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Erosive osteoarthritis (EOA) is believed to be a clinically uncommon subset of generalized osteoarthritis (OA) characterized by a clinical course, which is frequently aggressive. The diagnosis of EOA is accepted only for patients meeting American College of Rheumatology clinical criteria for OA of the hand and showing radiographic aspects of articular surface erosions. Conditions to be considered in the differential diagnosis include primarily nodal generalized OA, psoriatic arthritis and rheumatoid arthritis. It is possible to find erosive changes resembling EOA in endocrine diseases, microcrystal-induced diseases, chronic renal diseases, autoimmune diseases and others. Despite the absence of a clear etiology, immunogenetic studies are useful in identifying a possible predisposition to developing EOA in some subjects. No definitive therapeutic approach to EOA has been reported. It is reasonable to assume that in the presence of a symptomatic EOA our therapeutic approach should differ from that used for common, nodal, non-EOA.

Osteonecrosis is a disease characterized by the death of marrow and bone tissues. All bones may be affected, most commonly those of the hip, knee, shoulder, ankle as well as the small bones of the hands and feet. When the disease involves a weight-bearing joint there is a significant risk that subarticular fracture may develop leading to disabling arthrosis and requiring, therefore, arthroplasty surgery. Osteonecrosis typically affects patients in their third, fourth and fifth decades of life and is associated with many factors including other diseases and co-morbidities. Multifocal osteonecrosis is defined according to the involvement of at least three separated anatomic sites. We describe the case of a young man with osteonecrosis of the shoulder and hip joints which required total arthroplasty. Among biochemical investigations, an increase in the plasminogen activator inhibitor type 1 (PAI-1) levels associated with mild hyperhomocysteinemia was present. Another finding was the HLA B27, without signs of spondyloarthropathies. In patients with osteonecrosis, especially if multifocal, a careful medical history, a complete physical examination and some biochemical investigations, particularly those related to thrombophilia and hypofibrinolysis, should be performed.

Objectives: The exact prevalence of erosive osteoarthritis (EOA) is still unknown. Aim of our study was therefore to evaluate the prevalence of osteoarthritis (OA) and EOA of the hand in a representative population from Venetian area., Methods: All people aged > 40 years of a small town in Venetian area has been considered. A complete clinical evaluation has been performed in all subjects. For those patients with clinical signs or symptoms of OA, radiographic evaluation of the hands has been carried out., Results: The entire population aged >40 years living in Silea entered the study. Signs or symptoms of OA were present in 200 out 640 subjects (31.2%), whereas EOA was diagnosed in 17 (8.5%) all female, mean age 57.5 +/-10.1 years (range 41-74) mean disease duration 5.1 +/-3.8 years (range 1-15). The most frequently involved joint was the second DIP of the right hand., Conclusion: The prevalence of EOA in the Venetian area seems to be around 8.5%.

Different animal studies show that several proinflammatory cytokines are essential for natural resistance to specific infections, particularly versus intracellular organisms. However, uncontrolled overproduction of some proinflammatory cytokines, in diseases such as rheumatoid arthritis, can be just as dangerous to the host as the absence of the same cytokines. Reduction in the production and/or activities of proinflammatory cytokines in rheumatoid arthritis remains a therapeutic objective for many patients. The tumour necrosis factor-alpha (TNF-alpha) blockers infliximab, etanercept and adalimumab and the recombinant interleukin 1 (IL-1) receptor antagonist anakinra are effective in patients with active rheumatoid arthritis. However, there is a growing body of clinical evidence that neutralization of TNF-alpha is associated with an increased risk of opportunistic infections, including mycobacterial diseases. Blockade of IL-1 activity with the IL-1 receptor antagonist (IL-1Ra) appears, at present, to be relatively safe. Postmarketing experience and pharmacovigilance programs are necessary to determine the overall safety profile of the new agents. At this time, treating physicians must weigh carefully the benefits of biologics against their safety, particularly in patients at risk of infection.

