Acquired Hemophilia in a Patient With Rheumatoid Arthritis: Case Report and Literature Review

Acquired hemophilia (AH) is a rare bleeding disorder caused by the spontaneous development of autoantibodies against coagulation factors, most commonly factor (F) VIII (acquired hemophilia A, AHA). The clinical manifestation of AHA includes mostly spontaneous hemorrhages into skin, mucous membranes, muscles, soft tissues, or joints. AHA should be suspected when a patient with no history of hemorrhages presents with bleeding and an unexplained prolonged activated partial thromboplastin time. The diagnosis is based on the clinical picture, the presence of low FVIII activity and evidence of FVIII inhibitor. In around half of patients, an underlying disorder (rheumatic diseases, malignancy, infections) or taking some drugs are associated with AHA; the remaining cases are idiopathic. Rheumatoid arthritis is a chronic inflammatory condition, marked by swelling and tenderness of small joints; it is usually treated with steroid and immunosuppressive drugs such as methotrexate, TNF-alpha inhibitors, and other biologic therapies (abatacept, tocilizumab, rituximab).We presented a patient with rheumatoid arthritis who developed acquired hemophilia A with hemarthroses; starting from this case, we focused on the literature about AHA in rheumatic diseases. We found 35 cases, 15 in systemic lupus erythematosus and 12 in rheumatoid arthritis, while the remaining cases were reported in Sjögren's syndrome, polymyalgia rheumatica, systemic sclerosis, and psoriatic arthritis. Ecchymosis and cutaneous hematomas were the main clinical features while hemarthroses was quite a rare condition, shown in just three patients

Update on Vertigo in Autoimmune Disorders, from Diagnosis to Treatment

The prevalence of autoimmune diseases has been increasing over the last 20 years. The clinical presentation of this large and heterogeneous group of disorders depends on whether the involvement is organ-specific or non-organ-specific. Dizziness, vertigo, and disequilibrium are common symptoms reported by patients with vestibulocochlear involvement. The association of vertigo and autoimmune diseases has been largely documented, suggesting that autoimmune disorders could be overrepresented in patients with vertigo in comparison to the general population. The aim of this review is to present the recent literature findings in the field of autoimmune-mediated diseases with cochleovestibular involvement, focusing on the clinical presentation, diagnosis, and treatment of immune-mediated inner ear diseases including autoimmune inner ear disease (AIED), Meniere’s disease, and bilateral vestibulopathy, as well as of systemic autoimmune diseases with audiovestibular disorders, namely, Behçet’s disease, Cogan’s syndrome, sarcoidosis, autoimmune thyroid disease, Vogt-Koyanagi-Harada syndrome, relapsing polychondritis, systemic lupus erythematosus, antiphospholipid syndrome, IgG4-related disease, and ANCA-associated vasculitides

Validation of the Italian version of the educational needs assessment tool in rheumatoid arthritis patients and factors associated with educational needs

The educational needs assessment tool (ENAT) is a seven-domain questionnaire assessing the educational needs (EN) of patients with rheumatoid arthritis (RA). The aim of this study was to validate the Italian version of the ENAT and to identify factors associated with EN in people with RA. The original English ENAT version was translated into Italian according to Beaton's method and subjected to Rasch analysis for validity testing. Socio-demographic and clinical variables were tested for associations with the ENAT domain scores using a multivariable linear regression model. The ENAT translated well into Italian and retained its construct validity. Some adjustments were needed when pooling the Italian and English datasets. The overall score of the ENAT had a high median: 82.8 (interquartile range (IQR): 57.5 to 100) i.e., 72.4% of the maximum score. The highest score was observed in the domain "Arthritis process" and the lowest was in "Support systems". Only gender was independently associated with EN (females having higher EN than males). The Italian ENAT is feasible for the use in the clinical setting and may help the health care practitioners to tailor educational interventions for RA patients. The characteristics of the patients, particularly female gender, may be associated with higher EN

Practical approach to vasculitides in adults: an overview of clinical conditions that can mimic vasculitides closely

Primary systemic vasculitides are rare diseases affecting blood vessel walls. The type and patterns of distribution of the organs affected usually reflect the size of the vessels predominantly involved, and the patterns of clinical manifestations are generally useful to reach a specific diagnosis. However, presenting symptoms may lack adequate specificity for a prompt diagnosis, leading to a diagnostic (and therapeutic) delay, often causing irreversible damage to the affected organs. Due to their rarity and variable clinical presentation, the diagnosis of primary vasculitides could be challenging for physicians. Vasculitis mimickers, i.e. the clinical conditions that could be likely mistaken for vasculitides, need to be carefully ruled out, especially before starting the immunosuppressive therapy. We present here a practical approach to the diagnosis of primary systemic vasculitides involving large, medium and small size vessels, and reviewed most of the conditions that could mimic primary systemic vasculitides

Leukocytoclastic vasculitis associated with multifocal sensory neuropathy responsive to intravenous immunoglobulins: a case report

Leukocytoclastic vasculitis (LCV) is a common form of small-vessel vasculitis, which commonly presents as palpable purpura or petechiae, caused by deposition of circulating immune complexes on vessels walls that attracts granulocytes which damage the vascular endothelium and leading to erythrocytes extravasation. The skin is the most commonly involved organ, but also renal, gastrointestinal, pulmonary, cardiovascular and neurological systems may be affected. Skin lesions may be the initial signs of systemic vasculitis. Systemic symptoms may be present, such us fever, myalgia, abdominal pain and arthralgia. The presence of neuropathy/mononeuritis multiplex is expression of severe vasculitic involvement. Herein, we describe the case of a patient with leucocytoclastic vasculitis associated to sensitive neuropathy, responsive to intravenous immunoglobulins (IVIg) therapy, after the failure of classic systemic treatments

Do obesity and overweight influence disease activity measures in axial spondyloarthritis? A systematic review and meta‐analysis

the aim of our systematic review and meta-analysis was to investigate whether overweight/obesity are associated with higher disease activity measures in axial spondyloarthritis (axSpA) patients

Do obesity and overweight influence disease activity measures in axial spondyloarthritis? A systematic review and meta-analysis

the aim of our systematic review and meta-analysis was to investigate whether overweight/obesity are associated with higher disease activity measures in axial spondyloarthritis (axSpA) patients

Ear, nose, and throat in ANCA-associated vasculitis: a comprehensive review

Ear, nose, and throat (ENT) involvement is a common feature in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), particularly in granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis. Over the last decade, substantial advancement has been made in understanding AAV pathogenesis, classification, and treatment. Typical ENT symptoms may include sinonasal, otologic, pharyngeal, and laryngeal manifestations. The otolaryngologic symptoms of AAV sometimes might be misdiagnosed in etiology as infectious or allergic. Thus, rapid recognition and early diagnosis of AAV as the cause of the symptoms prevent the risk of irreversible organ damage. The high impact of ENT symptoms on the quality of life of AAV patients confirms the importance of their early treatment through specific local and systemic approaches. Appropriate interdisciplinary management to early recognition of AAV and initiation of treatment may reduce morbidity in these patients. The purpose of this comprehensive review is to describe the clinical, histological, and radiological findings of ENT involvement in AAV and to update their surgical and therapeutic management, with a focus also on the role of a multidisciplinary team, involving the otorhinolaryngologist

Does gender influence clinical expression and disease outcomes in COVID-19? A systematic review and meta-analysis

Severe Acute Respiratory Syndrome coronavirus-2 (SARS-CoV) has been recently characterized, and soon spread around the world generating a pandemic. It has been suggested that men are more severely affected by the viral disease (COVID-19) than women