[Pseudo-Sjogren's syndrome in type IV hyperlipoproteinemia. Description of a clinical case]. / Pseudo-sindrome di Sjögren in corso di iperlipoproteinemia di tipo IV. Descrizione di un caso clinico.

A case of type IV hyperlipoproteinemia, obesity, mild diastolic hypertension, xerostomia and bilateral parotid gland enlargement is described. The labial biopsy showed a fatty infiltration, but not inflammatory infiltrates. On the basis of these data, a diagnosis of pseudo-Sjögren's syndrome was made.

Ureteral involvement in the Churg-Strauss syndrome: a case report.

We report a case of the Churg-Strauss syndrome with bilateral ureteral stenosis, secondary obstructive uropathy and anuria. The literature regarding urological implications of this disease is reviewed, and the surgical and medical management of our case is detailed.

[Rheumatic polymyalgia and temporal arteritis. Case contribution and bibliographic review]. / La polimialgia reumatica e l'arterite temporale. Contributo casistico e revisione bibliografica.

Polymyalgia rheumatica (PMR) is a frequent clinical syndrome in the elderly, characterised by morning aching and stiffness in the shoulder and girdle muscles associated with an elevated erythrocyte sedimentation rate and responsive to low-dose corticosteroid therapy. PMR may be associated with temporal arteritis (AT), a systemic granulomatous vasculitis predominantly affecting branches of the carotid artery in patients over the age of 50. Recent studies suggest an immunological pathogenesis for PMR and TA. Personal experience on 56 cases of PMR and TA, and a survey of the literature is reported and the articular manifestations and the differential diagnosis, are underlined. Polymyalgia rheumatica and temporal arteritis. Personal experience and survey of the literature.

Relationship between areas of popliteal cysts and radiological damage in rheumatoid arthritis and in osteoarthritis.

Fifty-five patients, (30 Rheumatoid Arthritis (RA) and 25 Osteoarthritis (OA], with knee synovial effusion and popliteal cysts, visualized through arthrograms, were studied. A relationship was sought between radiological findings and area of the cysts, measured through a millimeter grid. Ten radiological parameters were graded and summed up to obtain a "total knee score". A "total geode score" was also obtained by scoring, separately, the geodes. In addition two specific indexes were used--for comparison--the erosive index, modified after Berens and Lin, in RA and the Kelligren's index in OA. In RA a statistically significant, inverse correlation was found between the x-ray scores and the area of the cysts, while such a relationship was not observed in OA. However, only a third of the cysts accounted for the inverse relationship in RA. Furthermore, two control groups of RA and OA patients revealed a striking association between degree of radiological damage and frequency of popliteal cysts. Therefore, the hypothesis that popliteal cysts might have a protective effect against the articular-bone damage in RA, can be held only in few cases.

[Paraneoplastic polymyalgia rheumatica. Case contribution]. / La polimialgia reumatica paraneoplastica. Contributo casistico.

Polymyalgia rheumatica (PMR) is an inflammatory disease which mainly affects the elderly and is highly responsive to steroid therapy. PMR can be associated with giant cell arteritis as well as with malignancy. Three cases of malignant neoplasms of the digestive apparatus beginning with a clinical picture similar to that of the "idiopathic" PMR, but with poor response to steroid therapy are presented. In one case the primary neoplasm was found only at the autopsy. These observations suggest that a careful clinical evaluation and a long follow-up are necessary for a correct diagnosis of "idiopathic" PMR.

Bronchoalveolar lavage analysis of interstitial lung disease in CREST syndrome.

The bronchoalveolar lavage (BAL) technique was used to characterize inflammatory cells in the lower respiratory tract of seven nonsmoking patients with CREST syndrome and interstitial pulmonary fibrosis. Differential cell counts in the BAL fluid showed a significant increase of neutrophils (p less than 0.05) and eosinophils (p less than 0.01) in comparison to normal subjects. In three patients there was also an increase of lymphocytes. No correlation was found between BAL and duration of scleroderma and/or lung function tests. The BAL findings in these scleroderma patients resemble those of idiopathic interstitial fibrosis suggesting that a similar inflammatory process occurs within the lower respiratory tract.

Toxicity due to remission inducing drugs in rheumatoid arthritis. Association with HLA-B35 and Cw4 antigens.

Twenty-five patients with rheumatoid arthritis (RA) who developed toxicity while taking remission inducing drugs and 30 without toxicity were studied for possible associations with class I and II HLA antigens. A strong association has been found between nephritis and dermatitis due to Tiopronin (a D-Penicillamine like compound) and class I antigens B35-Cw4, and between dermatitis due to gold thiosulphate and B35. Compared to healthy controls a lower DR5 frequency was observed in patients with RA except for the Tiopronin related nephritis group.

Juvenile rheumatoid arthritis in Turner's syndrome.

An increased risk for autoimmune diseases has been recognized in Turner's syndrome (T.S.). However autoimmune rheumatic or connective tissue disorders have not been described. We report here on a 8-8/12 year old caucasian girl with T.S. and Juvenile Rheumatoid Arthritis (JRA). The hypothesis that the association is more than casual is discussed.

Association between DR3 and thrombocytopenia due to gold or tiopronin. Case reports and review of the literature.

