[Orofacial manifestations of systemic sclerosis: A study of 30 consecutive patients]. / Les manifestations buccofaciales de la sclérodermie systémique : étude de 30 patients consécutifs.

INTRODUCTION: The face is frequently involved in systemic sclerosis. The main stomatologic manifestations include limited mouth opening, xerostomia, skin atrophy, trigeminal neuralgia. The objective of this study was to describe oral and facial manifestations observed in scleroderma patients from our cohort. METHODS: Between March and October 2006, a stomatologic consultation was included in the follow-up of scleroderma patients seen during consultation or daily hospital in internal medicine or dermatology units. Demographic, clinical and biological data were collected. Stomatologic examination comprised measure of the mouth opening, sugar's and Schirmer's tests, orthopantomogram analysis, and evaluation of the repercussion of symptoms on quality of life using a visual analogical scale (VAS between 0 and 10). RESULTS: This study included 30 patients (women 87 %, mean age 58.6 + or - 13.6 years). Mean duration of systemic sclerosis (n=20 limited cutaneous form, n=10 diffuse form) was eight years. Stomatologic manifestations were: skin atrophy (n=28), peribuccal rhagades (n=25), telangiectasia (n=21), decreased mouth opening (n=20), xerostomia (n=20), xerophtalmia (n=16), periodontal ligament space widening (n=10), bone resorptions (n=2), trigeminal neuralgia (n=1). Xerostomia was considered more discomforting (mean VAS=3.8) than decreased mouth opening (mean VAS=2.6). Xerostomia was the second more discomforting sign of scleroderma and was significantly associated to the limited cutaneous form (p=0.045) and to anticentromeres antibodies expression (p=0.002). Decreased mouth opening was correlated to oesophageal involvement (p=0.025). CONCLUSION: Oral and facial manifestations are frequently observed in scleroderma patients. These manifestations lead to major functional discomfort, mainly due to decreased mouth opening that seems to be frequently associated to oesophageal involvement. Xerostomia is also frequent and is commonly observed in anticentromere antibodies positive cutaneous limited forms of systemic sclerosis. Evolution of radiographic abnormalities like periodontal ligament space widening (33 % of cases), or osteolytic lesions (7 %) is poorly known.

[Orofacial manifestations of systemic sclerosis: a study of 30 consecutive patients]. / Les manifestations buccofaciales de la sclérodermie systémique: étude de 30 patients consécutifs.

INTRODUCTION: The face is frequently involved in systemic sclerosis. The main stomatologic manifestations include limited mouth opening, xerostomia, skin atrophy, trigeminal neuralgia. The objective of this study was to describe oral and facial manifestations observed in scleroderma patients from our cohort. METHODS: Between March and October 2006, a stomatologic consultation was included in the follow-up of scleroderma patients seen during consultation or daily hospital in internal medicine or dermatology units. Demographic, clinical and biological data were collected. Stomatologic examination comprised measure of the mouth opening, sugar's and Schirmer's tests, orthopantomogram analysis, and evaluation of the repercussion of symptoms on quality of life using a visual analogical scale (VAS between 0 and 10). RESULTS: This study included 30 patients (women 87%, mean age 58.6+/-13.6 years). Mean duration of systemic sclerosis (n=20 limited cutaneous form, n=10 diffuse form) was eight years. Stomatologic manifestations were: skin atrophy (n=28), peribuccal rhagades (n=25), telangiectasia (n=21), decreased mouth opening (n=20), xerostomia (n=20), xerophtalmia (n=16), periodontal ligament space widening (n=10), bone resorptions (n=2), trigeminal neuralgia (n=1). Xerostomia was considered more discomforting (mean VAS=3.8) than decreased mouth opening (mean VAS=2.6). Xerostomia was the second more discomforting sign of scleroderma and was significantly associated to the limited cutaneous form (p=0.045) and to anticentromeres antibodies expression (p=0.002). Decreased mouth opening was correlated to oesophageal involvement (p=0.025). CONCLUSION: Oral and facial manifestations are frequently observed in scleroderma patients. These manifestations lead to major functional discomfort, mainly due to decreased mouth opening that seems to be frequently associated to oesophageal involvement. Xerostomia is also frequent and is commonly observed in anticentromere antibodies positive cutaneous limited forms of systemic sclerosis. Evolution of radiographic abnormalities like periodontal ligament space widening (33% of cases), or osteolytic lesions (7%) is poorly known.

