[Bacteremia and pneumonia due to Yersinia enterocolitica serotype O:3 in an immunocompetent patient]. / Bactériémie et pneumopathie à Yersinia enterocolitica sérotype O:3 chez un immunocompétent.

The authors report a rare case of Yersinia enterocolitica O:3 pneumonia in an immunocompetent 70-year old man. There was no evidence of acute gastrointestinal disease. Diagnosis was confirmed by blood cultures. He responded with resolution of the infection after 21 days of therapy with a third-generation cephalosporin then by cotrimoxazole. Only 15 cases have been reported so far. Most of the patients were immunocompromised. This is the first case in France.

The relationship of anti-endothelial cell antibodies to anti-phospholipid antibodies in patients with giant cell arteritis and/or polymyalgia rheumatica.

Sera from patients with giant cell arteritis and/or polymyalgia rheumatica were tested for the presence of IgG, IgM and IgA antibody to endothelial cells (AEC), cardiolipin (ACL) and phosphatidylethanolamine (APE) using enzyme-linked immunosorbent assays. There were strong correlations between ACL and APE, but also between AEC and ACL IgM (p < 0.02) and between AEC and APE IgA (p < 0.003). Inhibition of AEC binding was achieved by absorption onto EC, but ACL and APE binding was also significantly reduced. In contrast, the binding of AEC antibody could not be inhibited by incubation with CL. Our data suggest that AEC constitute a heterogeneous population of autoantibodies.

Abnormal distribution of CD45 isoforms expressed by CD4+ and CD8+ T cells in rheumatoid arthritis.

CD45RO+ T cells are referred to as memory or helper-inducer while CD45RA+ T cells are regarded as naive or suppressor-inducer T cells. The former population predominates in the peripheral blood and even more in the synovial fluid of patients with rheumatoid arthritis, to the expense of the latter population. Within the CD45RB+ compartment, there appears to be more of the fully-differentiated than of the early-differentiated CD4+ T cells. In spite of the fact that these lymphocytes are close to undergoing apoptosis, this programmed cell death is inhibited in the rheumatoid synovium.

IgA in Sjögren's syndrome.

Patients with Sjögren's syndrome (SS) display two sets of immunological abnormalities. B cells are oligoclonally activated, resulting in hypergammaglobulinaemia, elevated levels of circulating immune complexes (CIC) and non-organ specific autoantibodies. The cellular arm of the immune response is also involved, as shown by the predominance of activated T cells within the exocrine gland infiltrate. IgA could well bridge the gap between activated B cells and defective T cells and by doing so, play a pivotal role in the pathogenesis of SS. This interpretation is supported by the high proportion of IgA in immunoglobulin(Igl) production at the mucosal level. Additionally, IgA is the Igl class most dependent on T cell help. A number of studies over the past 15 years have reported high levels of serum and secretory IgA, IgA-rheumatoid factor and IgA-containing CIC. A correlation between disease activity and the latter abnormalities has recently been shown. There is, however, a need for longitudinal assessment of total IgA and IgA autoantibodies in order to further evaluate their role in the pathogenesis of the disease.

Reappraisal of tests for xerostomia.

Our goal was to establish the practical usefulness of combinations of tests (saliva flow rate, SFR; salivary lysozyme, Lys; salivary lactoferrin, Lf; sialography, SG; salivary gland scintigraphy, SGS; and labial salivary gland biopsy, SGB) for the oral component of Sjögren's syndrome (SS). These tests were applied to 40 patients with primary SS (group A, defined by the presence of keratoconjunctivitis sicca, a positive response to two of three selected questions for xerostomia and the presence of two of four autoantibodies), 16 patients with secondary SS (group B), 16 patients with connective tissue disease but no evidence of secondary SS (group C) and 14 normal controls (to establish the threshold of the six tests). SFR was decreased in 68, 56 and 19% of the patients in groups A-C respectively [sensitivity (sens) 68%, specificity (sp) 81%, positive predictive value (PPV) 90% and negative predictive value (NPV) 50% for primary SS]. Lys was elevated in 3, 0 and 0% of the patients in groups A-C (sens 3%, sp 100%, PPV 100% and NPV 66%). Lf was reduced in 58, 69 and 25% of the patients in groups A-C (sens 58%, sp 75%, PPV 82% and NPV 53%). SG was positive in 74, 27 and 13% of the patients (sens 74%, sp 87%, PPV 93% and NPV 41%). SGS was positive in 75, 63 and 25% of the patients (sens 75%, sp 75%, PPV90% and NPV 45%). SGB was abnormal in 95, 94 and 25% (sens 95%, sp 75%, PPV 90%, NPV 14%).(ABSTRACT TRUNCATED AT 250 WORDS)

