated with increased disease activity in systemic lupus erythematosus (SLE). We therefore investigated possible associations of dietary methyl donors and products with CD40L methylation status in SLE. Methods Food frequency questionnaires were employed to calculate methyl donor micronutrients in 61 female SLE patients (age 45.7 ± 12.0 years, disease duration 16.2 ± 8.4 years) and compared to methylation levels of previously identified key DNA methylation sites (CpG17 and CpG22) within CD40L promotor of T-cells using quantitative DNA methylation analysis on the EpiTYPER mass spectrometry platform. Disease activity was assessed by SLE Disease Activity Index (SLEDAI). Linear regression modelling was used. P values were adjusted according to Benjamini & Hochberg. Results Amongst the micronutrients assessed (g per day), methionine and cysteine were associated with methylation of CpG17 (β = 5.0 (95%CI: 0.6-9.4), p = 0.04; and β = 2.4 (0.6-4.1), p = 0.02, respectively). Methionine, choline, and cysteine were additionally associated with the mean methylation of the entire CD40L (β = 9.5 (1.0-18.0), p = 0.04; β = 1.6 (0.4-3.0), p = 0.04; and β = 4.3 (0.9-7.7), p = 0.02, respectively). Associations of the SLEDAI with hypomethylation were confirmed for CpG17 (β=-32.6 (-60.6 to -4.6), p = 0.04) and CpG22 (β=-38.3 (-61.2 to -15.4), p = 0.004), but not the mean methylation of CD40L. Dietary products with the highest impact on methylation included meat, ice cream, white bread, and cooked potatoes. Conclusions Dietary methyl donors may influence DNA methylation levels and thereby disease activity in SLE.

scholarly journals Fatigue in patients with systemic lupus erythematosus and neuropsychiatric symptoms is associated with anxiety and depression rather than inflammatory disease activity

Lupus ◽  
2021 ◽  
pp. 096120332110050
Author(s):  
Rory C Monahan ◽  
Liesbeth JJ Beaart-van de Voorde ◽  
Jeroen Eikenboom ◽  
Rolf Fronczek ◽  
Margreet Kloppenburg ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Neuropsychiatric Symptoms ◽  
Anxiety And Depression ◽  
Systemic Lupus ◽  
Sf 36 ◽  
Neuropsychiatric Sle ◽  
Inflammatory Phenotype ◽  
The Mean

Introduction We aimed to investigate risk factors for fatigue in patients with systemic lupus erythematosus (SLE) and neuropsychiatric symptoms in order to identify potential interventional strategies. Methods Patients visiting the neuropsychiatric SLE (NPSLE) clinic of the Leiden University Medical Center between 2007–2019 were included. In a multidisciplinary consensus meeting, SLE patients were classified as having neuropsychiatric symptoms of inflammatory origin (inflammatory phenotype) or other origin (non-inflammatory phenotype). Fatigue was assessed with the SF-36 vitality domain (VT) since 2007 and the multidimensional fatigue inventory (MFI) and visual analogue scale (VAS) since 2011. Patients with a score on the SF-36 VT ≥1 standard deviation (SD) away from the mean of age-related controls of the general population were classified as fatigued; patients ≥2 SD away were classified as extremely fatigued. Disease activity was measured using the SLE disease activity index-2000. The influence of the presence of an inflammatory phenotype, disease activity and symptoms of depression and anxiety as measured by the hospital anxiety and depression scale (HADS) was analyzed using multiple regression analyses corrected for age, sex and education. Results 348 out of 371 eligible patients filled in questionnaires and were included in this study . The majority was female (87%) and the mean age was 43 ± 14 years. 72 patients (21%) had neuropsychiatric symptoms of an inflammatory origin. Fatigue was present in 78% of all patients and extreme fatigue was present in 50% of patients with an inflammatory phenotype vs 46% in the non-inflammatory phenotype. Fatigue was similar in patients with an inflammatory phenotype compared to patients with a non-inflammatory phenotype on the SF-36 VT (β: 0.8 (95% CI −4.8; 6.1) and there was less fatigue in patients with an inflammatory phenotype on the MFI and VAS (β: −3.7 (95% CI: −6.9; −0.5) and β: −1.0 (95% CI −1.6; −0.3)). There was no association between disease activity and fatigue, but symptoms of anxiety and depression (HADS) associated strongly with all fatigue measurements. Conclusion This study suggests that intervention strategies to target fatigue in (NP)SLE patients may need to focus on symptoms of anxiety and depression rather than immunosuppressive treatment.