Churg-Strauss syndrome revealed by granulomatous acute pericarditis: two case reports and a review of the literature.

BACKGROUND: Churg-Strauss syndrome (CSS) is a necrotizing systemic vasculitis with extravascular granulomas and eosinophilic infiltrates of small vessels. CSS is usually revealed by nonspecific signs of necrotizing vasculitis in a context of late-onset asthma and blood eosinophilia. It is considered a systemic vasculitis with the highest prevalence of cardiac involvement and can lead to rapid-onset heart failure due to specific cardiomyopathy. Pericardial effusion may also occur during CSS and is usually well tolerated. OBJECTIVE: The objective of these case reports was to indicate that CSS may present as tamponade, with or without other visceral involvement. METHODS: Among CSS patients treated during the past 10 years at 2 French university hospitals, we have identified and described 2 cases revealed by tamponade with pericardial biopsy-proven granulomatous vasculitis. We have also reviewed the international medical literature in PubMed on cardiac involvement in CSS. RESULTS: The first case report describes a 66-year-old man who had an isolated cardiac tamponade with both inflammatory syndrome and eosinophilia. Long-term remission was obtained with corticosteroids. The second case report describes a 46-year-old woman whose CSS presented with tamponade and associated central nervous system and myocardial involvement. Remission was obtained with corticosteroids and cyclophosphamide. In both cases, CSS was assessed by histological analysis of a pericardial sample. CONCLUSIONS: CSS may present as isolated cardiac tamponade. Whereas pericarditis with myocardial injury warrants immunosuppressive therapy, isolated pericarditis without other visceral involvement of poor prognosis only requires corticosteroid therapy.

[Screening of abdominal aortic involvement using Doppler sonography in active giant cell (temporal) arteritis at the time of diagnosis. A prospective study of 30 patients]. / Dépistage de l'atteinte aortique abdominale par échographie-doppler au diagnostic de maladie de Horton. Etude prospective de 30 patients.

BACKGROUND: Inflammatory involvement of extracranial large-sized arteries occurs in 10-20% of patients with giant cell (temporal) arteritis. Aortic involvement may reveal giant cell arteritis or occur as a late-onset complication, and represents one of the most serious manifestation of the disease with the risk of aortic dissection and/or aneurysm rupture. The thoracic aorta is more frequently involved but abdominal aortitis may also occur in giant cell arteritis. To date, few data are available about abdominal aorta changes at the initial stage of giant cell arteritis. PATIENTS AND METHODS: This prospective monocentric study was conducted between May 1998 and May 2002, and included 30 consecutive patients with biopsy-proven giant cell arteritis. Standard clinical and biological data were collected. Each patient underwent an abdominal aortic Doppler-sonography that looked for aneurysm, ectasia, thickening of the vascular wall, and hypoechoic halo around the aorta. RESULTS: Among the 30 patients of this study (25 women, 5 men, mean age 68.5 years), 4 (13%) had an abdominal aortic aneurysm, with a low diameter (23 to 27 mm), measuring 2 to 5.5 cm in length. A vascular wall thickening superior or equal to 3 mm was noted in 17 patients (68%). A 4 to 8 mm periaortic hypoechoic halo was found in 10 patients (33%). This halo was present in 3 out of the 4 patients with aneurysm. CONCLUSION: Aortic involvement is a potentially serious complication of giant cell arteritis. The question of a systematic screening of this complication remains open to discussion. Our study shows that Doppler sonography may detect morphological abnormalities on the abdominal aorta at the initial stage of giant cell arteritis. These abnormalities comprise mild aneurysms, thickening of the vascular wall and periaortic halo, which could correspond to inflammatory locations of the disease. Complementary studies are needed to assess their specificity and their seriousness.

