Spinal cord sarcoidosis: report of seven cases.

BACKGROUND: Spinal cord involvement in sarcoidosis is rare, occurring in <1% of patients with sarcoidosis. METHODS: We report seven cases of spinal cord sarcoidosis, seen in two French hospitals over a 13-year period. Presentation of disease, methods of diagnosis and response to treatment, with quantification according to the reduction of the modified Rankin scale (MRS), were noted. RESULTS: Six patients presented insidious paresthesias or weakness and one a sudden paraplegia. Average MRS at diagnosis was to 2. Spine MRI showed one or several intramedullary lesions in all cases. Diagnosis was confirmed by extra-neural tissue biopsies in all cases, including mediastinoscopy (two patients), coelioscopy (one patient), bronchoscopy (one patient), salivary gland biopsy (one patient) and skin biopsy (two patients). The average follow-up for the group was 49.4 months. All patients responded to corticosteroid therapy with a median reduction of MRS of one point. Five patients received immunosuppressive therapy: cyclophosphamide (two patients), methotrexate (two patients), azathioprine (one patient), mycophenolate mofetyl (one patient), with an inconstant benefit. Patients who received cyclophosphamide presented severe fungaemia. CONCLUSION: Based on our study and literature analysis, we propose an algorithm for treatment of spinal cord sarcoidosis.

[Toxic or drug-induced granulomatous reactions]. / Granulomatoses d'origine médicamenteuse ou toxique.

PURPOSE: To review the current concepts in toxic and drug-induced granulomatous reactions. CURRENT KNOWLEDGE AND KEY POINTS: Granulomatous reactions are induced by various chemical agents, treatments or foreign bodies. According to the breaking way into the organism, the lungs, the liver, the kidneys or the skin are mainly concerned, but systemic granulomatosis mimicking sarcoidosis is possible. Therefore systematic analysis of environmental, occupational and leisure exposures and quest for medical or illicit drugs is mandatory to identify the responsible agent. Over the recent period, chronic beryllium disease, interferon-alpha therapy, BCG immunotherapy and allopurinol have been more frequently involved. FUTURE PROSPECTS AND PROJECTS: Literature review uncovers a variety of potential toxic exposures and highlights the necessity of a clear sighted research to identify them.

[Sarcoidosis and sarcoid reactions in cancer]. / Place de la pathologie granulomateuse au cours des cancers.

PURPOSE: Relationships between granulomatosis and cancers have been suspected for a long time. Nevertheless, few evidence has been reported until recently. Here, we present a literature review about the association of granulomatosis and neoplasia. CURRENT KNOWLEDGE AND KEY POINTS: Aside from granulomatosis due to infectious disease, granulomas can be observed in cancer patients, mainly in two situations. Patients may rarely present with typical sarcoidosis occurring before, during or after the diagnosis of cancer. Recent studies have documented such a relationship particularly with lymphomas, testicular and lung cancers, melanomas and hepatocarcinomas. Secondly granulomas may be found as a sarcoid reaction in the vicinity of the tumour itself or more frequently in regional lymph nodes. Sarcoid reaction, reported in Hodgkin's disease and gastric adenocarcinomas, may be associated with a better prognosis. Granulomatous reaction could play an important role in the host's defences against metastatic extension. Immunotherapy such as interferon has been reported to induce systemic sarcoidosis probably by reproducing some physiopathological mechanisms involved in sarcoidosis. FUTURE PROSPECTS AND PROJECTS: Clinicians need novel non invasive diagnostic methods to differentiate neoplasia from benign sarcoid reactions. The 18-fluorodeoxyglucose (18-FDG) PET-scan has failed in this indication but the adjunction of a [3-(18)F]-alpha-methyltyrosine ((18)F-FMT) PET-scan could be useful. Biopsies is still necessary in most of cases.

[Hypocomplementemic urticarial vasculitis]. / Vascularite urticarienne hypocomplémentémique.