Serial measurements of anticardiolipin antibodies in primary Sjögren's syndrome.

Twenty-four out of 54 patients with primary Sjögren's syndrome (SS) were shown to be positive for IgG and/or IgM anticardiolipin antibodies (aCL). Extraglandular manifestations were related to the IgG-, but not to the IgM-aCL. Twenty SS patients were examined over a 3 year period. Of these, 6 displayed a marked increase in IgG- and IgM-aCL and, among them, 4 developed extraglandular manifestations of SS throughout the follow-up.

Polymorphonuclear cell functions in patients with primary Sjögren's syndrome.

Polymorphonuclear (PMN) cell functions were evaluated in twenty-two patients with primary Sjögren's syndrome (SS) and 45 healthy controls. Adherence, phagocytosis, chemiluminescence and bactericidal ability were not significantly reduced in Sjögren's syndrome patients. Chemotaxis and chemokinesis were markedly reduced in SS patients and the results of the former test correlated well with those of the latter. These observations suggest that the decrease in PMN cell mobility is attributable both to a primary cell abnormality and a serum inhibitory effect.

Practical usefulness of IgA-containing immune complex determination in the serum of patients with primary Sjögren's syndrome.

OBJECTIVE: To evaluate the relevance of IgA-containing immune complexes (IC) as a predictor of lymphocytic infiltration of the minor salivary glands, and thus to determine the necessity of the minor salivary gland biopsy as a diagnostic test for primary Sjögren's syndrome (SS) in patients complaining of dryness of the mouth. METHODS: IgA-containing IC, as well as anti-SSA and anti-SSB antibodies, were measured using enzyme-linked immunosorbent assays in 116 consecutive patients presenting with dry mouth but no connective tissue disease. The specificity, sensitivity, and positive (PPV) and negative predictive values (NPV) of these tests were calculated in relation to the results of the minor salivary gland biopsy and to the criteria for primary SS. RESULTS: Sixty-five patients had a focus score > or = 1.IgA-containing IC were detected in 45 of them, compared with five of the remainder (specificity 89%, sensitivity 69%, PPV = 88% and NPV = 69%). When the IgA-containing IC, and the anti-SSA and anti-SSB tests were associated, the sensitivity and NPV were improved (81 and 79%, respectively), while specificity and PPV were maintained (88 and 90%, respectively). CONCLUSION: Given the reliability of this combination of tests for the diagnosis of primary SS, the minor salivary gland biopsy might be indicated only in those patients without any serological abnormality.

IgA glycosylation abnormalities in the serum of patients with primary Sjögren's syndrome.

Since there is no information regarding the glycosylation status of immunoglobulin A (IgA) in patients with primary Sjögren's syndrome (pSS), the sialic acid and galactose contents of IgA1 and IgA2 were evaluated in 17 pSS patients and in 14 normal controls (NC), using new enzyme-linked immunosorbent assays. The proportion of sialylated IgA1 and IgA2 was augmented (p < 0.001 and < 0.05, compared with NC), whereas that of galactosylated IgA1 and IgA2 was reduced (p < 0.01 and < 0.02, respectively). The level of SA IgA1 correlated the amount of IgA-containing immune complexes (p < 0.01), serum IgA (p < 0.01) and IgA-rheumatoid factor (p < 0.01). This demonstrates a number of IgA abnormalities in pSS patients. There were no correlations between SA and Gal, however, nor could any difference be ascribed to extraglandular manifestations.

