Clinical profile and long-term outcome of granulomatosis with polyangiitis (GPA): A corporate hospital-based study from northern India

ObjectiveTo study the clinical profile and long-term outcome of granulomatosis with polyangiitis (GPA).MethodsRecords of patients with GPA attending our rheumatology clinic between April 2009 and February 2015 were retrieved. Clinical and laboratory data, details of treatment given and outcome were obtained and analysed. Remission and relapse were defined according to British Society for Rheumatology guidelines. Vasculitis damage index (VDI) was used for quantifying organ damage.ResultsThere were 45 patients (26 female, 19 male) with the mean age of 46 ± 12 years and median disease duration at diagnosis of 20 months. Lung, ear, musculoskeletal system, eye, nose and kidney were most frequently involved in that order. Disease was severe in 29 and limited in 16. Miscellaneous presenting features included saddle-nose deformity in 5 patients, digital gangrene in 2 and complete heart block, renal mass and renal infarction in 1 each. Tissue biopsy contributed to diagnosis in 26/30 (86%). Anti-neutrophil cytoplasmic antibody was positive in 33 (73%) patients. Prednisolone + cyclophosphamide (oral in 25, intravenous in 10) and prednisolone + rituximab were initially used for inducing remission in 35 and 4 patients, respectively. Rituximab was also used for induction in 4 patients who failed on cyclophosphamide. Mycophenolate or methotrexate were used as immunosuppressants in limited disease. Azathioprine was used for maintenance of remission. Median follow-up was 49 months. One patient died of intractable diffuse alveolar haemorrhage and stroke within 3 months. 34 patients achieved complete remission. Sustained remission (≥2 years) occurred in 24 (median 3.5 years). Major relapse occurred in 24 (53%). Of these, 3 had a second and 2 had a third relapse. Two patients relapsed after 11 years in remission. Preferred treatment for relapse included rituximab and cyclophosphamide. Mean VDI of patients in this series was 3.02.ConclusionGPA remits with standard treatment but relapses frequently, leading to substantial morbidity. Mortality is uncommon.     

Epidemiology of Meckel's diverticulum: A nationwide population-based study in Taiwan: Characteristics of the cases from surgery between 1996 and 2013

The diverse presentation of Meckel''s diverticulum (MD) is a diagnostic challenge for clinicians and most previous studies consist of single institutional case series. The aim of this study was to review the related diagnoses of MD and to investigate the epidemiological characteristics using Taiwan''s National Health Insurance Research Database.We conducted an observational study using a population-based database. Patients diagnosed with MD who concurrently received intestinal surgery were identified. We analyzed the patients’ demographic characteristics and relevant diagnoses using χ² test and 2-sample t test.We identified 2453 newly diagnosed MD patients from 1996 to 2013 and 1227 patients (50%) with intestinal obstruction, gastrointestinal bleeding, and acute appendicitis (acute abdominal pain) were defined as symptomatic. The male to female ratio was 2.4:1 with half of the patients experiencing symptomatic MD before the age of 20 years’ old. The age-specific and annual incidence were calculated for all MD and symptomatic MD. Among the symptomatic MD patients, intestinal obstruction was present in 583 (48%), acute appendicitis was present in 464 (38%), and gastrointestinal bleeding was present in 283 (23%) patients. Intestinal obstruction was the most common preoperative diagnosis in the 0 to 10 years and >20 years’ age groups, and acute appendicitis (acute abdominal pain) was the most common diagnosis in the 11 to 20 years’ age group.This population-based 18 years’ epidemiologic study described the distributions of MD symptoms among different age groups, which may help clinicians gain a better understanding of this diagnostically challenging gastrointestinal anomaly.     

Epidemiology and clinical course of Behçet's disease in the Reggio Emilia area of Northern Italy: A seventeen‐year population‐based study

Objective

To investigate the epidemiology and clinical course of Behçet's disease (BD) over a 17‐year period in a defined area of northern Italy.

