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Les auteurs mettent au point a travers un cas clinique des metastases pulmonaires secondaires a un cancer renal, la place des antiangiogeniques en tant que therapeutiques ciblees dans le traitement du cancer renal metastatique. L’evolution de ce cas sous traitement avec le sorafenib etait marquee par une nette regression des metastases pleuropulmonaires sur la radiographie thoracique et le scanner thoracique de controle apres un an de traitement, et la survie actuellement dans ce cas est de 15 mois, et aucun effet secondaire n’a ete notifie. Cette classe therapeutique commence a prendre une place dans les strategies therapeutiques de la prise en charge adjuvante du cancer du rein chimioresistant, ce qui apparait comme une avancee surtout que ce domaine connaissait peu de therapeutique efficace.

Resume Introduction L’enquete etiologique devant une granulomatose systemique doit considerer certaines formes rares de l’infection tuberculeuse. Le rhumatisme tuberculeux de Poncet (RTP) en est l’exemple. C’est une entite rare et sujette a controverse. Observation Un patient de 32 ans a presente une polyarthrite febrile avec eruptions cutanees erythematopapulaires. Le bilan a montre un syndrome inflammatoire biologique et des adenopathies mediastinales. Les biopsies pratiquees ont mis en evidence une granulomatose cutanee et ganglionnaire mediastinale. Les lesions cutanees ont spontanement regresse. La culture du prelevement ganglionnaire etait positive au BK. L’evolution etait favorable sous traitement antibacillaire avec resolution des signes articulaires en quelques jours. Conclusion Le RTP peut etre la circonstance de decouverte d’une tuberculose viscerale evolutive. Sa connaissance doit faire rechercher une tuberculose devant une granulomatose systemique avec atteinte articulaire et cutanee pouvant simuler une sarcoidose.

Bilateral primary non-Hodgkin's lymphoma (NHL) of the adrenals is uncommon. Less than 70 cases have been described in the literature. The symptoms of disease are variable and depend on the tumor size and the presence of adrenal insufficiency . We report a case of large diffuse B-cell adrenal lymphoma discovered in a 40-year-old woman presenting bilateral lumbar pain. Hormonal exploration demonstrated adrenal insufficiency. Imaging explorations showed a large and bilateral adrenal mass. Percutaneous CT-guided biopsy of the adrenal and search for extension revealed primary bilateral adrenal lymphoma. After glucocorticoid substitution, treatment was based on a CHOP regimen chemotherapy; outcome was unfavourable after the second cure; the patient died from septic shock. The diagnosis of primary adrenal non-Hodgkin lymphoma should be investigated in patients with a rapidly growing bilateral adrenal mass associated with adrenal insufficiency.

The case of a 52-year-old man with retroperitoneal fibrosis and ankylosing spondylitis is described. Inflammatory low back pain and acute renal insufficiency prompted a computed tomography scan of the abdomen with contrast agent injection. A fibrous sheath surrounding the aorta and attracting the ureters toward the midline was seen, strongly suggesting retroperitoneal fibrosis. The diagnosis of ankylosing spondylitis was based on the presence of inflammatory low back pain responsive to nonsteroidal anti-inflammatory drugs, syndesmophytes at the lumbar and cervical spine, bilateral sacroiliitis, and presence of the HLA-B27 antigen. Prednisone therapy in a daily dosage of 1 mg/kg induced a marked improvement. Only nine cases of concomitant retroperitoneal fibrosis and ankylosing spondylitis have been reported. These two conditions share similarities in some of the etiologic factors and anatomic localizations, suggesting that both may stem from a predisposition to fibrotic diseases.

Resume Les auteurs rapportent l’observation d’un homme de 52 ans presentant une fibrose retroperitoneale (FRP) associee a une spondylarthrite ankylosante (SPA). La FRP est diagnostiquee a l’occasion de lombalgies inflammatoires et d’une insuffisance renale aigue. La tomodensitometrie abdominale avec injection de produit de contraste a mis en evidence un aspect de manchon fibreux periaortique avec attraction mediane des ureteres, tres evocateur de FRP. Le diagnostic de spondylarthrite ankylosante est pose devant l’existence de lombalgies inflammatoires repondant aux anti-inflammatoires non steroidiens, d’une syndesmophytose lombaire et cervicale et d’une sacro-iliite bilaterale avec typage HLA B27 positif. Le patient est traite par prednisone a la dose de 1 mg/kg/j avec une nette amelioration. L’association FRP et SPA est tres rare, neuf observations seulement sont retrouvees dans la litterature. Les analogies de certaines conditions etiologiques et de localisations anatomiques rapprochent ces deux affections et suggerent un terrain predisposant aux transformations fibreuses.