Progress in the knowledge of pathogenic mechanisms and a better definition of the disease, together with the availability of new technologies, have recently improved the value of laboratory investigations in osteoarthritis (OA). The main objectives of these findings are early diagnosis, assessment of disease activity and severity, and evaluation of therapeutic effects. In this context, biochemical markers are potentially useful, as they are non-invasive and non-expansive. However, among the numerous substances increasingly proposed for these purposes, very few may be considered as true disease markers in OA; COMP, antigenic keratan sulphate, hyaluronic acid, YKL-40, type III collagen N-propeptide and urinary glucosyl-galactosyl pyridinoline seem to be the most promising. However, serum or urinary determinations of these molecules are difficult to interpret adequately, due to their complex metabolism. Careful analysis of synovial fluid, mainly directed to leukocyte count and crystal detection, is still essential for diagnosis, but also for the evaluation of the levels of important markers of local inflammation, such as metalloproteinases and cytokines, which seem to be crucial in the pathogenesis of OA.

We describe the emergence of arthritis following a physical trauma, in a young man with clinical and histopathologic features of primary anetoderma (Jadassohn type) of 13 years' duration. Diagnosis of post-traumatic arthritis in a young patient with genetic predisposition was assumed. Indeed septic arthritis and other possible cause of arthritis were ruled out.

Objective: To investigate serum and salivary neopterin and interferon-gamma as possible markers of immune system activation in primary Sjögren's Syndrome (pSS)., Methods: Serum and salivary neopterin and interferon-gamma concentrations were determined in 30 untreated patients with pSS and matched with several other clinical and laboratory parameters., Results: The mean concentration of neopterin was significantly higher in pSS patients (8.12+/- 3.36 nmol/L in serum and 9.50 +/-7.61 nmol/L in saliva) than in normal controls (p<0.05). Significant correlations were found between serum neopterin and beta2-microglobulin, serum IgG as well as lip biopsy score. Salivary neopterin concentration was inversely related to Shirmer-I test, tear break-up time and stimulated salivary flow rate. Serum and salivary levels of interferon-gamma were normal and no correlation with the other parameters was found., Conclusion: In pSS patients serum neopterin may represent a useful marker of cell-mediated immunity. On the other hand, salivary neopterin seems to reflect theglandular damage.

Henoch-Schönlein purpura (HSP) is considered to be a small blood vessel systemic vasculitis. Numerous microorganisms have been implicated in triggering HSP. We describe an unusual case of HSP with glomerulonephritis and renal failure requiring haemodialysis in a young adult man who subsequently developed paroxysmal nocturnal haemoglobinuria (PNH) with several haemolytic episodes. Bacterial infections, especially those of the respiratory and urinary tract, might trigger both the diseases.

For many years, Sjögren's syndrome was a purely descriptive diagnosis of symptoms such as xerostomia and dry eye (sicca syndrome). The different classification criteria proposed for Sjögren's syndrome comprise a rather variable spectrum of diagnostic possibilities, at one extreme of which we find an array of exclusively objective parameters while, at the other extreme, the objective parameters and patients' symptoms balance out. Improved accuracy in the diagnosis of Sjögren's syndrome can be attained only through the combination of a symptoms questionnaire, histopathology, scintigraphy or sialography or evaluation of the unstimulated salivary flow and specific autoantibodies. In these last few years, further methods have been proposed to increase diagnostic accuracy: the analysis of various salivary constituents, saliva and tear ferning tests, the search for new autoantigens and, above all the use of ultrasonography and magnetic resonance imaging. Color-power Doppler and magnetic resonance sialography have recently been proposed as promising techniques to improve sensitivity and diagnostic specificity. This study discusses the data present in the literature and personal experience regarding diagnostic methods in a group of 350 patients affected by primary Sjögren's syndrome.

