The strongest predictive factor for the development of interstitial lung disease (ILD) in myositis (IIM) patients is the presence of different antisynthetase antibodies. The aim of this study was to compare the clinical characteristics, radiological findings and therapeutic response between the anti-SS-A positive and negative antisynthetase syndrome (ASS) patients. A prospective study of 315 IIM patients was conducted including 27 anti-Jo-1 positive ASS patients. Mean disease duration was 46.6 (range 4-198) months. All patients fulfilled the classification criteria for IIM. All patients underwent chest radiography, pulmonary function tests and HRCT at he time of diagnosis and 6 months after the immunosuppressive therapy. Routine laboratory tests, RF, ANA, anti-ENA, anti-SS-A, anti-histidyl-transfer RNA antibody (Jo-1) measurements were performed in all patients. ILD was found to be present in 70.4% of ASS patients. The anti-SS-A negative ASS group had a more frequent association with alveolitis and responded well to immunosuppressive therapy (p < 0.05). HRCT scan showed more fibrosis in the SS-A positive group. 15.8% of patients died due to pulmonary or cardiac complications. In conclusion, coexistence of anti-SS-A and anti-Jo-1 antibody may be a good predictor for a more coarse and severe ILD in IIM patients who require a more aggressive approach in therapy.

OBJECTIVE:Interstitial lung disease (ILD) may be a characteristic, often serious, manifestation of mixed connective tissue disease (MCTD). In this retrospective study, the frequency and clinical picture of ILD were determined in patients with MCTD using two diagnostic tests: high-resolution computed tomography (HRCT) and inhaled aerosol clearance times of (99m)Tc-labelled diethylene-triamine pentaacetate ((99m)Tc-DTPA). In addition, pulmonary function, effects of therapy and a variety of immunoserological markers were also assessed.METHODS:One hundred and forty-four consecutive patients with MCTD were selected from the clinic, irrespective of the presence or absence of ILD. All patients underwent a detailed clinical assessment, chest HRCT scanning, chest radiography, inhaled aerosol of (99m)Tc-DTPA clearance times, and all pulmonary function tests. Patients who had active ILD received corticosteroid (CS) or CS in combination with cyclophosphamide (CPH). All investigations were repeated after 6 months of immunosuppressive therapy.RESULTS:Ninety-six out of 144 MCTD patients (66.6%) had active ILD, 75 of this group (78.1%) showed ground glass opacity, 21 patients (21.8%) ground glass opacity with mild fibrosis with HRCT. Forty-five patients with active ILD received 2 mg/kg/day CS for 6-8 weeks alone and 51 patients CS in combination with CPH (2 mg/kg/day). Six months later, after therapy, 67 out of 96 MCTD patients with ILD (69.8%) showed a negative HRCT pattern, ground glass opacity with mild fibrosis developed in 15 patients (15.6%), and fibrosis was detected in 13 patients (13.5%). Only one patient showed subpleural honeycombing. (99m)Tc-DTPA was rapid in all 96 MCTD patients with active ILD (28.7 +/- 8.2 min, normal value >40 min). After therapy the (99m)Tc-DTPA was normalized, 79 out of 96 patients (82.3%). Carbon monoxide diffusion capacity (DLCO) was reduced in 33 out of 96 MCTD patients with active ILD (34.3%), while there were no significant differences in the pulmonary function tests between the active versus inactive stage of ILD or versus patients without ILD. The sera of 96 MCTD patients with active ILD contained a high level of immune complexes (ICs), and the total haemolytic complement levels (CH50/ml U) decreased. After 6 months of therapy, the IC levels decreased and CH50/ml levels normalized (MCTD patients before and after active ILD: IC optical density = 355 +/- 227 vs 206 +/- 92, P<0.001; CH50/ml, 38.0 +/- 12.6 U vs 64.3 +/- 13.0 U, P<0.001).CONCLUSIONS:HRCT is the gold standard for diagnosis of ILD. However, we used another method, (99m)Tc-DTPA, in order to compare this technique with HRCT. This latter technique has not been studied previously in MCTD. The elevated levels of IC and increased complement consumption indicated that IC-mediated alveolocapillary membrane damage and tissue injury might play a role in the pathogenesis of ILD in MCTD.

Among the 193 patients (82 female, 111 male) treated primarily for Hodgkin's disease at our clinic between 1990 and 2001 and followed up until 2003, 42 (22%) had mediastinal bulky tumours (MBTs) by the Cotswolds criteria. The rate of MBT diagnosis was significantly greater in the early stage of the disease, these patients were younger and--in contrast to the other group--they all received combined therapy. No significant differences were found in the overall and relapse-free survival rate in the two groups, but relapse and death rates were lower in the patients with bulky tumours. Of the total number of patients, 27 underwent a total of 31 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) examinations, mainly for the evaluation of post-treatment residual mass viability. In the 12 positive cases, the majority of patients received further therapy. During the mean follow-up time of 58 months (range 5-98 months) after obtaining negative results, progression of the disease was found in 2 cases 14 and 23 months later, respectively. Based on our results, we conclude that FDG-PET examinations show a good correlation with clinical follow-up results.

