Acquired Haemophilia A. Which is the best therapeutic choice in older adults? Single center study of 4 cases.

Acquired haemophilia A (AHA) is a rare bleeding disorder due to autoantibodies directed against coagulation factor VIII. The treatment is based on recombinant activated factor VII and activated prothrombin complex concentrate. However, mainly in older patients, severe thrombotic complications have been reported.

Myositis in primary Sjögren's syndrome: data from a multicentre cohort.

Objectives: In primary Sjögren's syndrome (pSS), muscle pain and/or muscular weakness is relatively frequent while myositis has been reported in 3% of patients. The aim of this study was to describe the prevalence of myositis in a multicentre Italian pSS cohort and to address the clinical manifestations, histological findings and therapeutic strategies.

Methods: Clinical, serological and therapeutic data from a pSS cohort of patients were retrospectively collected.

Primary Sjogren's syndrome as a multi-organ disease: impact of the serological profile on the clinical presentation of the disease in a large cohort of Italian patients.

Objective: The aims of this study were to describe the clinical presentation of primary SS (pSS) in a large cohort of patients by assessing the prevalence of the patient subgroups at high risk for severe extraglandular manifestations and to explore the influence of the patients' serological profile on disease severity and on immunosuppressive drug utilization.

Methods: Cumulative demographic, clinical, serological, histological and therapeutic data of 1115 pSS patients were retrospectively evaluated. Independent serological markers for glandular and extraglandular disease manifestations were identified by logistic regression.

Biomarkers of lymphoma in Sjögren's syndrome and evaluation of the lymphoma risk in prelymphomatous conditions: results of a multicenter study.

Objectives: To define the biomarkers associated with lymphoproliferation in primary Sjögren's syndrome (pSS) by distinguishing in separate groups the two best-recognized non-malignant prelymphomatous conditions in pSS, i.e., salivary gland swelling and cryoglobulinemic vasculitis (CV).