Hypocomplementemic urticarial vasculitis is a rare disorder characterized by the presence of C1q precipitins associated with a syndrome of urticarial vasculitis, arthralgias, ocular inflammation and obstructive-lung disease. We report the case of a 48-year-old woman with hypocomplementemic urticarial vasculitis. Because of dependance to corticosteroids, cyclophosphamide-pulse therapy was started and resulted in significant clinical improvement. Mycophenolate mofetil was effective as maintenance therapy and resulted in complete resolution of rash, arthralgias, arthritis and uveitis, but had no effect on the obstructive-lung disease.

[Systemic sclerosis in men]. / Sclérodermie systémique chez l'homme.

PURPOSE: To study the initial clinical features and describe the outcome of systemic sclerosis in a cohort of French men. METHODS: Patients with systemic sclerosis based on Leroy's criteria were included. In this retrospective study we compared a cohort of men to a cohort of women, diagnosed between 1997 and 2005 in departments of internal medicine and rheumatology. RESULTS: One hundred and twenty-one patients were included amongst which thirty-six men. The mean follow-up duration was 6.5 years. The time to diagnosis was significantly shorter in men than in women. Diffuse cutaneous systemic sclerosis, cutaneous ulcers and interstitial syndrome on chest radiograph were more frequent at diagnosis in men than in women. An environmental factor (silica) was observed in only nine men. During the follow-up, incidence of restrictive lung disease was significantly higher in men than in women (37% versus 14% p=0.01) with higher rates of oxygen dependency (22% versus 5% p<0.01). Cumulated survival rates in men were 92% at 5 years, 72% at ten years and 43% at 15 years, respectively. The mean survival was 13 years in men (IC 95%: 10-16) versus 23 years in women (IC 95%: 10-36) with no statistical difference (p=0.27). CONCLUSION: If interstitial and restrictive lung disease, oxygen dependency and diffuse systemic sclerosis were more frequent in men than in women, this data did not provide any evidence of survival difference between men and women with systemic sclerosis.

[Tropheryma whipplei and Whipple disease: false positive PCR detections of Tropheryma whipplei in diagnostic samples are rare]. / Tropheryma whipplei et maladie de Whipple: résultats de la recherche par polymerase chain reaction (PCR) dans les échantillons à visée diagnostique.

BACKGROUND: PCR can be used to detect T. whipplei (Tw) in samples from variable tissue types and body fluids. We report clinical, evolutive characteristics and final diagnosis in patients with positive Tw PCR assay. METHODS: Retrospective study of Tw PCR realized since 10years in a microbiology laboratory. RESULTS: Twenty-five Tw PCR assays were positive among 200 realized. Diagnosis was not confirmed in six cases. One patient was missing for follow up. Eighteen patients presented with Whipple's disease. Among these 18 patients, 14 had a classic Whipple's disease, three patients presented an endocarditis and one patient isolated neurological manifestations. Ten patients presented fever, seven a weight loss and 12 joint involvement. Four patients presented cutaneous manifestations, only six had gastrointestinal symptoms. Neurological involvement was reported in five cases, pulmonary symptoms in four cases, cardiac involvement in six cases and ocular signs in two cases. Anemia was reported in four patients and elevated levels of acute-phase reactants in 14 cases. Positive predictive value of Tw PCR for Whipple's disease diagnosis was 75%. Thirteen patients had a good evolution with antibiotics. Three patients presented recurrence and two cases with cardiovascular involvement died. CONCLUSION: Whipple's disease is rare but often mentioned in internist experience. The diagnosis should be every time confirmed. Tw PCR assay is an important diagnostic tool but is not sufficient to establish the diagnosis and must be interpreted with histopathology and immunohistochemical testing results.

[Non-tuberculous systemic granulomatosis mimicking sarcoidosis but related to a specific etiology. Study of 67 cases]. / Granulomatoses systémiques pseudosarcoïdosiques d'étiologie déterminée et non tuberculeuse. Etude de 67 observations.