Resume Introduction. – La vascularite urticarienne hypocomplementemique de McDuffie est une entite rare recemment individualisee au sein des vascularites. Nous en rapportons deux nouvelles observations. Exegese. – Cas 1 : patiente de 41 ans hospitalisee pour polyarthralgies inflammatoires avec eruption urticarienne generalisee evoluant dans un contexte febrile. La biologie avait montre une proteinurie, une serologie rhumatoide positive, des anticorps antinucleaires negatifs et une hypocomplementemie. La biopsie cutanee avait montre une vascularite leucocytoclasique. La biopsie renale avait montre une glomerulonephrite extramembraneuse. L’evolution sous corticotherapie a ete marquee par des poussees entrecoupees de remissions. Cas 2 : patiente de 39 ans, suivie pour polyarthrite chronique bilaterale et symetrique non deformante et non destructrice avec syndrome de Raynaud, eruption urticarienne et toux chronique avec dyspnee revelant une fibrose pulmonaire interstitielle diffuse sur la radiographie et la tomodensitometrie thoracique. La biologie avait montre un syndrome inflammatoire, une serologie rhumatoide positive, une hypocomplementemie, des anticorps antinucleaires negatifs. La biopsie cutanee avait montre une vascularite leucocytoclasique. La patiente a ete traitee par prednisone et cyclophosphamide avec une evolution favorable. La vascularite urticarienne hypocomplementemique de McDuffie est une affection a manifestations systemiques diverses. Son existence est encore contestee par certains. Son traitement n’est pas codifie. Il varie en fonction des atteintes observees. Il reste fonde essentiellement sur les corticoides. Conclusion. – La vascularite urticarienne hypocomplementemique de McDuffie est une entite defendable au sein des vascularites urticariennes par ses particularites cliniques et biologiques.

Pyomyositis is a primary pyogenic muscular infection. It has been originally described in the tropics. Presenting signs and symptoms are not specific. It is frequently misdiagnosed in the early stages. We report the case of a 47-year-old woman with systemic lupus erythematosus treated with corticosteroids who presented a thigh pyomyositis evidenced by echography and MRI. Outcome was uneventful after antibiotics and surgical drainage. Pyomyositis is increasingly described among immunocompromised people, including lupus patients treated with corticosteroids.

Resume Nous rapportons un cas de myopathie severe revelant une hypothyroidie et un syndrome tumoral hypophysaire avec hyperprolactinemie. Observation Un homme âge de 33 ans souffre depuis six mois de myalgies, crampes et faiblesse musculaire. Le bilan revelait un taux eleve de creatine phosphokinase, une hypothyroidie auto-immune, une hyperprolactinemie et un syndrome tumoral hypophysaire. L'electromyogramme etait normal et la biopsie musculaire ne montrait pas de signes inflammatoires. L'evolution etait rapidement favorable avec un traitement par L.thyroxine. Discussion La frequence de la myopathie hypothyroidienne est estimee entre 30 et 80 %. Ses principaux symptomes sont la fatigabilite musculaire, les crampes et les myalgies. Par ailleurs, l'hyperprolactinemie avec syndrome tumoral hypophysaire, observee ici, releve d'un mecanisme reactionnel a l'hypothyroidie basse. Ces differentes manifestations de l'hypothyroidie regressent avec un traitement par L.thyroxine. Conclusion Bien qu'il soit rare qu'une myopathie inaugure le diagnostic d'hypothyroidie, une endocrinopathie doit etre evoquee devant toute myopathie.