[Empirical treatment of granulomatous hepatitis of unknown origin: practice investigation in the French National Society of Internal Medicine]. / Traitement d'épreuve d'une hépatite granulomateuse symptomatique de cause indéterminée: enquête de pratique au sein de la Société nationale française de médecine interne.

PURPOSES: Ten to fifteen percent of granulomatous hepatitis are idiopathic. If symptoms like prolonged fever are present, empirical treatment is discussed. The goal of this study is to describe the empirical treatment proposed in this situation by French specialists of internal medicine. METHODS: We conducted a practice investigation among the French national society of internal medicine (SNFMI), using an anonymous questionnaire that related a case of idiopathic granulomatous hepatitis. This questionnaire was proposed to all French internists present at the SNFMI congress in June and December 2004. French specialists of internal medicine had to answer if they would prescribe an empirical treatment and if so, to specify this treatment. RESULTS: Thirty-six French specialists of internal medicine answered to the questionnaire. In the proposed situation, 89% of them initiate an empirical treatment. In 18/36 cases (50%), a first-line anti-tuberculosis empirical treatment is proposed (quadritherapy in 11 cases). In 7 cases (19%), an empirical treatment with prednisone, 0.4 mg/kg/d (N=1) and 1 mg/kg/d (N=6), would be prescribed. Seven internists (19%) would prescribe an empirical treatment with cyclins at the dose of 100 to 400 mg/d. Median duration of the empirical treatment would be 28 days (range: 8-252d). The evaluation parameters mentionned are: fever (69%), weight (59%), seric level of C-reactive protein (59%), and liver biology (53%). In case of failure of first-line empirical treatments, 69% of all questionned internists prescribe a second-line treatments: prednisone at the dose of 0.4 to 2 mg/kg/d (72%), anti-tuberculosis treatments (16%), cyclins 200 mg/d (12%), with a median duration of 28 days. Seven internists (19%) propose to combine two empirical treatments. DISCUSSION: Faced with a problem of idiopathic granulomatous hepatitis, French internists questionned propose four therapeutics options: no treatment, anti-tuberculosis treatment, cyclins or steroids treatment. First-line anti-tuberculosis treatment is a coherent proposition regarding to the high prevalence of tuberculosis. There are only few data available concerning empirical treatment with steroids or cyclins. Specific proposition of such empirical treatments should be defined. CONCLUSIONS: The management of idiopathic granulomatous hepatitis is difficult. Our study shows that therapeutics practices of French internists are heterogenous. The main proposition consists in a first-line anti-tuberculosis empirical treatment, that has to be evaluated after four weeks, and switched with steroids (prednisone, 1 mg/Kg/d) in case of failure. This study is not an expert proposition but contributes to suggest clinical practice guidelines for a rare, complex, heterogenous, and typically internist situation.

[Qualitative and quantitative evaluation of an internal medicine assistance line dedicated to the diagnosis and treatment of diseases for general practice]. / Évaluation qualitative et quantitative d'un service d'assistance téléphonique de médecine interne dédié à l'aide diagnostique et thérapeutique de pathologies relevant du champ de la médecine générale.