PURPOSE: Systemic granulomatosis (SG) are frequently encountered in internal medicine. Despite a large list of aetiologies, the investigations remain often negative leading to the diagnosis of atypical sarcoidosis. The spectrum of the causes, as well as evolution of these SG is not clearly delineated in the literature. METHOD: We analyzed the case reports of all but tuberculous GS submitted at the National Meetings of the National French Society of Internal Medicine from 1990 to 2006. RESULTS: Sixty-seven cases were included in the study. The average age at the beginning of the symptoms was 47.8 years and 28.4% of the patients were female. The median diagnostic delay was one year. General symptoms were present in 73.1% of the cases. The involved organs were the liver (46.3%), lungs (25.4%), lymph nodes (22.4%), digestive tract (16.4%), skin (16.4%), spleen (14.9%). The granuloma were detected mainly in the liver (38.8%), lymph nodes (17.9%), bone marrow (16.4%) and lungs (11.9%). Elevated erythrocyte sedimentation rate or increased C reactive protein serum levels were noted in 65.6% of the patients. Before diagnosis, 19.4% of the patients received a corticotherapy. The most common diagnoses were infections (65.6%) followed by drugs (19.5%), "toxic substances" or various foreign bodies (5.9%), neoplasias (5.9%) and immune deficiencies (3%). The evolution was favourable in 80% of the cases but 8.3% of the patients died. The disease course of the patients having received a corticotherapy prior to the diagnosis was more unfavourable with a death rate of 45%. CONCLUSION: In atypical sarcoidosis (fever, advanced age, increased acute phase reactants...) a specific aetiology and especially an infectious disease should be ruled out before considering the diagnosis of sarcoidosis. Corticotherapy is a factor of poor prognosis.

[Chronic inflammatory demyelinating polyneuropathy and sarcoidosis: fortuitous association?]. / Polyradiculonévrite chronique et sarcoïdose: association fortuite.

We report the case of a 36-year-old-man who first presented two relapses of chronic inflammatory demyelinating polyneuropathy (CIDP) before a diagnosis of sarcoidosis was made. He subsequently presented two combined relapses of CIDP and sarcoidosis. Each time, the outcome was favorable after treatment with intravenous immunglobulins and steroids. The possible relationship between CIDP and sarcoidosis is discussed.

[Pentoxifylline: a cheap substitute for anti-TNFalpha agents?]. / Pentoxifylline: l'anti-TNF du pauvre?

PURPOSE: Pentoxifylline (PTX) is a phosphodiesterase inhibitor drug used to improve peripheral vascular disease. In vitro studies demonstrated that PTX has anti-TNFalpha properties. We did a selective review of clinical trials which used PTX in patients with inflammatory rheumatic and non-rheumatic diseases. CURRENT KNOWLEDGE AND KEY POINTS: Most of the identified clinical trials were uncontrolled and involved a low number of patients. Use of PTX in systemic lupus erythematous, Behçet's disease and sarcoidosis yielded significant preliminary results. Moreover, PTX markedly reduced proteinuria in several glomerulonephritis (lupus nephritis, membranous nephropathy, diabetic nephropathy). FUTURE PROSPECTS AND PROJECTS: Further randomized and controlled clinical trials are required to examine whether PTX can improve outcome in patients with inflammatory diseases. Meanwhile, PTX should not be used for the treatment of these diseases.

[Venous thromboembolism and cancer]. / Maladie veineuse thromboembolique et cancer.

OBJECTIVES: The risk of venous thrombosis during cancer is largely increased especially in case of chemotherapy, surgery, advanced stage disease, coagulation abnormalities. Survival of patients with cancer experiencing venous thrombosis seems to be worse. Although thrombosis may be a presenting feature of occult malignancy, there are insufficient data to support a more extensive screening than comprehensive medical history, physical examination, routine laboratory tests and chest radiography. CURRENT KNOWLEDGE AND KEY POINTS: Pathophysiology of venous thrombosis during cancer is unspecific: venous stasis, vessel wall damage, hypercoagulability). Other factors like platelet abnormalities or the direct responsibility of chemotherapy or hormonotherapy have recently been though to play a causative role. Treatment of cancer-associated thrombosis usually requires at least 6 months of low-molecular-weight heparin therapy rather than oral anticoagulant. Inferior vena cava filters are not indicated. Primary prophylaxis of thrombosis during cancer could safely been achieved with low-molecular-weight heparin. Central venous catheters can be associated with thrombotic complications. Many risks factors have been identified: catheter's type, modalities of catheter's implantation, type of perfusion, bulky mediastinal mass... Prophylactic anticoagulation is not routinely recommended. FUTURE PROSPECTS AND PROJECTS: Knew oral anticoagulants could facilitate the treatment of venous thrombosis occurring during cancer in the next years.