L’hypoparathyroidie est rarement diagnostiquee chez les patients atteints de lupus. Observation Mme HS, âgee de 39 ans est suivie depuis 3 ans pour lupus. Elle fut hospitalisee pour des paresthesies aux membres inferieurs, un syndrome depressif avec insomnie et un amaigrissement chiffre a 5 kg. Son bilan ne montre pas de signe d’activite du lupus mais une hypoparathyroidie. La supplementation vitamino-calcique a permis de retablir le bilan phospho-calcique et la normalisation de l’espace QT. Discussion L’association lupus-hypoparathyroidie est rarement rapportee dans la litterature. Les auteurs suggerent l’hypothese d’un mecanisme physiopathologique commun expliquant la combinaison des deux pathologies. L’idee la plus plausible est celle d’une predisposition genetique ou une generalisation du processus auto-immun.

Infectious complications of sickle cell disease are common and can be serious and difficult to diagnose. Epidemiological aspects of these infections are well documented. The most common germ in children is pneumococcus followed by Haemophilus influenzae and minor salmonella. In adults gram-negative germs including minor salmonella are the most frequent. The purpose of this report is to describe a case of a Citrobacter freundii spondylitis with prevertebral abscess extending to dorsal and lumbar spinal areas. Diagnosis was made during work-up for persistent vaso-occlusive manifestations. Treatment consisted of percutaneous lumbar drainage associated with antibiotherapy.

Pyomyositis is a primary pyogenic muscular infection. It has been originally described in the tropics. Presenting signs and symptoms are not specific. It is frequently misdiagnosed in the early stages. We report the case of a 47-year-old woman with systemic lupus erythematosus treated with corticosteroids who presented a thigh pyomyositis evidenced by echography and MRI. Outcome was uneventful after antibiotics and surgical drainage. Pyomyositis is increasingly described among immunocompromised people, including lupus patients treated with corticosteroids.

The aetiological inquiry in the presence of systemic granulomatosis should consider some uncommon forms of tuberculosis infections. For instance, Poncet's tuberculous rheumatism is a rare entity subject to controversy.A 32-year-old man presenting febrile polyarthritis associated with spontaneously vanishing erythematous papular skin rash. The tests revealed a biological inflammatory syndrome and mediastinal lymph nodes. The biopsies showed cutaneous and mediastinal adenoid granulomatosis. The cutaneous lesions resolved spontaneously. The culture of the ganglionic sample was positive. The evolution was favourable under treatment and the articular pain disappeared within a few days.Poncet's tuberculous rheumatism may underly evolving visceral tuberculosis. It's presence requires a search for tuberculosis when systemic granulomatosis with cutaneous and articular involvement may simulate sarcoidosis.

IgDkappa myeloma accounts for less than 2% of all myeloma cases. Orbital involvement is uncommon in myeloma and the authors suggest a possible predilection for IgD myeloma to involve the orbit.A 67-year-old women was admitted for diffuse bone pain and painful ptosis of the right eye. The symptoms had progressed over approximately 1 year and were associated with a severe decline in general health. The biochemical, hematological, and radiological investigations suggested the diagnosis of an IgD kappa myeloma. Orbital x-ray showed destruction of the greater and lesser right wing of the sphenoid bone. Treatment was polychemotherapy. The course of the disease was very rapid and death occurred within 17 months from renal failure and amyloidosis.Orbital involvement by myeloma occurs in 0.3% of cases and the majority are unilateral. The typical orbital symptoms include ptosis, diplopia, loss of vision, and pain. This was not considered to contribute to poor prognosis but may affect the functional prognosis.

Bilateral primary non-Hodgkin's lymphoma (NHL) of the adrenals is uncommon. Less than 70 cases have been described in the literature. The symptoms of disease are variable and depend on the tumor size and the presence of adrenal insufficiency . We report a case of large diffuse B-cell adrenal lymphoma discovered in a 40-year-old woman presenting bilateral lumbar pain. Hormonal exploration demonstrated adrenal insufficiency. Imaging explorations showed a large and bilateral adrenal mass. Percutaneous CT-guided biopsy of the adrenal and search for extension revealed primary bilateral adrenal lymphoma. After glucocorticoid substitution, treatment was based on a CHOP regimen chemotherapy; outcome was unfavourable after the second cure; the patient died from septic shock. The diagnosis of primary adrenal non-Hodgkin lymphoma should be investigated in patients with a rapidly growing bilateral adrenal mass associated with adrenal insufficiency.

