The Italian version of WOMAC is a reliable and valid instrument for evaluating the severity of OA of the knee, with metric properties in agreement with the original, widely used version.

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Alkaptonuria (AKU) is an ultra-rare disease developed from the lack of homogentisic acid oxidase activity, causing homogentisic acid (HGA) accumulation that produces a HGA-melanin ochronotic pigment, of unknown composition. There is no therapy for AKU. Our aim was to verify if AKU implied a secondary amyloidosis. Congo Red, Thioflavin-T staining and TEM were performed to assess amyloid presence in AKU specimens (cartilage, synovia, periumbelical fat, salivary gland) and in HGA-treated human chondrocytes and cartilage. SAA and SAP deposition was examined using immunofluorescence and their levels were evaluated in the patients' plasma by ELISA. 2D electrophoresis was undertaken in AKU cells to evaluate the levels of proteins involved in amyloidogenesis. AKU osteoarticular tissues contained SAA-amyloid in 7/7 patients. Ochronotic pigment and amyloid co-localized in AKU osteoarticular tissues. SAA and SAP composition of the deposits assessed secondary type of amyloidosis. High levels of SAA and SAP were found in AKU patients' plasma. Systemic amyloidosis was assessed by Congo Red staining of patients' abdominal fat and salivary gland. AKU is the second pathology after Parkinson's disease where amyloid is associated with a form of melanin. Aberrant expression of proteins involved in amyloidogenesis has been found in AKU cells. Our findings on alkaptonuria as a novel type II AA amyloidosis open new important perspectives for its therapy, since methotrexate treatment proved to significantly reduce in vitro HGA-induced A-amyloid aggregates.

Objectives: To assess the prevalence of hand, hip, and knee osteoarthritis (OA) in an older Italian community dwelling population and its association with disability. Method: A cross sectional survey of the whole community aged 65 years and over, was carried out in Dicomano, a small rural town in Tuscany, Italy. Subjects were screened by geriatricians for major chronic conditions, including hip, knee, and hand OA, using diagnostic algorithms based on the American College of Rheumatology (ACR) clinical criteria. A rheumatologist examined subjects who screened positive. Disability was assessed by a World Health Organisation questionnaire. Results: 697 of 864 eligible subjects (81% of the eligible population) were screened. OA of the knee, hand, or hip was identified by clinical ACR criteria respectively in 159, 139, and 81 subjects, and was confirmed by the rheumatologist in 158/182 (87%), 75/101 (74%), and 63.2% of cases. The estimated prevalence was 29.8%, 14.9%, and 7.7%, respectively. Only hip OA was significantly associated with disability in basic activities of daily living. Conclusions: About one third of community dwelling older people are affected by symptomatic peripheral OA. Hip OA was strongly associated with disability.