[Common variable immunodeficiency with autoimmune manifestations: study of nine cases; interest of a peripheral B-cell compartment analysis in seven patients]. / Déficit immunitaire commun variable avec manifestations auto-immunes: étude de neuf observations; intérêt d'un immunophénotypage spécifique des lymphocytes B circulants chez sept patients.

PURPOSE: Autoimmune manifestations (AIM) are associated to common variable immunodeficiency (CVI) in about 20 to 25% of the cases. This study presents the clinical, biological characteristics and the evolution of nine patients developing CVI and AIM. A peripheral B-cell compartment analysis has been performed in seven cases. METHOD: This multicenter retrospective study analyses nine patients, six men and three women, within a population of 32 CVI. RESULTS: The mean age was 27 years at the time of diagnosis of AIM and 30 years at the time of diagnosis of CVI. The diagnosis of AIM preceded the diagnosis of CVI in five cases. Thirteen AIM of different types were observed: autoimmune hemolytic anemia (AHA, 3), immune thrombocytopenic purpura (ITP, 2), Evan's syndrome (2), primary biliary cirrhosis (1), rheumatoid arthritis (1), alopecia totalis (1), myasthenia gravis (1). The peripheral B-cell compartment was investigated in seven patients: five patients with autoimmune cytopenia presented with a diminution of memory B cells (CD27+IgD-) and immature B cells (CD21-) levels; the patient with primary biliary cirrhosis and myasthenia gravis had only a diminution of memory B cells level; the last patient with ITP presented with a normal level of memory B cells. Five among the seven patients with autoimmune cytopenia required a specific treatment using corticosteroids, high dosages of intravenous immunoglobulin, then splenectomy after failure of the medical management, with severe infectious complications in one case. CONCLUSION: The association of AIM and CVI is not fortuitous. The most common AIM is autoimmune cytopenia. The peripheral B-cell compartment analyses show that a majority of patients have a defect in memory B-cells. Treatment regimens are not standardized and splenectomy increases the risk of infectious complications.

[Whipple's disease endocarditis: report of 5 cases and review of the literature]. / Endocardites de la maladie de Whipple: cinq observations et revue de la littérature.

PURPOSE: Endocarditic lesions (infectious endocarditis) associated with Whipple's disease are exceptional. We report five cases from the cardiovascular and pneumologic hospital Louis Pradel in Lyon. METHOD: We have collected all cases of Tropheryma whipplei endocarditis diagnosed between 1995 and 2004. RESULTS: Five men with a mean age of 53 years at time of diagnosis. The symptoms were essentially cardiovascular: murmur, embolism in 3 cases, and heart failure secondary to valvular insufficiency in 2 cases. The valvular involvement, double in 3 cases, was more often aortic. Vegetations were present in all patients and valvular destruction sometimes very important. A low grade fever was present in 4 cases, associated with weight loss in 2 cases. The only extra-cardiac symptoms were arthralgias or arthritis in all cases, considered in 3 patients as seronegative rheumatoid arthritis, B27+ spondylarthritis, and psoriasic arthritis. Their was no other clinical manifestations of Whipple's disease, particularly digestive, ocular, neurologic or adenopathy, and duodenal biopsies secondarily performed in 4 cases were non contributive. This differs from literature as an extra-cardiac location was identified in 11 out of 17 cases. The diagnosis was obtained by histology and PCR on the cardiac valves, as all the patients underwent surgery. The evolution was favourable with a prolonged antibiotic therapy. CONCLUSIONS: These report confirms the existence of endocarditic forms of the Whipple's disease, in which the single extra-cardiac manifestation is rheumatologic, and reminds us the usefulness of histology and PCR on the cardiac valves at the time of valvular surgery.

Whipple disease. Clinical review of 52 cases. The SNFMI Research Group on Whipple Disease. Société Nationale Française de Médecine Interne.