Symptomatic osteonecrosis of the hip and knee in patients with systemic lupus erythematosus: Prevalence, pattern, and comparison of natural course

Lupus ◽  
2021 ◽  
pp. 096120332110310
Author(s):  
Mehmet Ersin ◽  
Mehmet Demirel ◽  
Mehmet Ekinci ◽  
Lezgin Mert ◽  
Çiğdem Çetin ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Rheumatic Diseases ◽  
Lupus Erythematosus ◽  
Bone Structure ◽  
Survival Rates ◽  
Knee Joints ◽  
Joint Involvement ◽  
Systemic Lupus ◽  
Kaplan Meier ◽  
The Mean

Objective Osteonecrosis (ON), also known as avascular necrosis, is characterized by the collapse of the architectural bone structure secondary to the death of the bone marrow and trabecular bone. Osteonecrosis may accompany many conditions, especially rheumatic diseases. Among rheumatic diseases, osteonecrosis is most commonly associated with systemic lupus erythematosus (SLE). We assessed prevalence and distribution pattern of symptomatic ON in patients with SLE and compare the natural courses of hip and knee ON. Methods 912 SLE patients admitted between 1981 and 2012 were reviewed. SLE patients with symptomatic ON were retrospectively identified both from the existing SLE/APS database. The prevalence of symptomatic ON was calculated; with ON, the joint involvement pattern was determined by examining the distribution of the joints involved, and then the data about the hip and knee joints were entered in the Kaplan-Meier analysis. Kaplan-Meier methods were used to calculate 5- and 10-year rates of ON-related hip (the hip group) and knee survival (the knee group). Results Symptomatic ON developed in various joints in 97 of 912 patients with SLE, and the overall prevalence of ON was detected as 10.6%. The mean age at the time of SLE and ON diagnoses were 27.9 ± 9.9 (14–53) and 34.2 ± 11.3 (16–62) years, respectively. The mean duration from diagnosis of SLE to the first development of ON was 70.7± 60.2 (range = 0–216) months. The most common site for symptomatic ON was the hips (68%, n=66), followed by the knees (38%, n = 37). According to Kaplan-Meier analysis, hip and knee joint survival rates associated with 5-year ON were 51% and 88%, and 10-year survival rates were 43% and 84%, respectively. Conclusion We observed that the prevalence of symptomatic ON in patients with SLE was 10.6%. With the estimated 10-year survival rates of 40% versus 84% for the hip and knee joints, respectively, hip involvement may demonstrate a more aggressive course to end-stage osteoarthritis than the knee involvement.

Shrinking lung syndrome in pediatric systemic lupus erythematosus

Lupus ◽  
2021 ◽  
pp. 096120332110103
Author(s):  
Alfonso Ragnar Torres Jimenez ◽  
Nayma Ruiz Vela ◽  
Adriana Ivonne Cespedes Cruz ◽  
Alejandra Velazquez Cruz ◽  
Alma Karina Bernardino Gonzalez
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Pleural Effusion ◽  
Pulmonary Function ◽  
Lupus Erythematosus ◽  
Pulmonary Function Tests ◽  
Age At Diagnosis ◽  
X Rays ◽  
Systemic Lupus ◽  
Function Tests ◽  
Restrictive Pattern

