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We present an additional case of focal myositis which, after surgical excision of the muscular mass, did not evolve to generalized polymyositis. To our knowledge immunological evaluations of this disease have never before been carried out. Immunohistochemical analysis of the muscular mass showed the presence of activated endothelial cells, CD4 and macrophage cells in the perivascular and endomysial areas, suggesting an immune-mediated mechanism of muscular damage. At the same time the normal distribution of the peripheral blood lymphocyte subpopulations and the normal levels of serum IL-1 beta, IL-6, TNF-alpha, soluble IL-2R and soluble CD8 underline the non-systemic nature of the disease.

To evaluate the prevalence of psoriatic arthritis (PsA) in Italian patients with psoriasis and to compare the Moll and Wright criteria, the European Spondylarthropathy Study Group (ESSG) criteria, and Amor criteria when applied to this patient population.We examined 205 unselected patients with psoriasis. The diagnosis of PsA was based upon the clinical experience of a rheumatologist. After, we tested these classification criteria. HLA class I and II antigens were also analyzed.Thirty-six percent (75) of psoriatic patients were considered by a clinical expert to have PsA. Moll and Wright criteria identified 46 patients (22%) with PsA; 49 patients (24%) were identified by ESSG criteria and Amor criteria; 12 patients identified by Amor criteria but not by ESSG criteria presented enthesitis or dactylitis; 10 patients identified by ESSG but not by Amor criteria had peripheral synovitis. In patients with peripheral arthritis and psoriasis, the evaluation of NSAID response was critical to fulfilling Amor criteria. However, it was not easy to retrospectively evaluate NSAID response using these criteria. The sensitivity was low for each of the 3 classification criteria (from 61 to 65%), whereas the specificity was high (from 99 to 100%).Our study confirms a high prevalence of PsA among an unselected population of Italian patients with psoriasis. Our data reveal the inadequacy of the existing criteria for PsA, including the ESSG criteria and Amor criteria for the classification of spondyloarthropathy.

Soluble CD4 (sCD4) and sCD8 were measured in the sera of 19 patients with active polymyalgia rheumatica (PMR).We correlated the results obtained with lymphocyte subpopulations, soluble interleukin 2 receptors (sIL-2R), and clinical and laboratory variables at diagnosis. In addition 15 patients were prospectively studied during a 6 month period of prednisone therapy. Assays of the sCD4 and sCD8 molecules and of the sIL-2R were performed using an enzyme-linked immunosorbent kit.Serum sCD8 and sIL-2R levels were significantly elevated in patients with active disease compared to normal controls, while serum sCD4 and the relative percentage of CD8+ T cell levels decreased. In the 15 patients prospectively studied sCD8 levels fell significantly after 1 week of therapy along with the remission of clinical disease and normalization of erythrocyte sedimentation rate. At the end of the study period, sCD8 values did not differ from normal controls and they were significantly reduced compared to baseline values. CD8+ lymphopenia persisted at the end of the study. sCD4 levels remained significantly lower during the study period. sIL-2R levels fell significantly at the end of the study period. However, the 6-month levels of sIL-2R remained significantly higher compared to controls.The rise of serum sCD8 levels observed in patients with PMR with active disease suggests an early activation of CD8 T cells. The therapeutic effect of steroid in PMR may be partially mediated by its effect on CD8 activated cells.

38 untreated patients suffering from rheumatoid arthritis (RA) were studied to evaluate the relationship between serum sIL-2R levels and laboratory and clinical indexes of disease activity and circulating lymphocyte subpopulations. Furthermore, we serially analyzed the correlation between the clinical response to oral gold (Auranofin) treatment and serum sIL-2R levels in 28 RA patients.Patients received a complete rheumatological examination at entry and every 3 months during the study. A complete biochemical analysis was executed every month. Serum sIL-2R levels were evaluated before entering and at the 3rd and 6th month by ELISA: Phenotype analysis of peripheral blood mononuclear cells was performed by a two-color technique using the association of specific monoclonal antibodies. Samples were analyzed by a FACS-scan 440 cytofluorimeter with a single Argonion laser.Serum sIL-2R were significantly higher in RA patients than in controls and had a significant negative correlation with disease duration and a positive correlation with serum IgG and C3 levels. A significant positive correlation was found between serum sIL-2R levels and circulating CD3 + HLADR+, CD3-HLADR+ and CD20 + CD5-cells. After 6 months of auranofin therapy no differences in serum sIL-2R in comparison with basal levels were found in either responders or in non-responders.Serum sIL-2R level is not a good index of activity in RA patients and is not a useful marker of response to AU therapy.

Low molecular weight IgM (LMW IgM), the monomeric subunit of pentameric IgM, was measured in the serum of 27 patients with primary Sjögren's syndrome. LMW IgM was also measured in a control group consisting of 24 patients with psoriatic arthritis (PA) and 8 patients with active rheumatoid arthritis (RA). LMW IgM was found in the majority of patients with primary SS (63%) and those patients had a longer disease duration than those without SS (80.7 +/- 43.9 months vs 37 +/- 18.3, p = 0.01). Although the differences were not significant, SS patients with LMW IgM showed higher rates of: seropositive disease (71% vs 50%), anti-Ro (59% vs 30%) and anti-La antibodies (12% vs 0%), extraglandular involvement (76% vs 60%) and raised gammaglobulins levels (47% vs 30%) compared to those without LMW IgM. Our only two patients with active RA and vasculitis had LMW IgM. None of the patients with PA showed LMW IgM, regardless of their Kammer subgroup classification, disease activity or radiological evidence of erosions. The presence of LMW IgM in a high percentage of patients with primary SS appears to be the expression of a dysregulation of B cell state that may predispose these patients to developing malignant lymphoproliferation.