Whipple disease is a rare, multiorgan disease with prominent intestinal manifestations. We report a retrospective clinical study of 52 patients recruited in various parts of France from 1967 to 1994. Seventy-three percent of the patients were male. Clinical manifestations preceding the diagnosis were articular for 35 patients (67%), digestive for 8 patients (15%), general for 7 patients (14%), and neurologic for 2 patients (4%). At a later stage of the disease, 44 patients (85%) presented diarrhea, weight loss, and malabsorption, while 8 patients (15%) did not show any gastrointestinal symptom throughout the development of the disease. Forty-three patients (83%) presented arthralgia or arthritis, and 11 (21%) had prominent neurologic symptoms. In addition, cardiovascular symptoms were present in 9 patients (17%); mucocutaneous symptoms, in 9 patients (17%); pleuropulmonary symptoms, in 7 patients (13%); and ophthalmologic symptoms, in 5 patients (10%). All patients but 1 were given a positive diagnosis on histopathologic criteria: jejunal biopsy for 46 patients (90%), lymph node biopsy for 3 patients (6%), brain biopsy for 1 patient (2%), postmortem jejunal and cerebral biopsy for 1 patient (2%). With treatment, the disease evolved favorably in 47 patients (90%), while 5 patients (10%) had unfavorable outcomes (2 deaths from neurologic involvement, 1 patient with chronic dementia, and 2 patients with digestive symptoms insensitive to antimicrobial therapy). Of the 41 patients initially treated successfully and whose treatment has been completed, clinical evolution after discontinuation of treatment was favorable in 34 cases (83%). Clinical relapses occurred in 7 patients. No relapse was observed after treatment by trimethoprim-sulfamethoxazole, alone or following a combination of penicillin and streptomycin, or after the combination of penicillin and streptomycin, whatever the oral follow-up treatment prescribed. The evolution of patients showing a relapse was favorable in all cases after reintroduction of antibiotic therapy. These results are discussed in the light of previously published series and case reports of Whipple disease. The diagnosis of the disease remains difficult at an early phase or when digestive symptoms are absent. It is noteworthy that proximal enteroscopy is sometimes misleading, considered normal on macroscopic examination and nonspecific on pathologic grounds. A normal erythrocyte sedimentation rate represents another pitfall. Histopathology is the key for positive and differential diagnosis, and may require multiple and repeated biopsies. Findings from molecular biology confirm the central role of an uncultured Gram-positive bacillus which was named in 1992 Tropheryma whippelii. A recent report suggests that polymerase chain reaction (PCR) analysis of peripheral blood might allow the diagnosis of Whipple disease in some cases. However, immunologic or cellular parameters such as macrophagic function may play an important, although not clearly elucidated, role in the pathogeny of the disease. Trimethoprim-sulfamethoxazole should be considered the antimicrobial agent of choice in the treatment of Whipple disease, minimizing the risk of cerebral involvement and relapses.

Growth factors and proinflammatory cytokines in the renal involvement of POEMS syndrome.

The POEMS syndrome is a multisystemic syndrome associated with plasma cell dyscrasia, characterized by the combination of polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes. Renal involvement in POEMS syndrome is rare (26 reported cases). It has been described as membranoproliferative glomerulonephritis-like lesions (MPGN-like), mesangiolytic glomerulonephritis, or thrombotic microangiopathy. Proinflammatory cytokines (TNF-alpha, IL-1, IL-6) have been implicated in the physiopathogenesis of POEMS syndrome, particularly when there is renal involvement. Growth factors (FGF-beta, TGF-beta, PDGF) have been implicated in renal lesions of the same histological type but of different origins. An increase in serum vascular endothelial growth factor (VEGF) has been reported in POEMS syndrome (20 of 22 cases). Circulating levels of these factors were determined in 4 patients with POEMS and renal involvement (3 MPGN-like, 1 MPGN-like, and mesangiolysis) and compared with those obtained in 4 patients with POEMS without clinical renal involvement and in 4 patients with primitive membranoproliferative glomerulonephritis (MPGN). TNF-alpha, IL-1beta, and IL-6 were determined with an immunoradiometric assay, and VEGF, PDGF, FGF-beta, and TGF-beta with an enzyme-linked immunosorbent assay. Among the patients with POEMS syndrome, there was no difference in proinflammatory cytokines and growth factors between those with or without renal involvement. VEGF is the only growth factor that differentiates MPGN in POEMS syndrome from primitive MPGN.