Cutaneous manifestations of primary Sjögren's syndrome are underestimated.

The association of kerato-conjunctivitis sicca and xerostomia has been termed Sjogren's syndrome (SS). Although this disease is referred to as a non-organ-specific autoimmune condition, the vast majority of the deleterious effects of primary SS are restricted to the exocrine glands. Among them, the lacrymal and salivary glands are at the foreground, owing to the severity of the objective consequences and the importance of the subjective manifestations. As a result, cutaneous manifestations are minimized, albeit relatively common. We have carefully analyzed the literature to draw up an inventory of the possible skin complications of this syndrome. In addition to xerosis and epidermal IgG deposits, they include vasculitis and cutaneous B cell lymphoma. Alopecia, vitiligo and papular lesions have also been reported to be associated with primary SS.

Relations of the histopathology of sublingual and labial salivary glands to the clinical presentation in primary Sjögren's syndrome.

This study was designed to further analyse the histopathologic appearance of the sublingual and labial salivary gland biopsies performed in 50 patients suspected of having Sjögren's syndrome (SS). Three kinds of lesions were evaluated: occurrence of foci (an aggregate of more than 50 lymphocytes), lymphocytic infiltration of the duct walls, and the remaining abnormalities (such as scattered lymphocyte infiltrates, interstitial sclerosis, dilatation and regression of ducts and acini). The occurrence of foci correlates well with SS and even better when there is a lymphocytic infiltration of the ducts within the foci. The remaining changes were not specific for SS.

Monoclonal immunoglobulins in the serum of patients with primary Sjögren's syndrome.

Monoclonal immunoglobulins (M Igl) were detected in the serum of 10 of 20 patients with primary Sjögren's syndrome (prim. SS), by using high resolution electrophoretic technique combined with immunofixation. The finding of three free lambda chains and two IgG lambda suggests that the lambda-expressing B-clones are involved in this process, but the class distribution is different from one series to another, and from one country to another. The prim. SS patients with multiogran disease have the higher incidence of M Igl.

A longitudinal study of lung impairment in patients with primary Sjögren's syndrome.

OBJECTIVE: A longitudinal evaluation of lung involvement in primary Sjögren's syndrome (SS). METHODS: Eighteen non-smoking women fulfilling the European criteria for primary SS were followed for 55 months (range 26-137 mos.). These were consecutive patients with exclusion for current smokers and patients with lung diseases. Every patient underwent clinical examination, chest radiographs and lung function tests (spirography, flow/volume loop and CO lung diffusing capacity measurements). No patient was given any immunosuppressive or mucolytic therapy. RESULTS: Cough, dyspnea on exertion and recurrent bronchitis were observed in 50, 40 and 20% of the patients respectively and their frequency did not change with time. Chest radiographs were and remained normal. At presentation, lung volumes and diffusing capacity were in the normal range, whereas expiratory flows in the small airways tended to be in the low range. With time, the peak expiratory flow (PEF) significantly increased (95.8 +/- 4.6 v 103.5 +/- 4.6, mean +/- SE, % of predicted, Wilcoxon, p < 0.05) whereas the lung transfer factor for CO (TLCO) and the transfer coefficient (KCO = TLCO/alveolar volume) decreased (92.9 +/- 4.0 v 87.0 +/- 4.0 and 89.7 +/- 2.4 v 84.2 +/- 2.6 respectively, p < 0.05 for both). The TLCO decrease, corrected for the duration of follow-up, correlated with the titers of IgA circulating immune complexes (CIC) at presentation and to a lesser extent with the occurrence of cough. CONCLUSION: During the follow-up of these primary SS patients, respiratory symptoms did not change, the recurrent respiratory infection rate was low, and no cases of pulmonary hypertension or lymphoma was observed. The diffusion capacity decrease was associated with IgA CIC titers at presentation. This impairment could contribute to dyspnea during its evolution over a lifetime but is too slight to explain the dyspnea on exertion seen in most of our patients.

Salivary lactoferrin in primary Sjögren's syndrome.