Objectives: Arthralgia is among the most frequent musculoskeletal symptoms occurring in patients with chronic lymphocytic thyroiditis (CLT), often causing pain and physical impairment over extended periods of time. Our study aims to characterize arthralgia and, in particular, polyarthralgia (PA) associated with CLT and to evaluate the influence of thyroid replacement therapy., Methods: Of 130 patients affected with CLT attending the Division of Rheumatology for rheumatic complaints, the authors sequentially selected 20 patients with PA without any known rheumatic diseases. Of these 20 patients, 8 were euthyroid, 2 hyperthyroid, and 10 hypothyroid. The last group had never undergone therapy for thyroid disease; then, they were treated with thyroxine and followed up for 24 months. Clinical assessment included the number of joints affected by pain (NAJ), the visual analogic scale of pain (VAS), the duration of PA (number of days), and the intake of acetaminophen., Results: At baseline, the only correlations in the 20 patients with PA were between NAJ and antithyroid microsomal antibodies (r =.782; P <.001), thyroid stimulation hormone (r =.651; P =.001), and erythrocyte sedimentation rate (r =.511; P = 0.021), respectively. During follow-up, although symptoms improved in the 10 hypothyroid patients treated with thyroxine, a statistical significance was reached only after 12 months., Conclusions: Patients with CLT may be affected with PA severely, even in absence of hypothyroidism. Replacement therapy with thyroxine in hypothyroid patients with CLT induced a progressive but gradual improvement of symptoms., (Copyright 2002, Elsevier Science (USA). All rights reserved.)

Infliximab is a chimeric anti-tumor necrosis factor alpha (anti-TNF-alpha) monoclonal IgG1 antibody successfully used for the treatment of active rheumatoid arthritis (RA) not completely controlled with methotrexate or other disease modifying anti-rheumatic drugs. We evaluated both clinical efficacy and safety of infliximab in 63 patients with persistently active RA (Disease activity score > or = 3.7). All the patients received infliximab (3 mg/Kg) at week 0, 2, 6 and then every 5 weeks in combination with methotrexate (7.5-10 mg/week) in an open label study. At week 14th, ACR 20% response criteria have been fulfilled by 43 (91.4%) out of 47 patients, 31 (72%) of them achieving also an ACR 50% and 9 (21%) an ACR 70% response. At the time of this report 33 patients touched 22 weeks of treatment: ACR 20% response was achieved in 95%, while ACR 50% and ACR 70% were respectively found in 78% and 39% of the cases. Only 1 case of bronchopulmonary mycosis and 2 of mild urticaria were observed. The initiation of infliximab therapy in patients with active RA resulted in a rapid and sustained improvement of articular manifestations and quality of life. Even though, major adverse events were rare, clinicians should be aware of this possibility.

Background: We studied the prevalence of fibromyalgia in 3 different groups of patients affected respectively with systemic lupus erythematosus, systemic sclerosis (scleroderma) and primary Sjögren's syndrome. The typical fibromyalgia findings encountered in these diseases were examined., Methods: We enrolled 250 consecutive outpatients: 100 with systemic lupus erythematosus, 50 with systemic sclerosis, 100 with primary Sjögren's syndrome and 2 control groups (30 healthy subjects and 75 patients with primary fibromyalgia). Fibromyalgia features were evaluated by algometry, VAS for pain, Mc Gill Pain Questionnaire and Fibromyalgia Impact Questionnaire., Results: Fibromyalgia has been found in 1 case (1%) with systemic lupus erythematosus, 1 case with systemic sclerosis (2%), 22 cases (22%) with primary Sjögren's syndrome and in 1 (3.3%) of the healthy controls. The number of tender points was significantly higher (p<0.01) in the patients with Sjögren's syndrome in comparison with the other groups. Fibromyalgic findings were similar in the patients with primary fibromyalgia and Sjögren's syndrome with fibromyalgia, unless for both poor sleep and low algometric thresholds which were more frequently found in primary fibromyalgia (respectively p<0.001 and p=0.05)., Conclusions: Our study suggests that fibromyalgia is relatively frequent in primary Sjögren's syndrome, while in systemic lupus and systemic sclerosis its prevalence is not different from that found in the healthy controls. Typical fibromyalgia findings, except algometric values, were similar between the cases with Sjögren's syndrome plus fibromyalgia and fibromyalgia alone.