'Localized amyloidosis': 123I-labelled SAP component scintigraphy and labial salivary gland biopsy.

In apparently localized amyloidosis, there is no appropriate test to determine whether systemic deposits exist. We studied the value of serum amyloid P component (SAP) scintigraphy and labial salivary gland (LSG) biopsy on patients with apparently localized amyloidosis in 12 patients who had neither clinical nor biological evidence of systemic amyloidosis. All patients had an LSG biopsy and echocardiography. Iodine-123-labelled serum amyloid P component (123I-SAP) scintigraphy was performed in all patients. Whole-body scintigraphy was done, and tissue retention was evaluated at 24 h and 48 h. Of these 12 patients, three had amyloidosis in their LSG and had abnormal 123I-SAP scintigraphy; these three had a secondary clinical history of systemic amyloidosis. Three other patients had abnormal 123I-SAP scintigraphy without detectable systemic amyloid deposits, but one had a previous history of bilateral carpal tunnel syndrome treated with infiltration. 123I-SAP scintigraphy in association with LSG biopsy may be helpful in determining the localized or systemic character of amyloid disease.

Hepatic granulomatosis in a patient with Graves' disease.

We report a case of granulomatous hepatitis in a patient with hyperthyroidism resulting from Graves' disease. A 30-year-old man presented with massive weight loss, jaundice, tachyarrhythmia and goitre. Liver function tests showed mild cytolysis and cholestasis and massive hyperbilirubinaemia. The echogram of liver and bile ducts was normal and no infection was found. A liver biopsy revealed a mixed cytolytic and cholestatic hepatitis with intralobular epithelioid granulomas. No specific cause was identified, and sarcoidosis and primary biliary cirrhosis were ruled out. The outcome was favourable with antithyroid therapy and short-term glucocorticoid therapy, and the patient was totally free of symptoms after 2 years. To our knowledge, this is the first case of granulomatous hepatitis to be reported in association with Graves' disease. The clinical evolution of the liver disease paralleled the evolution of hyperthyroidism.

Mucosal ulcerations revealing primitive hypereosinophilic syndrome.

We report the case of a 27 year-old man developing recurrent oral aphtosis associated with fever and 8 kg of weight loss. Moderate splenomegaly was observed on physical examination and neurological and cardiac examination were normal. Laboratory findings included marked eosinophilia at 3280 giga/l. Bone marrow (BM) examination revealed a myeloproliferative syndrome with mature eosinophils. Splenectomy was performed because of a suspected nodule on the BM, the histopathology revealed a myeloid metaplasia. The diagnosis of myeloproliferative form of hypereosinophilic syndrome (HES) was made. He was treated with interferon-alfa and hydroxyurea. After two years of treatment he had no ulcer recurrence and eosinophil count was at 180 giga/l. Mucosal manifestations as a prodromal symptom of HES are rare. The histology of the lesions shows numerous eosinophils; immunohistochemical analysis confirms the presence of eosinophil peroxydase, major basic protein and eosinophil derived neurotoxin. A few cases have been described. Death occurs 11 months to 5 years after the diagnosis of oral ulcerations. The treatment consists of interferon-alfa and hydroxyurea.

Residual inflammatory process after aortoiliac reconstructive surgery.

BACKGROUND: The aim of the present study was to investigate the inflammatory reaction and its evolution in patients who underwent a prosthetic vascular procedure. Moreover the participation of this chronic process, during the follow-up, as a promoting or a consequence of vascular injury must be discussed. METHODS: Thirty-four patients were enrolled in the study. All patients had an aortic disease and underwent a prosthetic vascular procedure. Preoperative exclusion criteria were an emergency situation, diabetes, infection, chronic inflammatory disease, cancer and hemopathy. Postoperative exclusion criteria were the same together with abdominal complications and additional surgery during the follow-up. The inflammatory process was investigated with the measurement of blood acute phase proteins, haptoglobin, alpha1-glycoprotein acid, C-reactive protein and interleukin-6, before, immediately after surgery and several months after surgery. RESULTS: An increase in acute phase proteins was not observed to the same extent for all the studied patients. Before the surgical procedure, chronic inflammatory process was revealed by an increase in haptoglobin (52.9 p 100) and alpha1 glycoprotein acid (52.9 p 100) whereas increase in C-reactive protein (26.4 p 100) and interleukin-6 (92 p 100) are related to an acute process. Later after surgery, the chronic inflammatory process remained but differed from the observed process before surgery only by haptoglobin (61.7 p 100) and interleukin-6 (47 p 100). CONCLUSIONS: The presented results, observed during the follow-up of vascular surgery focused on persistent inflammatory process and the surgical procedure did not modify the time course of this process. The evolutionary disease could be considered as chronic and independent of the local effect.

