Primary Gougerot-Sjögren syndrome: a dermatological approach.

Gougerot-Sjögren syndrome (GSS) is a chronic heterogeneous non-organ-specific autoimmune disease, encompassing a wide spectrum of clinical manifestations. It is characterized by a lymphocytic infiltration of the exocrine glands, also called epitheliitis, resulting in xerostomia and keratoconjunctivitis sicca. The skin can also be involved; for example, xerosis is a consequence of epitheliitis. Dermatological consequences of polyclonal reactivity are vasculitis and manifestations of B-cell proliferation vary from plasma cell infiltrates to B-cell lymphoma.

[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.

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.

[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.

[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.

[Lymphoma involving the temporal artery]. / Localisation temporale d'un lymphome.

Involvement of the temporal arteries, considered to be the hallmark of giant cell arteritis, is rather rare in other pathologic processes. We describe a patient with lymphoma involving temporal artery. A 68-year-old man has been followed closely without therapy since 1989 for a low-grade non-Hodgkin lymphoma. He presented in 1995 with asymptomatic nodules on the temporal artery with preservation of the pulse. Temporal artery biopsy showed periarterial infiltration of mononuclear cells in keeping with follicular mixed cell lymphoma. The differential diagnosis of temporal arteritis must therefore, include other vasculitides, light chain amyloidosis but also lymphoma and emphasize the need for a temporal artery biopsy.

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.

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.

Enhanced sialyltransferase activity in B lymphocytes from patients with primary Sjögren's syndrome.

Despite the indisputable role of immunoglobulin (Ig)A in the pathogenesis of primary Sjögren syndrome (pSS), the causative abnormality remains largely unknown. As an extension of our report that IgA is oversialylated in this disease, the thrust of the present study was to measure the sialyltransferase (ST) activity in B lymphocytes. ST containing lysates of B cells from 17 pSS patients and 10 controls, were obtained using a combination of detergents, and incubated with affinity purified IgA that had been previously desialylated. The deposition of cytidine 5' monophosphate sialic acid (SA) by ST from B cells onto IgA was detected by two ELISA based upon the use of biotinylated lectins (Sambucus nigra agglutinin which is specific for alpha2-6 SA and Maackia amurensis which is specific for alpha2-3 SA). In parallel, the amount of SA on IgA from ten of the 17 patients and eight of the 10 controls was assayed using the same method. An excess of alpha2-3 and alpha2-6 SA on IgA was found in those patients with excessive activity of alpha2-3 and alpha2-6 ST. Thus, IgA hypersialylation in pSS patients may result from undue activity of ST.

Glycosylation of immunoglobulin A influences its receptor binding.

Immunoglobulin A (IgA), which is heavily glycosylated, interacts with a variety of receptors, e.g. the asialoglycoprotein receptor (ASGP-R), which binds terminal galactose residues, and the Fcalpha receptor (FcalphaRI). It has thus been proposed that elevated serum levels of IgA in primary Sjögren's syndrome (pSS) are caused by its defective clearance. To test this hypothesis, we developed a method (based on sialyl transferases eluted from a hepatoma cell line) to increase the amount of sialic acid (SA) on IgA, and used a battery of IgA1- and IgA2-specific glycosidases to reduce this amount. Binding of IgA1 and IgA2 to ASGP-R and FcalphaRI was found to be sugar dependent because oversialylated IgA bound less than native or desialylated IgA. However, individual sugars did not play a direct role in this binding. Given that IgA are oversialylated in pSS, defective clearance of IgA may indeed be ascribed to an excess of SA in IgA1 and IgA2.

[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.

Changes in glycosylation of immunoglobulins in primary Sjögren's syndrome.

Serum IgG and IgA are glycoprotein and significant glycoform abnormalities have been established in primary Sjögren's syndrome. The proportion of asialylated IgG is abnormally high in the patients, whereas IgA1 and IgA2 appear to be over-sialylated. This peculiarity might explain the defective binding of IgA to asialoglycoreceptors. Furthermore, the activity of alpha 2,6 sialyl transferase is higher in the IgA-producing B cells from the patients than in the controls, whereas the alpha 2,3 sialyl transferase operates in the former cells but not in the latter. The mechanism of this enzyme dysregulation warrants elucidation.

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.

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.