We report a case of a serious myopathy related to hypothyroidism with pituitary enlargement and hyperprolactinemia.Mr D.L 33 years old man suffering from myalgia, muscular weakness and cramps for six months. The laboratory check-up revealed a high serum creatin phosphokinase level, an autoimmune hypothyroidism, hyperprolactinemia and pituitary enlargement. The electromyogram was normal and the muscle biopsy showed no obvious inflammation. The outcome was favourable under L. thyroxin.The frequency of myopathy during hypothyroidism ranges from 30 to 80%. The main symptoms related are weakness, muscular cramps and myalgia. However, hyperprolactinemia and pituitary enlargement described in this case are due to reactional mechanism. These several hypothyroid manifestations improve remarkably under L.thyroxin.Proximal myopathy may rarely be displayed as the sole manifestation of hypothyroidism. Therefore, it is recommended that hypothyroid myopathy should be taken into account during differential diagnosis of proximal muscle weakness.

The aim of this study was to identify the spectrum of abnormalities revealed on high-resolution computerized tomography (HRCT) in patients with ankylosing spondylitis (AS), to compare findings with those of plain radiography and pulmonary function testing (PFT), and to look for correlations between lung involvement and AS severity. We prospectively studied 55 consecutive patients with a diagnosis of AS according to the modified New York criteria who attended our department over a period of 2 years. All patients had a detailed rheumatological examination and underwent plain chest radiography, chest HRCT and PFT. HRCT revealed abnormalities in 29 patients (52.7%), whereas plain chest radiography was abnormal in only 2. Abnormalities consisted of interstitial lung disease (ILD) ( n=4), apical fibrosis ( n=5), emphysema ( n=5), bronchiectasis ( n=4), ground glass attenuation ( n=2), and non-specific interstitial abnormalities ( n=26). Only apical fibrosis and bronchiectasis were statistically more frequent with increasing disease duration (significant trend chi(2)test, p=0.0029 and 0.028, respectively). PFT showed a restrictive process in 19 patients (34.5%). No correlation was noted between HRCT and PFT, nor with AS symptomatic and structural severity parameters. However, there was a statistically significant correlation between PFT and AS symptomatic and structural severity parameters. In conclusion,: this study confirms that the chest HRCT of patients with AS showed a great number of abnormalities undetectable by standard X-rays. The high incidence of lung abnormalities emphasizes the importance of excluding such a diagnosis in patients with AS even without respiratory symptoms.

The aim of the study was to evaluate the prevalence of sacroiliitis in a group of patients with Behcet's disease (BD). Pelvic X-rays of 27 patients with BD responding to the International Study Group of BD and 30 controls (15 AS and 15 sciatica) were read blind and sacroiliac involvement was graded according to the New York criteria. In a second step, patients or controls with equivocal sacroiliitis had a sacroiliac CT scan. Two patients with BD (7.4%) and all patients with AS had evident bilateral sacroiliitis (at least grade 2). One patient with BD and two patients with sciatica had equivocal sacroiliitis (grade 1). CT confirmed sacroiliitis in the two patients with BD and eliminated inflammatory sacroiliitis in the three other patients with equivocal sacroiliitis showing mild degenerative lesions. A review of the literature showed that sacroiliitis and AS are rarely associated with BD. There remains insufficient evidence to suggest that sacroiliitis is an intrinsic feature of BD and that BD belongs to the group of SpA.

Background Osteoporosis (OP) is now a well recognised feature of ankylosing spondylitis (AS). Dual energy x-ray absorptiometry, which is the most routinely used method to assess OP, fails to demonstrate spine OP in patients with late stage disease (syndesmophytes and facet joints ankylosis). Therefore, quantitative computed tomography (QCT) is the only method that can evaluate correctly the spinal OP in patients with advanced spinal changes. Objectives To study OP prevalence in AS using QCT and relationship between OP and AS symptomatic and structural severity. Methods 27 patients with AS were enrolled prospectively: 24 men (88.8%) and 3 women with mean age of 37.7 ± 9.2 years [17–66] and mean disease duration of 7.6 ± 4.7 years [0.4–19]. The patients were studied clinically, radiologically and by QCT. Results OP at the lumbar spine as defined by Z score Conclusion OP is frequent in AS. Patients with AS are more susceptible to develop OP when they have active and severe disease.

