[Systemic sarcoidosis and membranous glomerulonephritis]. / Sarcoïdose systémique et glomérulonéphrite extra-membraneuse.

Renal involvement in sarcoidosis is rare and more often related to calcium metabolism disorders or granulomatous interstitial nephritis. Glomerulonephritis is exceptional. There may be a long latency period between the development of active sarcoidosis and glomerular involvement and inversely. We report a case membranous glomerulonephritis revealing systemic sarcoidosis.

[Cardiac tamponade and myocarditis in Churg-Strauss syndrome]. / Tamponnade et myocardite aiguë au cours d'un syndrome de Churg-Strauss.

INTRODUCTION: The successive occurrence of pericardial tamponade and myocarditis during a Churg-Strauss syndrome is exceptionally described. We report a patient in whom pericardial tamponade and myocarditis were the presenting manifestation of a Churg-Strauss syndrome. CASE REPORT: A 58-year-old woman was admitted because of alteration of the clinical status with eosinophilia. One month ago, she was hospitalized for a pericardial tamponade treated by pericardial drainage. Acute myocarditis was diagnosed on chest pain during the second hospitalization. The etiologic inquiry ended in the diagnosis of Churg-Strauss complicated with a double cardiac involvement. A good response of clinical and biological anomalies was obtained after corticosteroid and immunosuppressive treatment. CONCLUSION: Isolated or multiple involvements of cardiac tunics should lead to make diagnosis of systemic vasculitis. A complete initial assessment and a close observation of the patients followed for Churg-Strauss syndrome is imperative to detect a cardiac achievement and set up an early treatment.

[Lupus pancreatitis: A case series of six patients]. / La pancréatite lupique : une série de six cas.

PURPOSE: The occurrence of acute pancreatitis in systemic lupus erythematosus (SLE) is known but rare, and is exceptionally the presenting manifestation. Its pathogenesis is multifactorial, and it is difficult to separate what belongs to vasculitis, thrombotic phenomena in the context of an associated antiphospholipid syndrome, or iatrogenic complications. We report on six cases of lupus pancreatitis. METHODS: This is a retrospective monocenter study of 110 patients with SLE. The diagnosis of lupus pancreatitis was established after exclusion of other causes of pancreatitis. RESULTS: Five women and one man (5.4%) with a mean age of 36.3 years presented with lupus pancreatitis. In four patients the pancreatitis was concurrent with the diagnosis of SLE and it occurred later during an exacerbation of the disease in the two remaining patients. In all patients, pancreatic manifestations were associated with other organ involvement. Clinical manifestations were: abdominal pain (n=6), vomiting (n=3), and fever (n=3). Elevated pancreatic enzyme was noted in all cases. All patients were treated by high doses of glucocorticoids. The outcome was favorable in five patients, and one patient died. CONCLUSION: Pancreatitis may be the presenting manifestation of SLE. Its pathogenesis is often multifactorial. The outcome is usually favorable with corticosteroids.

[Cardiac tamponade as an initial manifestation of systemic lupus erythematosus]. / Tamponnade cardiaque révélatrice d'un lupus érythémateux systémique.

Although pericarditis is the most frequent cardiac involvement in systemic lupus erythematosus (SLE), cardiac tamponade is very rare as an initial manifestation of this disease. We report the case of a 27-year-old patient in whom the diagnosis of SLE was discovered during cardiac tamponade.

[Pulmonary aneurysms in Behçet's disease completely resolved after medical therapy]. / Anévrisme pulmonaire complètement résolu après un traitement médical au cours de la maladie de Behçet.

Pulmonary artery aneurysm in Behcet disease compromises seriously the diagnosis since its fatal rupture is still the major cause of death in this disease. Pulmonary artery aneurysm requires urgent management and several treatments have been proposed, including steroids, immunosuppressive drugs, embolisation and surgery. Herein, we report a patient with Behçet's disease in whom multiple pulmonary artery aneurysms were completely resolved after a combined treatment with corticosteroids and azathioprine. Pulmonary artery aneurysms in Behçet's disease are reported to indicate poor prognosis and high mortality. Computed tomographic scans of the chest and angiography are the most common diagnostic procedures used in the diagnosis or evaluation of pulmonary artery aneurysm. Immunosuppression is the main therapy for the treatment of a vasculitis.

[Bronchopulmonary amyloidosis: report of four cases]. / L'amylose bronchopulmonaire : à propos de quatre cas.

Amyloidosis limited to bronchopulmonary apparatus is expressed as tracheobronchial deposits in diffuse plaques that can cause stenosis or parenchymal nodules or masses. In this regard, we report four cases of pulmonary amyloidosis and discuss the diagnostic difficulties of this location. These are two women and two men aged 60, 68, 44 and 57 years. They presented a pulmonary parenchymal amyloidosis in all cases associated with bronchial in one case. The diagnosis was confirmed by histology in all cases. The staging was negative in all cases. The evolution was marked by the stabilization of the lesions in all cases. Localized amyloidosis, which may be the only telltale sign of a systemic illness, its diagnosis requires finding other locations to better tailor the treatment strategy.