Parotid salivary lactoferrin (LF) levels were measured in 26 patients with Sjögren's syndrome (SS), 19 patients with keratoconjunctivitis sicca (KCS) and 32 normal controls, by using a radial-immunodiffusion technique. The levels of LF were higher in SS than in KCS patients (23 +/- 21, 10 +/- 10, p less than 0.03) and in SS patients than in normals (7 +/- 6 micrograms/ml, p less than 0.001). There was some relationship of the LF level to the labial salivary gland histopathology grade.

Juvenile relapsing periarteritis nodosa and streptococcal infection.

Classic polyarteritis nodosa is a multisystem inflammatory disease associated with necrotizing vasculitis of small and medium arteries. In most cases, the causes of polyarteritis nodosa remain unknown, but viruses (HBV, HCV, HIV) and microbes (especially streptococcus) have been considered as etiologic or contributing factors. A 13-year-old boy was admitted with fever, skin lesions, polyarthritis and muscle involvement. A muscle biopsy demonstrated a necrotizing vasculitis and antistreptolysin titre was tremendously increased. His condition improved following the administration of oral steroids but he experienced relapses 5 and 12 years later when penicillin withdrawal was attempted. The flares were accompanied by a major increase of antistreptolysin titre and response to corticosteroid was obtained. He is currently 38 years old and he remains well on prophylactic penicillin. Polyarteritis nodosa in children may occur after a streptococcal infection. It may be prudent to consider penicillin prophylaxis in patients with periarteritis nodosa when a streptococcal etiology is documented or highly suspected.

[Localized periarteritis nodosa with periostal new bone formation]. / Périartérite noueuse localisée et atteinte osseuse.

INTRODUCTION: Polyarteritis nodosa is a disease process with a wide clinical spectrum, ranging from a severe generalized multisystem disorder to a more benign condition which may remain limited to the skin, muscles and peripheral nerves, and is termed cutaneous polyarteritis nodosa. Periosteal bone formation is uncommon in this condition. EXEGESIS: We report two cases (two female patients of 80 and 55 years of age, respectively) who complained of red, tender nodules of legs and bone pain. Histological changes were those of necrotizing arteritis of the small and medium arteries in the panniculus and dermis. Bone X-ray showed periosteal bone formation. CONCLUSION: In patients with pain, skin changes and swelling of the lower limbs, the diagnosis of periarteritis nodosa with periostitis has to be considered.

[Idiopathic orbital myositis]. / Myosite orbitaire idiopathique.

INTRODUCTION: Orbital myositis is a subtype of nonspecific orbital inflammation confined to one or more of the extraocular muscles. EXEGESIS: Three female patients presented presumed orbital myositis, in each of whom the diagnosis was suspected clinically and confirmed by the computed tomography (CT) scan appearances. All presented with orbital pain, diplopia and periorbital edema. All patients responded to systemic corticosteroid with prompt resolution of symptoms and signs, but one patient had recurrent episodes of myositis, leading to the prescription of azathioprine. The clinical and radiological constellation of findings allowing a definitive diagnosis of the nonspecific orbital inflammatory syndrome in most cases is discussed. CONCLUSION: The nonspecific orbital inflammatory syndromes are a heterogenous group of conditions and the CT scan is a valuable tool in differentiating these conditions. Most cases of idiopathic orbital myositis require corticosteroids, but therapeutic alternatives to corticosteroids in refractory diseases are warranted.

[Muscle-localized tumorlike polyarteritis nodosa]. / Périartérite noueuse musculaire localisée pseudotumorale.

INTRODUCTION: Systemic vasculitis presenting as a tumorlike lesion are unusual and affect mainly the uro-genital system and breast. Muscle-localized tumorlike periarteritis nodosa are scarce. EXEGESIS: We report localized tumorlike polyarteritis nodosa affecting the forearm muscles in a 90-year-old woman. CONCLUSION: Other cases of polyarteritis nodosa localized to muscles are reviewed. This limited form has a benign clinical course and responds promptly to treatment with corticosteroids alone but relapses may occur.