Autologous hematopoietic stem cell transplantation has better outcomes than conventional therapies in patients with rapidly progressive systemic sclerosis.

We retrospectively evaluated the efficacy of autologous hematopoietic stem cell transplantation (AHSCT) in 18 patients with rapidly progressive diffuse cutaneous systemic sclerosis (rp-dcSSc), and compared their disease outcomes with those of 36 demographically- and clinically-matched patients treated with conventional therapies. Cutaneous involvement, by performing modified Rodnan skin score (mRss), lung diffusion capacity, by measuring diffusing capacity of lung for carbon monoxide (DLCO), and disease activity, by applying the European Scleroderma Study Group (ESSG) scoring system, were the outcome variables measured at the baseline time and then every 12 months for the following 60 months in both the AHSCT-treated patients and the control group. In the AHSCT group, treatment-related mortality was 5.6%. In this group, both mRss and ESSG scores showed a significant reduction 1 year after AHSCT (P<0.002); and these results were maintained until the end of follow-up. Conversely, DLCO values remained stable during the whole period of follow-up. Survival rate of AHSCT group was much higher than that observed in the whole control group (P=0.0005). The probability that the ESSG score and mRss would remain at a high level, and DLCO could decrease, was significantly higher in the control group as a whole and in the subgroup of control patients treated with cyclophosphamide than in the AHSCT group. This study confirms that the AHSCT is effective in prolonging survival, as well as in inducing a rapid reduction of skin involvement and disease activity, and preserving lung function in patients with rp-dcSSc.

Has the clinical spectrum of gout changed over the last decades?

OBJECTIVES: To evaluate if the clinical spectrum of gout has changed over the last decades, a cohort of 107 patients was studied in relation to the date of disease onset and of presentation to our Institution. METHODS: The structured questionnaires of 107 consecutive patients with gout seen between 1989 and 2009 were retrospectively reviewed. Patients were divided into two subgroups according to the year of the first acute arthritis attack: (a) patients with gout onset dating back to 1967-2001, and (b) patients with gout of later (2002-2009) onset. The patients were also subdivided according to the date of their first admission to our Institution: 1989-2006 and 2007-2009 admissions. RESULTS: The male to female ratio changed from 11.3 in the patients who had the first attack before 2002 to 2.4 in the second onset group (p=0.02). A family history of gout was slightly more frequent in the second subgroup (22.7% vs. 6.4%, p=0.05). The mean number of acute attacks was higher in patients seen before 2002 (p=0.01). Synovial fluid was examined more frequently in the subgroup visited for the first time after 2006 (p=0.001). CONCLUSIONS: Our data indicating that the patients' sex ratio has changed over time, with women more frequently affected, could be of importance to clinicians who often believe that gout is a disease affecting males only. The increased utilisation of synovial fluid analysis suggests a closer attention to the disease in recent years. Clinicians should be aware that gout is increasingly affecting women.

Calcium pyrophosphate deposition disease: clinical manifestations.

Calcium pyrophosphate deposition (CPPD) disease is an arthropathy caused by calcium pyrophosphate dihydrate (CPP) crystal deposits in articular tissues, most commonly fibrocartilage and hyaline cartilage. According to EULAR, four different clinical presentations can be observed: 1) asymptomatic CPPD; 2) osteoarthritis (OA) with CPPD; 3) acute CPP crystal arthritis; 4) chronic CPP inflammatory crystal arthritis. Acute CPP crystal arthritis is characterized by sudden onset of pain, swelling and tenderness with overlying erythema, usually in a large joint, most often the knee, wrist, shoulder, and hip. Occasionally, ligaments, tendons, bursae, bone and the spine can be involved. CPPD of the atlanto-occipital joint (crowned dens syndrome) can cause periodic acute cervico-occipital pain with fever, neck stiffness and laboratory inflammatory syndrome. Chronic inflammatory arthritis is characterized by joint swelling, morning stiffness, pain, and high ESR and CRP. The relationship between OA and CPPD is still unclear. The main problem is whether such crystals are directly involved in the pathogenesis of OA or if they are the result of joint degeneration. Diagnosis is based on evaluation of history and clinical features, conventional radiology, and synovial fluid examination. Non-polarized light microscopy should be used initially to screen for CPPD crystals based upon their characteristic morphology, and compensated polarized light microscopy, showing the crystals to be weakly positive birefringent, is recommended for definitive identification, although this last pattern only occurs in about 20% of samples. The main goals of CPPD therapy are control of the acute or chronic inflammatory reaction and prevention of further episodes.