Background. Patients with inoperable esophageal cancer have poor life expectancy without different types of palliation. The aim of this study was to compare stenting methods and investigate the complications and results.Methods. The endoscopic intubation was introduced in 1992 and the Rusch Polyflex stent became available from 1998. During this period 84+14 patients underwent palliation with Tygon and Polyflex prostheses. 53+10 patients had esophageal carcinoma. 21+21 had tumors of the cardia and 10+2 had pulmonary carcinoma. Among them 18+5 patients had fistula.Results. The swallowing ability improved in all cases and the bronchoesophageal fistulas were occluded. Early complications: perforations 7, bleeding 3+1. Two patients (2%) died. Late complications: food impaction 7, tumor overgrowth 6+2, tube migration 4+1. We had no Polyflex stent-insertion related complication.Conclusions, The risk of perforation during endoscopic intubation is high, especially in cases of tumors of the distal esophagus. Intubation with the Polyflex stent using fluoroscopic control is a safer procedure. Self-expanding stents have wider indications. The outcomes of treatments of fistulas are better when the placement of a Tygon tube is difficult or technically impossible. The improvement of the quality of life is as important a result as the improvement of the survival time due to better nutrition and prevention of aspiration.

The cell distribution and function of alveolar macrophages and T lymphocytes were investigated in the bronchoalveolar lavage (BAL) of 15 patients with systemic sclerosis (SSc). In alveolar macrophages, both spontaneous and PMA-stimulated TNF-α production were increased in SSc. PMA-induced IL-6 production was also elevated. Spontaneous IL-6 excretion of scleroderma alveolar macrophages was similar to the controls. Yeast and C3b-coated yeast (opsonised yeast) phagocytosis, chemotaxis and Fc receptor activity of alveolar macrophages were normal. The proportion of CD3, CD4 and CD8 T-lymphocyte subsets in the BAL was similar to the control values. The lymphocyte blast transformation index of the non-adherent cells deriving from the BAL fluid was markedly decreased.

Two cases of rare, benignant gastric tumors are reported. The suggest that while in the diagnosis of tumors with a mucous membrane involvement endoscopy has doubtless a leading role, tumors not infiltrating the mucous membrane are usually better recognizable by radiological (ultrasonography, computer tomography and double contrast x-ray) methods. An appropriate diagnosis followed by surgical removal of the tumor might result in a complete healing of the patient.

Multiplex lymphomatous polyposis is an uncommon disease characterized by polypoid accumulations of malignant lymphoid cells within the submucosa of long segments of the gastrointestinal tract. Primary gastrointestinal form of disease is referred as an extranodal variant of the entity knows as mantle zone lymphoma. Rarely, this typical lesion may appear as a secondary involvement of the alimentary tract in patients with primary nodal lymphomas. In present article, the clinical, histological features of our two cases of primary nodal lymphoma presenting with gastrointestinal symptoms and secondary involvement of the bowels are discussed. The nodal lymphomas of these patients were classified as a mantle zone and a follicle center cell origin lymphoma. The development of gastrointestinal symptoms occurred 29 and 41 months after the diagnosis. Our cases suggest that lymphomatous polyposis can manifest itself secondarily in patients with nodal lymphomas of not only the mantle cell type. The polypoid lesion might be mediated by lymphocyte homing receptors and the pattern of proliferation can be produced by more than one phenotypically different lymphoma.

OBJECTIVE--To detect myocardial involvement in 16 patients with systemic sclerosis using radiogallium scintigraphy METHODS--Sixteen patients with systemic sclerosis were investigated by myocardial gallium-67 citrate scintigraphy. Echocardiographic studies and (in the positive cases) magnetic resonance imaging were also performed. RESULTS--Increased myocardial gallium uptake was found in five of the 16 patients. None of these positive cases exhibited any 'classical' clinical, electrocardiographic or laboratory signs characteristic of myocarditis. The mean age of the positive cases was 60.8 (SD 5.0) years compared with 44.6 (10.7) years for the negative cases; no difference was detected in the mean disease duration between these two groups. Two of the affected cases belonged to the limited scleroderma subgroup. CONCLUSIONS--In spite of the low number of patients investigated, it is tempting to speculate that elderly patients with a disease onset around 60 years tend to be the candidates in whom a positive gallium-67 scintigraphy test may best indicate a silent myocardial interstitial inflammation.

The aim of the study was to investigate the pulmonary parenchymal abnormalities by high resolution computed tomography (HRCT) in patients with various forms of scleroderma. Three scans were performed sequentially in all cases: one at the level of aortic arch, one at the tracheal carina and one 1–2 cm above the diaphragm. Seventy cases with limited, 21 patients with diffuse cutaneous systemic sclerosis and 10 cases with circumscribed scleroderma were investigated. The 21 patients with diffuse scleroderma included three normal HRCT scan (14%), three with ground glass attenuation (14%), one with ground glass attenuation with fibrosis, three with fibrosis (14%), six with subpleural and five with diffuse honeycombing. The majority of cases with extensive honeycombing of the lungs had anti-Scl 70 autoantibody which was the characteristic antibody of the diffuse scleroderma subset. The 70 cases with limited cutaneous systemic sclerosis were characterized by normal HRCT (43%), ground glass opacity with or without fibrosis (18.6%), whereas fibrosis was detected in 22.9%, subpleural or diffuse honeycombing in 15.7% of the patients. Six of the ten cases with circumscribed scleroderma also showed a fibrosis. Cases with diffuse scleroderma are characterized by the presence of advanced fibrosis, whereas the extent of fibrosis is far less pronounced in limited scleroderma. Mild fibrosis may also be present in circumscribed scleroderma forms.