Two patients who developed thrombocytopenia while on Tiopronin and gold salts respectively were HLA typed. Their common haplotype was A25(10), B8, DR3. A survey of the literature showed that the association between DR3 and the sudden onset form of thrombocytopenia is striking. A genetic predisposition, besides other unknown factors, seems to play a crucial role.

[Idiopathic hypereosinophilic syndrome. Current clinico-therapeutic state]. / Sindrome ipereosinofila idiopatica. Attualità clinico-terapeutiche.

Idiopathic hypereosinophilic syndrome is a term labelling clinical illnesses characterized by blood hypereosinophilia and widespread infiltration of organs and tissues by mature eosinophils. Although any tissue can be affected the involvement of the nervous system and of the myocardium usually indicates worse prognosis. Histopathological studies carried out on myocardial tissues of hypereosinophilic patients, suggest that eosinophils are the main inducers of tissue damages. These cells seem to affect first the coronary endothelial cells, so leading to thrombi formation and then fibrosis. Eventually restrictive cardiomyopathy may occur. The pathogenetic events are not fully understood. However several studies have been focused on two eosinophil proteins: the major basic protein (MBP) as possible mediator of tissue damages and the cationic eosinophil protein (ECP) involved in thrombotic events. In the bone marrow a marked hyperplasia of the eosinophil series is usual, while in the blood only mature eosinophils circulate. Non specific abnormalities either of the cellular or humoral immunity have been described. Previous reports of a definitely incurable disease have been improved by modern therapeutic programs.

Indomethacin-related serum sickness-like illness with IgM lambda cryoparaprotein?

A unique association of a transient IgM lambda monoclonal gammopathy and a serum sickness-like illness, appearing after a short course of indomethacin, is described. The IgM lambda monoclone did cryoprecipitate along with fibronectin in hypotonic medium and disappeared with the resolution of the clinical picture. Emphasis is given to the role of some foreign antigens in the induction of benign monoclones.

Hypouricemic action of diflunisal in gouty patients: in vitro and in vivo studies.

Sixteen hyperuricemic gouty patients were treated with diflunisal, a novel salicylate, 500 mg BID. Serum and urine uric acid along with uric acid clearances were studied before and after a 7-day treatment. A clear hypouricemic action was observed, but 2 different mechanisms of action were seen when overexcretor patients and normoexcretor patients were analyzed separately. In overexcretors an allopurinol-like action was evident, whereas in normoexcretors a uricosuric action occurred. In vitro experiments showed a competitive inhibition of xanthine-oxidase exerted by diflunisal at low concentrations.

Clinical features, scintiscan characteristics and X-ray progression of late onset rheumatoid arthritis.

The clinical features, serology behaviour, scintiscan characteristics and the degree of X-ray progression according to the disease duration were compared in two RA groups with a disease onset after 65 years of age (LORA) and before 65 (EORA) respectively. No differences emerged in either the clinical findings or in the scintiscan picture. A sub-group of LORA showed acute flares of the illness presenting with very high ESR values and high fever. The erosive index (EI) evaluated in three areas (carpal-bone and wrist, MCP and PIP joints) revealed an undistinguishable degree of X-ray damage either in seropositive or seronegative patients, whereas seropositive EORA fared clearly worse than seronegative ones. A prospective study in two small sub-groups of LORA and EORA revealed a progression of X-ray erosions despite second line drug treatment in both groups, especially in LORA.

Pulmonary involvement in Sjögren's syndrome.

In this study we tried to value the frequency and the characteristics of the physiological abnormalities affecting the lungs in Sjögren's syndrome (SS). We studied 18 female nonsmokers (average age 53 years). The diagnosis has been made on the presence of at least two of the following abnormalities: keratoconjunctivitis sicca (Schirmer's test), xerostomia (scanning of the salivary glands, lip biopsy) and collagen vascular disease. We made the following tests: clinical examination, chest roentgenogram, spirometry, TGV, RAW and SAW valuation, study of the flow-volume curves, diffusion capacity test, bronchoalveolar lavage, bronchial biopsy. The physiological results have demonstrated the presence of a restrictive syndrome affecting above all the small airways (MEF25-32.7%) and a decrease of the diffusion capacity (DLCO-25%). There is, moreover, a constant lymphocytic infiltration of the bronchial mucosa and of the lung's interstitium. In conclusion the pulmonary involvement in SS seems to be constant, unpredictable and of remarkable clinical-physiological importance.

Clinical efficacy and adverse effects of tiopronin in rheumatoid arthritis. Report of a follow-up in 50 patients.

A follow-up of 15.4 months has been carried out in 50 patients suffering from classical or definite rheumatoid arthritis, treated with tiopronin, a new slow acting sulphydrylant agent. The attention was focused mainly on the frequency of the major side effects needing withdrawal of the drug in 20% of the patients; in other 20% of the cases we have observed the disappearance of the major side effects after reduction of the tiopronin dosage. The most important major side effect was renal toxicity. Five cases of proteinuria and among these four cases of nephrotic syndrome were recorded. These side effects disappeared 2-5 weeks after withdrawal of the tiopronin. From a clinical point of view a positive response was observed in 56% and a complete lack of efficacy in 22% of patients. The clinical benefit and a panel of side effects very close to those of D-penicillamine were confirmed.