[A great imitator for the allergologist: intolerance to gluten]. / Une grande simulatrice pour l'allergologue: l'intolérance au gluten.

Intolerance of gluten, resposible for Coeliac disease, is essentially shown by an auto-immune enteropathy, even if the cutaneous manifestation (herpetiform dermatitis) and perhaps certain neurological signs (cerebral syndrome, peripheral neuropathy) may be independent as well as associated with the intestinal illness. This affection is of immunological nature, occuring in a genetic field that predisposes to the illness (familial form: concordance of 70% in homozygote twins; 90% of patients show an HLA molecule of type DQ2, DQ8 in almost all the other cases. The exogenous factor is the gluten content contained in wheat, rye and barley, more precisely by the intermediary "the prolamines" which are the "reactive" element that induces a the same time an inflammatory reaction of type TH11 locally (expressed by the histological aspect of a duodenal biopsy evolving as villous atrophy) and a humoral response with production of anti-gliadine and anti-transglutaminase antibodies (the role of the latter enzyme is intervention in the local transformation of antigens to make them antigenic). It is an illness of adults as well as children and this point must now be emphasized. Recent epidemiological studies insist on a high prevalence (1/300 in Europe). Clinical expression, at the start very polymorphic and so misleading, before the appearance of the more classical signs of malabsorption and development, always feared, towards a lymphoma. These signs are haematological (anemia of various types, hyper platelets by hyposplenism, haemorrhagic signs) cutaneous (herpetiform dermatitis, cutaneous vasculitis) mucosal (aphtose), hepatic (cytolysis), neurophysical (fatigue, troubles of behaviour, cerebral syndrome, neuropathy) and osteo-articulitis (osteopenia, arthralgias, diffuse pains). The association of certain auto-immune illnesses must be emphasized (diabetes, Hashimoto thyroiditis, Gougerot disease, primitive biliary cirrhosis). To think early of the possibility of intolerance to gluten, is to give the means of a very easy diagnosis (measurement of anti-gliadin, anti-endomysium and anti-transglutaminase, and secondarily duodenal biopsy if necessary), and it is early elimination of gluten food which will make the various clinical manifestations disappear and so prevent the risk of evolution to a tumoral pathology.

[Subclavian and axillary arteritis in Horton's disease and rhizomelic pseudopolyarthritis. 10 cases]. / Artérites sous-clavières et axillaires au cours de la maladie de Horton et de la pseudopolyarthrite rhizomélique. 10 observations.

Ten patients aged from 60 to 73 years presenting with Horton's disease or polymyalgia rheumatica had arteritis of the upper limbs. Asymptomatic abolition of pulse in the upper limbs (1 case) or claudication at rest or exercise (9 cases) and/or Raynaud's phenomenon (5 cases) preceded (4 cases) or accompanied (1 case) the discovery of giant cell arteritis, or complicated the reduction or discontinuation of corticosteroid therapy. Diagnosis rested on the regular association of an inflammatory syndrome with multiple arterial tapered stenoses and/or arterial thrombosis in the post-vertebral subclavian, axillary or brachial arteries and, chiefly, on the demonstration (in 7 cases) of a giant cell granuloma at biopsy of the temporal artery. Corticosteroid therapy (1 mg/kg/24 h in 8 cases and 0.5 mg/kg/24 h in 2 cases) initially combined with anticoagulants in 4 cases resulted in rapid regression of ischaemic and systemic signs in all patients, thus avoiding surgical revascularization of the upper limbs.