Methods

All patients with incident BD diagnosed over a 17‐year period (from January 1, 1988 to December 31, 2004) living in the Reggio Emilia area were identified through the following sources: physicians at Reggio Emilia Hospital, medical practitioners, and community‐based specialists. We identified all patients registered in a centralized index and in the Reggio Emilia district database for rare diseases. Patients were followed up from the time of diagnosis until either their death or April 1, 2005.

Results

Eighteen patients (9 men and 9 women) had complete BD. Mean ± SD age at diagnosis was 33 ± 7 years. The incidence rate of BD was 0.24 per 100,000. The prevalence of BD on January 1, 2005 was 3.8 per 100,000. No patients died during the followup period. Although all patients developed oral ulceration during the disease course, 22.2% had no oral lesions at disease onset. Eye disease occurred in 55.6%. Ocular disease was more common in men and appeared at disease onset or within the first few years of disease onset (median 3 years). Only 1 patient had loss of useful vision in at least 1 eye at the end of followup. In all affected patients, visual acuity improved once treatment was started.

Conclusion

This population‐based study is the first to report the prevalence and incidence of BD in Italy. In Italian patients, BD is nonfatal and the prognosis of eye disease is good.     

Transformed Waldenström macroglobulinaemia: clinical presentation and outcome. A multi‐institutional retrospective study of 77 cases from the French Innovative Leukemia Organization (FILO)

Histological transformation (HT) to diffuse large B‐cell lymphoma (DLBCL) is a rare and poorly reported complication of Waldenström macroglobulinaemia (WM). We performed a retrospective study of 77 WM patients with biopsy‐proven transformation to DLBCL. The median time from WM diagnosis to HT was 4·6 years and 16 patients (21%) had never been treated for WM. At HT, extranodal sites were observed in 91% of patients with a rather high incidence of central nervous system, cutaneous or testicular involvement. Fluorodeoxyglucose‐positron emission tomography was performed in half of the patients and the median maximum standardized uptake value was 15 for transformed disease. More than 80% of cases with available data for assessment by the Hans’ algorithm harboured a non‐germinal centre B‐cell phenotype. First‐line treatment for transformation consisted of R‐CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone)‐like regimen in 85% of patients. The overall response rate after first‐line treatment was 61% and the median overall survival was only 16 months for the entire cohort. Time to transformation above 5 years (P = 0·0004) and elevated LDH (P = 0·02) were associated with worse outcome. Based on these findings, HT should be considered and lead to a biopsy in WM patients presenting with extranodal involvement, elevated LDH and constitutional symptoms. The optimal therapeutic approaches remain to be defined.     

Association of Life's Essential 8 with all-cause and cardiovascular mortality among US adults: A prospective cohort study from the NHANES 2005–2014

Background and aimsThis study aims to investigate the association of Life's Essential 8 (LE8), the recently updated algorithm for quantifying cardiovascular health (CVH) by the American Heart Association (AHA), with long-term outcomes among US adults.Methods and resultsThis population-based prospective cohort study analyzed data of 23,110 participants aged 20 years or older from the National Health and Nutrition Examination Survey from 2005 to 2014 and their linked mortality data through December 2019. LE8 score (range 0–100) was measured according to AHA definitions and was categorized into low (0–49), moderate (50–79), and high (80–100) CVH. The weighted mean age of the study population was 47.0 years (95% confidence interval [CI], 46.4–47.5 years), and 11,840 were female (weighted percentage, 51.5%; 95% CI, 50.9–52.1%). During a median follow-up period of 113 months (up to 180 months), 2942 all-cause deaths occurred, including 738 CVD deaths. The LE8 score was significantly and inversely related to mortality from all causes (adjusted hazard ratio [HR] for per 10-score increase in LE8 score, 0.86; 95% CI, 0.82–0.90) and cardiovascular disease (adjusted HR for per 10-score increase in LE8 score, 0.81; 95% CI, 0.75–0.87). Compared with participants having low CVH, those having high CVH had a reduction of 40% (adjusted HR, 0.60; 95% CI, 0.48–0.75) in the risk for all-cause mortality and 54% (adjusted HR, 0.46; 95% CI, 0.31–0.68) in the risk for cardiovascular mortality.ConclusionsHigher LE8 score was independently associated with lower risks of all-cause and cardiovascular mortality among US adults.