Objective: To assess the prevalence of fibromyalgia in primary Sjögren's syndrome and to evaluate the clinical differences between patients affected with both primary fibromyalgia and primary Sjögren's syndrome and those affected only with primary fibromyalgia., Methods: Clinical features of fibromyalgia were evaluated in 100 consecutive outpatients with primary Sjögren's syndrome and, as controls, in 90 patients with non-insulin-dependent diabetes mellitus, in 75 patients with primary fibromyalgia and in 30 healthy subjects., Results: Fibromyalgia was recorded in 22% of patients with primary Sjögren's syndrome, in 12.2% with diabetes and in 3.3% of healthy controls. In the primary Sjögren's syndrome group the prevalence was significantly higher than in healthy controls (P < 0.01), but not significantly different than in diabetes. Moreover, primary Sjögren's syndrome with fibromyalgia and primary fibromyalgia patients did not differ with respect to the number of tender points, while the mean pain threshold was lower in the latter (P = 0.05). Purpura, hypergammaglobulinemia, rheumatoid factor, and a focus score > or = 1 on lip biopsy were significantly more frequent in primary Sjögren's syndrome patients without than with fibromyalgia., Conclusions: As recently reported by other authors, our study confirms the moderate increase of fibromyalgia prevalence in primary Sjögren's syndrome. Typical fibromyalgic findings are quite similar to those of primary fibromyalgia, but surprisingly, primary Sjögren's syndrome patients with fibromyalgia show a less severe global involvement than those with primary Sjögren's syndrome alone.

Nasopharyngeal carcinoma has long been reported as the predominant type of cancer associated with dermatomyositis in many several Asian countries, including Hong Kong, Singapore, and Southern-Cina. Dermatomyositis is one of the idiopathic inflammatory myopathies showing characteristic cutaneous manifestations. Reviews from the western literature have demonstrated that certain cancers, such as ovarian and breast carcinoma in women and lung and prostate carcinoma in men, are highly associated with DM relative to the general population. We report the case of a Caucasian Italian patient with nasopharyngeal carcinoma and dermatomyositis. Considering the rarity of nasopharyngeal carcinoma among whites, both the detection and the report of each new case are noteworthy in defining the geographic and ethnic distribution of this tumor.

There are only few studies, regarding primary Sjögren's syndrome (pSS) long-term clinical course. Moreover, it has been often studied in a restricted number of patients, employing different recruitment and diagnostic criteria. During a 10 years follow-up, we longitudinally evaluate clinical course as well as severe complications and mortality rates in 68 patients with pSS, diagnosed according to the Fox's criteria. Patients were divided into 2 groups according to the autoantibodies pattern detected at the diagnosis: anti-Ro and/or La positive and anti-Ro La negative. Glandular manifestations of pSS were distinctively present in the majority of patients already at time of the diagnosis and serological findings remained typically constant during the whole follow-up. Increased IgG, IgA and ESR as well as low C4 serum levels were significantly prevalent in the Ro and/or La positive group. Finally, we did not found any significant increase in the mortality rate of pSS patients in comparison with the general population.

To date there is no agreement as to which imaging technique is best for the evaluation of the oral component of primary Sjögren's Syndrome (SS). The purpose of the present study has, therefore, been to determine the reliability of Magnetic Resonance (MR) in the evaluation of salivary alterations in patients with SS. The study involved 23 patients suffering from SS according to the European criteria. All the patients underwent ultrasonography and MR of the major salivary glands, parotid sialography and biopsy of the minor salivary glands. The first control group was made up of 50 healthy subjects who underwent parotid ultrasonography. The second control group comprised 23 subjects who underwent MR of the head and neck for other non parotid pathology. The ultrasonography, MR and sialography images were evaluated by a single observer during a single session and scored from 0 to 4. In the SS patients ultrasonography was abnormal in all 23 cases (100%): 3 patients showed grade 1 alterations (13%); 5 grade 2 (21.7%); 9 grade 3 (39.1%); 6 grade 4 (26.1%). In the healthy controls, grade 0 was found in 36 subjects (72%) while the remaining 14 subjects revealed grade 1 alterations (28%). Using MR imaging only one of the SS patients showed grade 0 alterations (4.3%), 7 showed grade 1 alterations (30.4%), 9 grade 2 (39.1%), 5 grade 3 (21.7%) and only 1 grade 4 (4.3%). MR imaging sensitivity was 95.8% while specificity was 100%. For ultrasonography, considering grade 1 as non pathological, we found a sensitivity of 88.4% and specificity of 100%. The MR score for SS patients was compared to that obtained with sialography and ultrasonography. There was a good correlation between MR and sialography (r = 0.528, p = 0.010) while the correlation between MR and ultrasonography was not statistically significant. This study confirms that, of the diagnostic procedures available for evaluation of salivary gland involvement in SS, the most useful initial examination is ultrasonography. When there is some doubt or there are subtleties, MR is a valid alternative to classical sialography.