Twelve patients with psoriatic arthritis (PsA) and very active articular disease resistant to conventional second line therapy entered into a 6-month open study of cyclosporine A (CsA) at a starting dosage of 3 mg/kg/day. Comparisons of phenotypic characteristics of lymphocytes and response to mitogens of peripheral blood mononuclear cells (PBMC) were made between these patients with PsA before CsA therapy, 7 patients without prior 2nd line therapy, 14 untreated patients with psoriasis alone, and 61 healthy controls. We confirmed a significant reduction of the basal percentage of CD8+ cells and an increase in the CD4/CD8 ratio in patients with PsA before CsA therapy compared to controls. These abnormalities were not present in patients with PsA without prior 2nd line therapy and in patients with psoriasis alone. Peripheral blood activated T cells (CD3+, HLA-DR+), natural killer (NK) (CD3-, CD16+ and/or CD56+), total B and CD5+ B cells were decreased only in patients with PsA before CsA therapy. The reduction of non-MHC restricted cytotoxicity T (CD3+, CD16+ and/or CD56+) was observed in all the 3 groups of patients compared to controls. After the 6 months of CsA therapy we observed a significant increase of CD3+, HLA-DR+, CD3+, CD16+ and/or CD56+, total B, and CD20+, CD5+ cells in the 11 patients with PsA compared to pretreatment values. Contrary to azathioprine, CsA does not impair the NK cell population which has a protective role against cancer and viral infections.

A case of chronic neutrophilic leukemia (CNL), a rare myeloproliferative syndrome associated with monoclonal gammopathy of uncertain significance (MGUS-Type IgGk), is reported. Karyotypic study, carried out on bone marrow, excluded Philadelphia-pos. chronic myeloid leukemia (CML) and showed Y loss (45 XO). Only a few cases of CNL with paraproteinemia have been reported, but no case of associated karyotypic abnormalities and paraproteinemia has so far been described.

Serum levels of soluble interleukin 2 receptors (sIL-2R) were measured in 21 patients with polymyalgia rheumatica (PMR)/giant cell arteritis (GCA) prior to steroid treatment. These levels were significantly elevated in patients with PMR/GCA compared with healthy controls (p = 0.002). A significantly longer duration of morning stiffness (p = 0.005) was observed in patients with a high concentration of sIL-2R. A significant correlation was observed at diagnosis between sIL-2R and erythrocyte sedimentation rate (ESR) (p = 0.01) and between ESR and C-reactive protein (CRP) (p = 0.005). We investigated prospectively a group of 10 patients over a period of 6 months of prednisone therapy. At the end of the study sIL-2R levels fell significantly compared to pretreatment values (p = 0.02), but remained significantly higher compared to controls (p = 0.02). ESR and CRP values also fell significantly compared to pretreatment levels (p = 0.0001 in both cases). We observed a significant correlation between the decrease in ESR values and the decrease in sIL-2R and CRP levels after 6 weeks (p = 0.01 in both cases) and after 6 months of therapy (p = 0.002 and p = 0.05). sIL-2R may be considered a useful serologic marker for monitoring response to steroid therapy in patients with PMR/GCA. This laboratory variable correlated more closely with ESR than with CRP. The presence of elevated levels of sIL-2R is likely to reflect T cell activation occurring in PMR/GCA. T lymphocyte activation persisted after 6 months of steroid therapy, despite rapid and continuous control of disease manifestations.

Twelve patients with psoriatic arthritis (PsA) were treated with low doses of cyclosporine A (CsA) (the initial dose was 3 mg/kg daily) and were entered into an open 6-month study. At the end of the study arthritis was improved in 7 patients (number of patients achieving a 50% or more reduction in the number of swollen or tender joints) and unchanged in 4 patients. Cutaneous psoriasis also improved significantly as shown by psoriasis area and severity index score. Only one patient withdrew from the study after one month because of severe nephrotoxicity. Serum creatinine fell to baseline value 6 weeks after the discontinuation of CsA. Three patients had minor side effects. CsA maintained articular improvement also in the 9 patients who are still taking this drug (mean duration of therapy of 12 +/- 0.8 months). There was no significant reduction of erythrocyte sedimentation rate during the study period. We assayed levels of soluble interleukin 2 receptors (sIL-2R) in serial serum samples obtained from 10 patients during the study period. Concentrations of sIL-2R were significantly increased in patients with PsA compared to controls. In 6 responder patients we observed a parallel decrease in joint pain/tenderness score and serum sIL-2R values. This finding was not observed in 4 nonresponders. Our results suggest that low dose CsA is a short term effective and safe therapy in patients with PsA and that serial sIL-2R levels are a useful means of measuring changes in disease activity.