Imaging of tophi with an extremity-dedicated MRI system.

OBJECTIVES: To describe the MRI features of gout tophi in the soft-tissues or joints of the limbs by low-field extremity-dedicated MRI. METHODS: Nine consecutive patients, 8M/1W, affected by chronic tophaceous gout were studied. Mean patients' age was 71.3±11.5 years, mean disease duration 98.1±44.9 months, and mean serum uric acid concentration 9.2±2.8 mg/L. Diagnosis was based on the ACR classification criteria for gout, and by identification of MSU crystals in the tophi and synovial fluid. Conventional radiograms and MRI with an extremity-dedicated system were obtained of the joint areas involved by tophi. RESULTS: At T1 weighted MRI images, all tophi showed a homogeneous intermediate signal intensity, similar to that of muscle. Conversely, in T2 weighted images, a wide spectrum of signal intensity patterns was observed. The pattern of contrast enhancement was variable from intense homogeneous to peripheral and heterogeneous. Capsulo-ligamentous structures were often thickened and degenerated and, on occasion, could be recognised as inhomogeneous, hypointense ribbon-shaped elements in the context of the tophus. In only two cases, tendons were infiltrated by tophaceous matter. Bone marrow oedema (BME) and erosions were seen in 8 out of 10 bones adjacent to tophi. CONCLUSIONS: The MRI appearance of gout tophi using an extremity-dedicated machine is similar to that described in the literature using whole body machines. BME adjacent to the tophus was a frequent finding. This technique may occasionally help in the differential diagnosis of nodules and in the follow-up of the disease. It also represents a useful tool to investigate the pathogenesis of gout and to better understand its clinical progression.

[Clinical features of gout in a cohort of Italian patients]. / Caratteristiche cliniche della gotta in una coorte di pazienti Italiani.

OBJECTIVE: To assess the clinical characteristics of gout and its diagnostic approach in a group of Italian patients. METHODS: In a retrospective analysis, we evaluated 72 consecutive gouty patients examined in the years 2000-2007. We recorded demographic data, family history, comorbidities and disease characteristics (seasonality of the attacks, joints affected, serum uric acid concentration, and treatment). RESULT: 63/72 (87.5%) patients were men and 9 women, with mean age 61.9+/-13.7 years. 8/72 (11.1%) patients reported a familial history of gout. The first attack occurred mainly in the months of June, July and December. The first metatarsophalangeal joint was affected in 59.7% of patients and the hand in 25%. Treatment changed over the follow-up period, with a decreased use of NSAIDs (p<0.0001) and an increased use of colchicine (p=0.015) and allopurinol (p<0.0001). In 9 (12.5%) patients, joint aspiration was performed and monosodium urate crystals were found in synovial fluid or tophi. 42/72 (58.3%) patients fulfilled a minimum of 6 clinical criteria of the American College of Rheumatology, necessary for gout diagnosis. 47/72 (65.3%) patients, met the EULAR recommendations and had an 82% probability of being affected by gout. CONCLUSIONS: The diagnosis of gout is not always easy because of its changing clinical spectrum. Identification of MSU crystals in joint aspirates was obtained only in a minority of patients. In this setting the diagnosis with gout was often based on the observation of an acute intermittent monoarthritis involving mainly the first metatarsophlangeal joint, associated with hyperuricaemia and responsive to colchicine.

Risk factors for gouty dactylitis.

OBJECTIVE: To investigate the risk factors associated with dactylitis in gout. METHODS: The questionnaires of 73 consecutive gouty patients, diagnosed according to the ACR criteria, were reviewed. They were consecutively screened for the presence of dactylitis. RESULTS: Seven out of 73 (9.6%) patients showed dactylitis. They had (1) longer disease duration, (2) a higher number of involved joints, (3) higher serum uric acid concentration, (4) more tophi, (5) higher ESR, and (6) a higher number of ACR criteria besides crystal identification, than the others. By logistic regression, renal stones (OR 13.3, 95% CI 1.1-158.3), upper extremity involvement (OR 4.9, 95% CI 1.4-16.6), number of ACR criteria (OR 1.9, 95% CI 1.1-3.3), and ESR (OR 1.02, 95% CI 1-1.04), significantly predicted dactylitis. CONCLUSIONS: Dactylitis is a feature of gout representing an indicator of disease severity.