Most of the salivary glands diseases are characterized only by a few distinct clinical patterns. Medical history and clinical examination are still considered of great relevance. However, in order to obtain a definite diagnosis, imaging techniques are required in most of the cases. Salivary glands ultrasonography (US) is the technique to be used as the first because US can easily differentiate calculosis, inflammatory diseases and tumors. Sonography is also frequently needed to perform needle aspiration or biopsy (FNAC). Sialography should be used essentially for assessing chronic sialoadenitis as well as Sjögren's syndrome. At present, Magnetic Resonance sialography should be preferred because of the greater sensibility in diagnosing inflammatory diseases of the salivary glands. It allows to evaluate both intraglandular oedema and nodules, so that incannulation of the salivary duct is not required. Computer Tomography (CT) and Magnetic Resonance imaging (MR) are useful when neoplasm are suspected, particularly if deep areas of the gland, which cannot be visualized by US, are involved. Sequential scintigraphy is currently employed for assessing the functional status of all the 4 major salivary glands and evaluating the chronic evolution of glandular damage.

In spite of all recent years' international meetings, the question of diagnostic criteria of primary Sjögren's syndrome (pSS) is still under debate. The aim of our study is to define sensitivity, specificity and diagnostic accuracy of 3 sets of criteria: those of the European Community Study Group (ECSG), those proposed by Fox, and those proposed by Daniels. We considered 219 subjects complaining of dry mouth and/or dry eyes and/or parotid swelling, evaluated for pSS. The following parameters were considered golden standard for the diagnosis of pSS: focus score > or = 2 foci/mm2, double positivity for SSA and SSB antibodies, and a sialographic grade > or = 2. Our study demonstrates that ECSG criteria show a high sensitivity and a good specificity, resulting in a diagnostic accuracy similar, and sometimes higher, than that obtained with Fox and Daniels' criteria.

The hallmark of SAPHO Syndrome (Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis) is osteitis of the anterior chest wall, which can be so pronounced as to cause thoracic outlet syndrome, thrombosis of the subclavian vein, and compression of the superior vena cava. Suggestive skin manifestations, namely palmoplantar pustulosis and severe acne, generally antedate the bone and joint lesions. We report a case of SAPHO syndrome that went unrecognized for many years and eventually caused compression and thrombosis of the right subclavian vein requiring cleidectomy. SAPHO syndrome should be considered in every patients with anterior chest wall inflammation, particularly when skin lesions are also present.

Objective: Ten new cases with primary Sjögren's syndrome (pSS) whose disease began before age 16 are described. Special attention is paid both to the follow-up and treatment of this condition., Methods: Cases with juvenile pSS were retrospectively identified from our series of 180 pSS patients. Ocular, salivary, and extraglandular manifestations as well as a full laboratory evaluation including HLA-DR typing were retrieved., Results: A disease prevalence of 5.5% (10 cases, 8 female and 2 male) was found in our series. The mean age at onset was 11.0 years, but the disease started at the age of 4 in 2 patients. At onset, parotid swelling was found in 6 cases and extraglandular manifestations in 3. Throughout the follow-up period (mean 48.6 months from the time of diagnosis), the clinical picture was similar to that of pSS in adults, but oral involvement was generally milder. Extraglandular manifestations were always present but never severe. Pertinent laboratory abnormalities (e.g. rheumatoid factor, polyclonal hypergammaglobulinemia, leukopenia, increased ESR, ANA and anti-SSA/SSB antibodies) were found in all patients. Specifically, ANA and anti-SSA were always positive. Moreover, in our cases histocompatibility antigens HLA-DR3 and DR 52 were closely associated with the disease. Clinical outcome was difficult to predict; however, no serious complications have been observed so far. We obtained good results with low-dose steroids and/or hydroxychloroquine, especially with regard to the extraglandular manifestations and laboratory abnormalities., Conclusion: We confirm that juvenile pSS is not a rare condition. It closely resembles pSS in adults except for the extremely high prevalence of recurrent parotitis and immunological findings.