Rheumato, Volume 5, Issue 2 (June 2025) – 2 articles

Hemophilia is an X-linked genetic disorder that predominantly affects males, with females typically serving as asymptomatic carriers. Hemophilia A results from a deficiency or dysfunction of coagulation factor VIII, while a deficiency in factor IX causes hemophilia B. A less common condition, factor XI deficiency (formerly hemophilia C), is categorized as a rare bleeding disorder. The severity of hemophilia is classified based on the activity concentration of factors VIII and IX: severe (<1 IU/dL), moderate (1–5 IU/dL), and mild (6–<40 IU/dL). One of the most prevalent complications of hemophilia is hemarthrosis, bleeding into joint cavities, which, if unrecognized or untreated, can lead to hemophilic arthropathy. The pathophysiology of hemophilic arthropathy involves two key mechanisms: the accumulation of iron from blood in synovial joints, which cannot be cleared due to repeated bleeding, and the inflammatory response, resulting in synovial hyperplasia and the progressive destruction of cartilage and bone. Hemophilic arthropathy significantly impairs quality of life, causing chronic pain, joint deformities, and sometimes requiring surgical intervention. This thesis will examine the pathophysiology and management strategies for hemophilic arthropathy. Full article

Background: Although the Profile of Mood States has been proposed by the Initiative on Methods, Measurement, and Pain Assessment in Clinical Trials (IMMPACT) to measure emotional functioning in chronic pain patients, it has not yet been validated in these patients. Objective: To confirm the factor structure and internal consistency of the Shortened Version of the Profile of Mood States (37 items) in patients with chronic pain. Methods: A confirmatory factor analysis (CFA) was conducted to test the theoretical structure of six factors that result in a Total Mood Disturbance episode: Tension–Anxiety (six items), Depression–Dejection (eight items), Anger–Hostility (seven items), Vigor–Activity (six items), Fatigue–Inertia (five items), and Confusion–Bewilderment (five items). Participants: A total of 588 patients with chronic pain from Spanish primary care health and community centers completed the questionnaire. Results: The factors presented adequate reliability coefficients, McDonald’s Omega (ω) between 0.77 and 0.91, and appropriate average discrimination indexes (D), ranging from 0.35 to 0.67. The Total Mood Disturbance yielded excellent results, ω = 0.95, D = 0.61. The original structure was confirmed, ECVI = 4.361 (saturated ECVI = 2.395; independent ECVI = 21.855); RMSEA = 0.070, 90% CI [0.067, 0.072]; GFI = 0.974; AGFI = 0.971; CFI = 0.853; NFI = 0.812; and NNFI = 0.843. Conclusions: The Shortened Version of the Profile of Mood States has been proven to be a valid and reliable measure of emotional functioning in chronic pain patients. Full article