PURPOSE: Clinical reasoning and treatment challenges within the scope of general practice led to the development of an internal medicine assistance line provided by Nantes University Hospital. The primary outcome of this study was to describe callers' profile, their requests and answers provided. METHODS: A prospective, cross-sectional, observational, descriptive study was undertaken. For each call were identified the calling physician, her/his specialty and work setting, the call's object and adequacy, the answer provided, the time needed to connect with the assistance line, the time devoted by the internal medicine physician to provide an answer to the request, and whether the assistance line prevented a visit to the emergency room. Each calling physician was then called back to obtain demographic and professional characteristics, and data relating to the call and to the assistance line. RESULTS: Sixty-three days were analyzed and 276 calls identified. The 237 identified calling physicians were mainly females (54%, n=93), with a mean age of 46 years, graduated from Nantes University (65%, n=86), practicing ambulatory general medicine (69%, n=164) in Loire-Atlantique department area (82%, n=176) for a mean duration of 15 years. Calls were mostly associated with diagnostic challenges (61%, n=166) concerning clinical issues (57%, n=155). A sole telephone advice was the main type of answer provided (56%, n=147) and a visit to the emergency room was prevented for 17% of calls. CONCLUSION: The assistance line activity is adequate with its missions and seems to facilitate patients' healthcare delivery advocating for the development of similar structures in other units. Improvements relating to the information, availability and physicians' training should be considered.

Chronic myelomonocytic leukemia and primary Sjögren's syndrome.

Although associations of Sjögren's syndrome (SS) with malignant affections are well known, an association with chronic myelomonocytic leukemia (CMML) is infrequent. Our report concerns an 80-year-old woman with chronic polyarthrosis, hospitalized for an anemic syndrome, who also complained of buccal dryness. Clinical examination showed simply parotid swelling and discrete splenomegaly. The diagnosis of SS appeared to be primary. The hemogram, myelogram and bone biopsy indicated CMML. During SS, the possibility of occurrence of a lymphoproliferative syndrome is well documented, and other malignant affections are much less likely to be found in the absence of immunosuppressive treatment. The particular case of SS and associated CMML detected at the same time suggests either the favorable role of monocytic proliferation on immunity or a stem clonal anomaly affecting monocytes and B lymphocytes.

[Epidemiology of Horton's disease. Is there an environmental factor, infective in particular?]. / Epidémiologie de la maladie de Horton. Existe-t-il un facteur d'environnement, en particulier infectieux?

Epidemiological studies of temporal arteritis have essentially only been reported in the English literature. The authors of this study were concerned with this aspect of temporal arteritis in the Loire-Atlantique region of France over a period of 10 years (1970-1979). The high prevalence in white races has been confirmed. The annual incidence in France is comparable to that seen in Northern Europe and the USA. The incidence of the disease is especially high between 70 and 80 years. The apparent female predominance is related to the greater life expectancy in women. The study of several conjugal cases does not suggest the intervention of an infectious agent. The same is true for isolated cases where the responsibility of a bacterial or viral agent has not been demonstrated. Other environmental factors (sun exposure, life-style, socio-professional classification) do not affect the incidence of the disease. The genetic background would seem to be of particular importance. This study found a significantly higher prevalence of HLA DR4 antigen, confirming the results of American and British studies. However, in contrast to previous studies, this series did not confirm an increase in HLA B8 antigen.

[Primary Gougerot-Sjögren syndrome with necrotizing polymyositis: favorable effect of hydroxychloroquine]. / Syndrome de Gougerot-Sjögren primitif avec polymyosite necrosante: effet favorable de l'hydroxychloroquine.

A 61-year-old woman was admitted for acute arthralgias and proximal weakness in all four limbs. Clinical examination found xerostomia, xerophthalmia, enlarged parotid glands. The Schirmer test was positive and the salivary gland biopsy showed a mononuclear, lymphoid, sometimes nodular infiltrate. A muscle biopsy showed necrotizing myopathy with perifascicular atrophy. The patient refused steroids and was given hydroxychloroquine (600 mg daily). She improved gradually, and one year later there were no further complications. The favorable course both of Sjögren's syndrome and polymyositis with hydroxychloroquine treatment is unexpected. This led us to suppose that both could have had a common cause.

