Objective To estimate the annual incidence and prevalence of and frequency of mortality associated with antiphospholipid syndrome (APS). Methods An inception cohort of patients with incident APS in 2000–2015 from a geographically well‐defined population was identified based on comprehensive individual medical records review. All cases met the 2006 Sydney criteria for APS (primary definition) or had a diagnosis of APS confirmed by physician consensus (secondary definition). Levels of lupus anticoagulant, IgM and IgG anticardiolipin antibodies, and anti–β2‐glycoprotein I antibodies were tested in a centralized laboratory. Incidence rates were age‐ and sex‐adjusted to the 2010 US white population. Prevalence estimates were obtained from the incidence rates, assuming that there was no increased mortality associated with APS and that migration in or out of the area was independent of disease status. Results Among this cohort in 2000–2015, 33 cases of incident APS, as defined by the Sydney criteria, were identified (mean age of patients 54.2 years; 55% female, 97% white). The annual incidence of APS in adults ages ≥18 years was 2.1 (95% confidence interval [95% CI] 1.4–2.8) per 100,000 population. Incidence rates were similar in both sexes. The estimated prevalence of APS was 50 (95% CI 42–58) per 100,000 population, and was similar in both sexes. Six patients (18%) had a concurrent diagnosis of systemic lupus erythematosus. The most frequent clinical manifestation was deep vein thrombosis. The overall frequency of mortality among patients with APS was not significantly different from that in the general population (standardized mortality ratio 1.61, 95% CI 0.74–3.05). Conclusion APS occurred in ~2 persons per 100,000 population per year. The estimated prevalence was 50 per 100,000 population. Overall mortality was not notably different from that observed in the general population.
Background: Anti-phosphatidylserine prothrombin antibodies (aPSPT) are reported to be highly associated with the lupus anticoagulant (LAC) in established antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE) cohorts. Further, aPSPT has been suggested to be a useful surrogate LAC marker. However, validation studies replicating this relationship in an all-comer study population in the diagnostic clinical setting are lacking.Objectives: To determine the sensitivity and specificity of aPSPT to the LAC in an allcomer population undergoing evaluation for suspected APS.Methods: An assembled cross-sectional cohort from June 2017 to December 2018 undergoing APS evaluations across all medical specialties were reviewed for LAC, aPSPT, anti-cardiolipin (aCL), and anti-β2 glycoprotein-1 (β2GP1). Sensitivities, specificities, and negative and positive predictive values were calculated. Results and Conclusions:A cohort of 166 eligible patients was identified. Seventyone percent were female, 89% White, 15% with SLE, and 21% with APS. The aPSPT was found to be the most specific to the LAC. Specificity of IgG aPSPT was 100% (96%-100%) and IgM aPSPT was 97% (91%-100%) to the LAC. Corresponding positive predictive value for IgG aPSPT was 100% (89%-100%) and IgM aPSPT was 95% (84%-99%). In contrast, the sensitivities of aPSPT to the LAC were less robust, only in the 40%-50% range. The findings validate previously reported findings and lends extension to an all-comer population. These findings corroborate aPSPT as a potentially useful clinical marker of the LAC.
A 33-year-old man presented with new-onset, asymmetric, migratory oligoarthritis in the setting of several weeks of nausea and vomiting, diarrhoea, fevers and dysuria. He was initially treated in the inpatient setting with broad-spectrum antibiotics due to concern for an evolving sepsis presentation. Arthrocentesis of a large right knee effusion revealed inflammatory synovial fluid without findings suggestive of septic arthritis. Human leucocyte antigen B27 was positive and, taken together with the antecedent history of gastroenteritis, dysuria and inflammatory oligoarthritis, the clinical diagnosis was most consistent with reactive arthritis. Antibiotics were discontinued. His treatment course proved refractory to non-steroidal anti-inflammatory drugs and intra-articular and systemic glucocorticoid therapy with concurrent use of sulfasalazine and ultimately necessitated treatment with a tumour necrosis factor alpha inhibitor.
BackgroundHodgkin lymphoma is a hematologic malignancy usually confined to lymphatic structures and commonly associated with constitutional symptoms. Bony involvement and musculoskeletal symptoms are uncommon and typically seen in advanced disease. In this case, we report an unusual presentation of classical Hodgkin lymphoma and highlight diagnostic challenges leading to the misdiagnosis and treatment as chronic recurrent multifocal osteomyelitis.Case presentationA 38-year-old white man presented with lower extremity musculoskeletal pain. Imaging studies revealed multifocal lytic and sclerotic osseous axial lesions. Multiple core needle bone marrow and excisional lymph node biopsies were non-diagnostic. Having met the criteria, a tentative diagnosis of chronic recurrent multifocal osteomyelitis was given. He was treated with non-steroidal anti-inflammatory medications with partial clinical response but had persistent symptoms. A second medical opinion was pursued. An open bone marrow biopsy was performed and yielded a diagnosis of classical Hodgkin lymphoma after 13 months of diagnostic uncertainty. A chemotherapy regimen of doxorubicin, bleomycin, vinblastine, and dacarbazine was instituted with complete symptomatic and radiologic response.ConclusionsThis case illustrates diagnostic difficulties of a musculoskeletal presentation of Hodgkin lymphoma, challenges of non-diagnostic bone marrow and lymph node biopsies, and resultant diagnostic delays in delivering a potentially curative therapy. Had the additional open bone marrow biopsy not been performed, the diagnosis and treatment of Hodgkin lymphoma would have been missed.
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