Shrinking Lung Syndrome (SLS) is a rare and little known complication associated with Systemic Lupus Erythematosus (SLE), characterized by progressive and unexplainable dyspnea, pleuritic pain, small pulmonary volumes and elevation of the diaphragm on chest X-rays as well as restrictive pattern on pulmonary function tests. Objective To describe clinical, radiological and treatment characteristics in pediatric patients with SLS. Material and methods This is a descriptive and retrospective study in patients under 16 years old with the diagnosis of SLE complicated by SLS at the General Hospital. National Medical Center La Raza. Clinical, radiological and treatment variables were analyzed. Results are shown in frequencies and percentages. Results Data from 11 patients, 9 females and 2 males were collected. Mean age at diagnosis of SLS was 12.2 years. Age at diagnosis of SLE was 11.1 years. SLEDAI 17.3. Renal desease 72%, hematological 91%, lymphopenia 63%, mucocutaneous 72%, neurological 9%, arthritis 54%, serositis 91%, fever 81%, secondary antiphospholipid syndrome, low C3 72%, low C4 81%, positive ANA 91%, positive anti-DNA 91%. Regarding clinical manifestations of SLE: cough 81%, dyspnea 91%, hipoxemia 81%, pleuritic pain 71%, average oxygen saturation 83%. Chest X-rays findings: right hemidiaphragm affection 18%, left 63%, bilateral 18%. Elevated hemidiaphragm 91%, atelectasis 18%, pleural effusion 91%, over one third of the cardiac silhouette under the diphragm 36%, bulging diaphragm 45%, 5th. anterior rib that crosses over the diaphragm 91%. M-mode ultrasound: diaphragmatic hypomotility 100%, pleural effusion 63%. Pulmonary function tests: restrictive pattern in 45% of the cases. Treatment was with supplementary oxygen 100%, intubation 18%, antibiotics 100%, steroids 100%, intravenous immunoglobulin 54%, plasmapheresis 18%, cyclophosphamide 54% and rituximab 18%. The clinical course was favorable in 81%. Conclusions SLS should be suspected in patients with SLE and active disease who present hipoxemia, pleuritic pain, cough, dyspnea, pleural effusion and signs of restriction on chest X-rays. Therefore, a diaphragmatic M-mode ultrasound should be performed in order to establish the diagnosis.

Hospitalization in patients with systemic lupus erythematosus at Tawam Hospital, United Arab Emirates (UAE): Rates, causes, and factors associated with length of stay

Lupus ◽  
2021 ◽  
pp. 096120332199008
Author(s):  
Reem Aldarmaki ◽  
Hiba I Al Khogali ◽  
Ali M Al Dhanhani
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Length Of Stay ◽  
Lupus Nephritis ◽  
United Arab Emirates ◽  
Lupus Erythematosus ◽  
Hospitalization Rate ◽  
Systemic Lupus ◽  
Factors Associated ◽  
Icu Admission ◽  
The Mean

Introduction Systemic lupus erythematosus (SLE) is a relapsing and remitting multiorgan disease associated with significant morbidity and mortality. The survival rate of patients with SLE has recently improved, which was associated with increased morbidity and hospitalization rates. Therefore, this study aimed to examine the rate and causes of hospitalization in patients with SLE and explore factors associated with increased length of stay (LOS). Methods Patients who visited rheumatology clinics (Tawam hospital, United Arab Emirates (UAE)) and fulfilled the American College of Rheumatology (ACR) SLE criteria were identified. Retrospective charts were reviewed to determine previous admissions. Demographic data, reason for hospitalization, duration of hospitalization, intensive care unit (ICU) admission, number of specialist consultations, medications used, and SLE characteristics at time of admission were collected. The hospitalization rate was calculated as the number of hospitalized patients divided by the total number of patients with the disease. We performed multivariable regression analysis for factors associated with increased LOS. Results A total of 91 patients with SLE (88 women and 3 men) met the inclusion criteria with a mean disease duration of 10.2 years (SD 5.5). A total of 222 admissions were identified, and 66 of 91 patients were admitted at least once. The mean crude hospitalization rate calculated was 29.8%. The primary reason for admission was pregnancy (29%), SLE activity (24%), and infection (20%). When combining primary and secondary reasons, the proportion of admissions due to SLE activity increased to 32%. The mean LOS was 5.9 (SD 6.0) days. About 7% of admitted patients required ICU admission. In multivariable analysis, patients with lupus nephritis, complications during hospitalization, and increased number of specialists consultations and who were admitted to ICU and started new medication were all associated with increased LOS. Conclusion A significant proportion of patients with SLE were hospitalized during their disease course. The hospitalization rate in this study appears to be higher than those reported elsewhere. Disease flare is the leading cause of admission in patients with SLE in this relatively young cohort. Lupus nephritis has been found to be significantly related to longer LOS. Measurements taken to reduce the incidence and severity of flares would likely decrease hospitalization rate and LOS in patients with SLE.