[Limitations of normal eosinophilia or possible influence of nonsteroidal anti-inflammatory agents]. / Les limites de l'éosinophilie "normale" ou l'influence possible des anti-inflammatoires non stéroïdiens.

Statistical analysis of eosinophilia of 331 patients at the onset of hospitalisation define a logarithmic standard population of 327 patients whose eosinophilia rates stand between 0 and 810/mm3, among them 24 lies between 500 and 810, and 4 whose eosinophilia rise over 1,000/mm3. These 4 patients had transient hypereosinophilia whose etiology was defined only in 2 cases. The 24 patients with eosinophilia between 500 and 810/mm3 were significantly more frequently men, with rheumatoid disease (specially sciatica) and users of non steroidal antiinflammatory drugs (NSAID) than the general population. It suggest that NSAID may rise eosinophilia rates inside normal limits.

[Evaluation of drug consumption by hospitalized patients. Prospective survey]. / Evaluation de la consommation médicamenteuse des sujets hospitalisés. Enquête prospective.

Drug use was assessed in a prospective 6-month study carried out in the Department of General Medicine V and Rheumatology of the Nantes Regional Hospital Center. An attempt was made to evaluate consumption as realistically as possible, and a judgment was made based on the consensus of the medical team as to whether the prescription was justified or not. The mean number of drugs consumed by each patient was 2.8; 60% being prescribed for cardiovascular and neuropsychiatric purposes. As 33% of the prescriptions were considered unnecessary, it would appear that use was excessive in these hospitalized patients.

[Iatrogenic disease. Prospective study, relation to drug consumption]. / Pathologie iatrogène. Enquête prospective, lien avec la consommation médicamenteuse.

The role of drug use in iatrogenic pathology was studied prospectively in a hospital department. It wa s found that patients received an average of 2.8 drugs and that 30% of the prescriptions were unnecessary. During this six-month study of all patients admitted to a general medicine and rheumatology department, there were 67 iatrogenic accidents affecting 12.3% of the population: 41.8% of the accidents were directly responsible for hospitalization, 35.4% were due to therapy initiated during hospitalization and 32.8% occurred during the hospital stay but were the result of earlier treatment. Moreover, 26% of the accidents were related to what was considered to be unnecessary therapeutics. This study shows that elevated iatrogenic pathology was due to excessive drug use. However, the degree of severity of the adverse effects varied considerably, and some could have been easily avoided.

[Paraneoplastic dermatopolymyositis]. / Dermatopolymyosites paranéoplasiques.

In a retrospective study covering 11 years, 59 adult cases of dermatomyositis or polymyositis were reviewed. The disease was of paraneoplastic origin in 9 cases (15,25 p. 100), the same proportion as that found in the literature (14,6 p.100). The striking female predominance (8/9 cases) and the patients' mean age (61.11 years) were higher than those noted in non-paraneoplastic dermato- and polymyositis in adults (female predominance 34/50, mean age 52.9 years). Clinically, out of 9 patients 7 had dermatomyositis and 2 had polymyositis. The disease was discovered at the same time as the neoplasia in 7 of the 9 cases. Muscle histology showed that in contrast with non-paraneoplastic cases of dermatomyositis-polymyositis those of paraneoplastic origin had moderate lesions without fibrosis.

[Iatrogenic drug-induced diseases, requiring hospitalization, in patients over 65 years of age. 1-year retrospective study in an internal medicine department]. / Pathologie iatrogène médicamenteuse après 65 ans, responsable d'une hospitalisation. Etude rétrospective sur 1 an dans un service de médecine interne.