Hypocomplementemic urticarial vasculitis (HUV) described by McDuffie is a rare entity recently individualized among vasculitis. We report two new cases.Case 1: a 41-year-old woman presented in 1994 with inflammatory polyarthralgia, diffuse urticaria, fever, and weight loss. Biology showed proteinuria, positive rheumatoid factor with hypocomplementemia and negative immunological tests. Skin and renal biopsies showed leukocytoclastic vasculitis and extramembranous glomerulopathy, respectively. Outcome within steroid therapy was marked by alternating clinical improvement and relapses. Case 2: a 39-year-old woman presented in 1994 with inflammatory polyarthritis, diffuse urticaria, Raynaud phenomenon, cough and dyspnea. Chest x-rays and CT scan showed interstitial fibrosis and echocardiography revealed pericarditis. Biology showed positive rheumatoid factor with hypocomplementemia and negative antinuclear antibodies. Skin biopsy showed leukocytoclastic vasculitis. Corticosteroids and cyclophosphamide improved the patient's condition. McDuffie HUV is a disease with varied systemic manifestations. Its existence is still contested by some authors. Treatment is still empirical and depends on the clinical features. It is based primarily on corticosteroids.McDuffie HUV is a defensible entity among urticarial vasculitis because of its particular clinical and biological features.

Sweet's syndrome belongs to the group of neutrophilic dermatoses. We report the case of a 36-year-old man admitted for stiff neck and fever. He had a history of recurrent oral aphtous ulcers, orchitis, phlebitis, two episodes of febrile acute polyarthritis with interstitial pneumonia. He presented a stiff neck and a temperature of 40 degrees C for two days associated with an erythematonodular eruption of the right periocular region. Laboratory exams showed an inflammatory syndrome with hyperleukocytosis. Skin biopsy showed dermic neutrophilic infiltrates, confirming the diagnosis of Sweet's syndrome. The patient improved dramatically with corticosteroids: the temperature fell and neck stiffness and skin lesions disappeared. In light of this case with a rich cohort of extracutaneous manifestations, we reviewed the literature on the characteristics of Sweet's syndrome. This syndrome is commonly associated with inflammatory and neoplastic diseases.

The incidence of pleuropulmonary disease in ankylosing spondylitis (AS) varies from 0 to 30% in the medical literature.1–4 The most frequently recognised manifestations are upper lobe fibrosis, mycetoma formation, and pleural thickening. The advent of high resolution computed tomography (HRCT) made it possible to examine the entire lung parenchyma and pleura in many conditions with diffuse lung disease by a non-invasive method. Consecutive patients with a diagnosis of AS according to the modified New York criteria5 who attend our department during one year were included in the study. All patients had a prospective rheumatological assessment conducted by two rheumatologists (AEM and AB) using a structured questionnaire, a pulmonary function testing measurement, posteroanterior chest radiography; on the same day an HRCT of the thorax was performed using …

Introduction Le myelome multiple a composante IgD Kappa est rare ( Observation Une femme, âgee de 67 ans, fut hospitalisee pour un syndrome douloureux osseux resistant aux traitements antalgiques usuels, un ptosis et une exophtalmie de l’œil droit evoluant depuis environ un an avec une importante alteration de l’etat general. Le bilan biochimique, hematologique et radiologique confirma l’existence d’un myelome a IgD Kappa. La tomodensitometrie de l’orbite montrait une osteolyse de la grande aile du sphenoide. Le traitement a consiste en une polychimiotherapie. La patiente est decedee au bout de 17 mois suite a une insuffisance renale et une amylose. Discussion L’atteinte de l’orbite au cours du myelome est rare (0,3 %). Elle est souvent unilaterale, et ses principaux symptomes sont le ptosis, la diplopie, les douleurs oculaires et la baisse de l’acuite visuelle. Elle ne constitue pas un element de mauvais pronostic, mais altere le pronostic fonctionnel.