Optimal Frequency of Visits for Patients with Systemic Lupus Erythematosus to Measure Disease Activity Over Time

2010 ◽  
Vol 38 (1) ◽  
pp. 60-63 ◽  
Author(s):  
DOMINIQUE IBAÑEZ ◽  
DAFNA D. GLADMAN ◽  
ZAHI TOUMA ◽  
MANDANA NIKPOUR ◽  
MURRAY B. UROWITZ
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Activity Index ◽  
Disease Activity Index ◽  
Systemic Lupus ◽  
The Mean ◽  
Over Time ◽  
Measure Disease Activity ◽  
Lupus Disease Activity

Objective.Adjusted mean Systemic Lupus Erythematosus Disease Activity Index (SLEDAI; AMS) measures lupus disease activity over time. Our aim was to determine optimal visit frequency for calculating AMS.Methods.Patients followed monthly for 12 consecutive visits were included. AMS was calculated using all of the SLEDAI 2000 (AMSGOLD using all 12 visits), only quarterly visits (AMS3, using visits 3 months apart), semiannual visits (AMS6, using first, middle, and last visits only), and annual visits (AMS12, using only the first and last visits). Comparisons of AMS3, AMS6, and AMS12 with AMSGOLD are made using descriptive statistics.Results.Seventy-eight patients were included (92% women, mean age at SLE diagnosis 30.1 yrs and at study start 46.2 yrs). The mean (SD) AMSGOLD for the entire year was 2.05 (1.66), for AMS3 1.99 (1.65), for AMS6 2.12 (1.87), and for AMS12 2.08 (1.83). Mean (SD) of the absolute differences with AMSGOLD: for AMS3 0.29 (0.33), for AMS6 0.45 (0.59), and for AMS12 0.61 (0.58). Differences that were < 0.5 were considered minimal while those ≥ 1 were deemed important. Comparing AMSGOLD to AMS3, 82% of the differences were minimal and 3% were important. When comparing to AMS6, 68% were minimal and 10% were important, while comparing to AMS12, 50% were minimal and 21% were important.Conclusion.Usual clinic visits occurring quarterly offer a good estimation of disease activity over a 1-year period and are preferred over semiannual and annual visits.

Efficacy of Danazol with Autoimmune Thrombocytopenia

1997 ◽  
Vol 3 (4) ◽  
pp. 251-255 ◽  
Author(s):  
Steven W. Kim ◽  
Lawrence Rice ◽  
John J. McCarthy
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Evans Syndrome ◽  
Systemic Lupus ◽  
Autoimmune Thrombocytopenia ◽  
Treatment Regimens ◽  
Duration Of Disease ◽  
Males And Females ◽  
The Mean ◽  
Favorable Side Effect Profile

Seventy-nine cases of autoimmune thrombocytopenia seen by the Baylor Hematology section of The Methodist Hospital between 1991 and 1996 were retrospectively reviewed to assess the effectiveness of danazol in the treatment of autoimmune thrombocytopenia. Among the 42 patients who received danazol, the mean initial platelet count prior to treatment was 24.3 ± 17.4 (SD) × 109/L with a mean duration of disease of 53 months. Most cases were idiopathic, but some patients had underlying secondary disorders (rheumatoid arthritis, systemic lupus erythematosus, HIV, and/or Evans' syndrome). Overall 57% of the patients treated with danazol had an excellent or a good response with three patients who had unmaintained remission for >11 months. Minimal side effects were noted. Fifty percent of the patients with associated secondary disorders achieved an excellent or good response. The hemolytic component of all three Evans' syndrome cases was well controlled with danazol. In two cases, danazol was effective where a variety of other treatment regimens were not. An excellent or a good response was found in 58%, 62%, and 53% in patients >65 years old, between 45 and 65 years old, and <45 years old, respectively. Response rates were similar in males and females, Seventy percent of the nonsplenectomized patients had an excellent or a good response compared to 33% in postsplenectomy patients. Overall in view of its favorable side-effect profile, it is rational early on to attempt to abrogate the need for splenectomy, it may salvage splenectomy failures, and there is a reasonable response rate in those refractory to multiple prior therapies. Key Words: Autoimmune thrombocytopenia—Idiopathic thrombocytopenic purpura—Danazol— Systemic lupus erythematosus—HIV—Evans' syndrome.

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