Iatrogenic pathology is mainly seen in the elderly. In a one year retrospective study, we showed that drug toxicity was responsible for 87 cases requiring hospitalisation in patients aged 65 years and above (7.7% of hospital admission for patients over 65 years). The major manifestations were: 21 cases of sera electrolyte disturbance, 19 concerning gastro-intestinal tract and liver, 16 cardiological disorders, 13 neurological complications, ten involving the endocrine system, six hematological complications. The most common drugs involved were: antihypertensive agents (36%), of which 20.5% were diuretics, psychotherapeutic drugs (24.8%), anti-inflammatory drugs (17.8%). The average cost per patient was calculated to 20,602 FF per patient. Impossibility for direct return to the original dwelling place was another complication in 29% of hospitalisations related to iatrogenic disorders. The high number of drugs taken daily increases the risk of drug interactions which was responsible for iatrogenic accidents in 12.6% of the patients of this study.

[Clavicle osteolysis in MacDuffie's syndrome]. / Ostéolyse claviculaire au cours d'un syndrome de MacDuffie.

MacDuffie's syndrome emerges from systemic vasculitis as including recurrent urticaria-like skin lesions, episodic angio-oedemas, articular manifestations and abdominal pains, with a tendency to affect women. Renal involvement may burden the prognosis. Hypocomplementemia is usual by deficiency of the classical pathway. Corticosteroid therapy is habitual. The case reported, fitting to this nosological entity offers two new features: in the first place, the osteolysis of the distal part of the clavicle whereas the joints are not affected by destroying lesions; then the non steroidal anti-inflammatory drugs was effective within a period of one year without complication.

[Abiotrophia related endocarditis: contribution of molecular biology]. / Endocardites liées aux bactéries du genre Abiotrophia: apport de la biologie moléculaire.

BACKGROUND: Former nutritionally-deficient (variant) streptococci were recently separated from other streptococci viridans to constitute a new Abiotrophia genus, subdivided into two species, Abiotrophia defectiva (ex-Streptococcus defectivus) and Abiotrophia adjacens (ex-Streptococcus adjacens). CASE REPORT: A woman was admitted to the hospital because of purpura and fever. Vegetations were shown by trans-esophageal echocardiography. Although blood samples were positive within 24 hours, accurate identification of the morphologically and biochemically streptococcus-like bacteria that had grown remained impossible. Molecular biology technicals permitted to identify Abiotrophia defectiva, ex-Streptococcus defectivus. DISCUSSION: This new genus is important to be identified in clinical practice, because of the increased virulence of these bacteria, when compared with Streptococci viridans. They are also frequently penicillin-resistant. Molecular biology technicals allow precocious diagnosis, and make possible the improvement of the prognosis.

[Temporal artery biopsy]. / Biopsie d'artère temporale.

Temporal artery biopsy is performed to confirm the diagnosis of giant cell arteritis. This proof is quite absolute and allows using corticosteroid treatment associated with considerable morbidity in elderly patients. Biopsy is necessary in patient supposed to suffer from giant cell arteritis. Treatment test, which is often difficult to interpret, is done only when temporal artery biopsy is impossible. The biopsy is easy, bilateralisation improves its efficiency. Sometimes the temporal artery biopsy allows to diagnose other vasculitides. Although this biopsy is easy, a strict clinical reflexion must precede its realisation.

[Longitudinal myelitis in systemic lupus erythematosus]. / Myélite longitudinale lupique.

INTRODUCTION: Myelitis occurs in less than 5% of the patients during the disease course of systemic lupus erythematosus (SLE). Longitudinal myelitis, characterized by inflammatory involvement of at least four medullar segments, is a particular form of myelitis. CASE REPORT: We report a 31-year-old woman with SLE, admitted for paraparesia and delirium. Lumbar puncture and MRI led to the diagnosis of longitudinal myelitis. The patient rapidly improved after corticosteroid therapy. CONCLUSION: Transverse myelitis in SLE patients has been already commonly reported, but longitudinal myelitis is uncommon. Longitudinal myelitis has to be suspected in case of paraplegia or tetraplegia, with sensory defects and bladder dysfunction. MRI shows typically T2 medullar hypersignals. This may result in neurologic sequela. Cyclophosphamide has been used in patients where corticosteroids were inefficient.