[Parotid mucormycosis: A new case and review of the literature]. / Mucormycose parotidienne : une nouvelle observation et revue de la littérature.

INTRODUCTION: Mucormycosis is a rare, devastating, fungal infection, which disproportionately affects non-controlled diabetic patients, notably during ketoacidosis. The authors report the case of cervical mucormycoses with a particularly favorable evolution in diabetic woman. REPORT: A 54-year-old woman, type 2 diabetic, had presented a left lateral cervical mass. The diagnosis was confirmed by histological examination. She was treated with Amphotericin B with favorable evolution. CONCLUSION: The mucormycose is a rare infection. The treatment is medical and surgical. The prognosis is severe with an overall mortality rate of 40%.

[Unusual presentation of Wegener's granulomatosis]. / Présentation inhabituelle d'une granulomatose de Wegener.

We report an unusual case of Wegener's granulomatosis revealed by spleen infarction and complicated by reno-vascular hypertension. A 33-year-old man with a history of spleen infraction and cerebral venous thrombosis was admitted for malignant hypertension, renal failure and nephritic syndrome. On Doppler renal ultrasonography, ostial stenosis of the right renal artery was evidenced. Right kidney was non functional on scintigraphy and the patient underwent a right nephrectomy. Granulomatous vasculitis of the right renal artery was found but anti-neutrophilic cytoplasmic antibodies were absent. The diagnosis of Wegener's granulomatosis was suspected. The patient was treated with cyclophosphamide, corticosteroids and plasma exchanges, and renal function markedly improved.

[Liver involvement in sarcoidosis: study of seven cases]. / L'atteinte hépatique au cours de la sarcoïdose: à propos de sept cas.

INTRODUCTION: Sarcoidosis is a systemic disorder of unknown aetiology that may involve many organs including the liver. METHODS: This is a monocentre retrospective and descriptive study over 12 years of patients with systemic sarcoidosis who presented with liver involvement. RESULTS: Amongst 21 patients with systemic sarcoidosis, seven presented a liver involvement (five males and two females; mean age 42.5 years). The liver involvement was the presenting manifestation in three. Liver enlargement was noted in five patients and abnormal liver function tests in four. In one patient, abdominal computed tomographic scan showed a nodular hepatomegaly. Liver histology demonstrated typical granulomatous lesions in six. One patient developed a Budd-Chiari syndrome that responded favourably to corticosteroids. Six out of the seven patients were treated with corticosteroids. CONCLUSION: Liver involvement in common in sarcoidosis and commonly asymptomatic. Treatment is not systematic.

[Etiological profile of digital necrosis of the upper limbs: analysis of 25 cases]. / Profil étiologique des nécroses digitales des membres supérieurs : analyse de 25 observations.

AIM: To investigate the etiologies of the upper limb digital necrosis based on a retrospective analysis of 25 cases. PATIENTS AND METHODS: We retrospectively reviewed the medical records of patients treated for digital necrosis of the upper limb in four departments of internal medicine from January 1997 to December 2003. RESULTS: There were 16 women and nine men, mean age 55 years. Eleven patients were smokers. Raynaud's phenomenon was noted in 12 cases. Connective tissue diseases were the most common cause (nine cases), all of them were women. The second cause was atherosclerosis (five cases) and Buerger's disease (five cases). In the other cases, the following diagnoses were found: vasculitis (three cases) and neoplasm (two cases). No cause could be identified in one female smoker. CONCLUSION: Digital necrosis is a common symptom, revealing a vascular pathology. Its causes are diverse. In women, it first suggests a connective tissue disease whereas in men, a diffuse arteriopathy. The etiological diagnosis strategy should consider drug intake, anamnesis and Raynaud's phenomenon history. However, in all cases the etiology investigations should not delay the treatment in order to preserve functional prognosis.

[Interstitial pneumonia complicating amyopathic dermatomyositis: a case report]. / Pneumopathie interstitielle compliquant une dermatopolymyosite amyopathique : à propos d'un cas.

Amyopathic dermatomyositis (ADM) is a clinical subtype of dermatomyositis, characterized by the absence of motor weakness and the presence of normal muscle enzyme levels. ADM is sometimes accompanied by neoplasm or interstitial pneumonia that shows a rapid progressive course both of them are associated with a poor prognosis. A 56-year-old woman with no medical history was referred to the department of medicine because of arthralgia with a remarkable weight loss. She also complained of rapidly progressive dyspnea, cough and photosensitivity. Physical examination on admission showed scaly erythema on the dorsum of the hands (Gottron sign) and periorbital edema with a purplish appearance (heliotropic rash), arthritis, but no muscle weakness. Auscultation of the chest identified audible fine crackles on the lower aspects of both lungs. Results of laboratory findings on admission revealed a lymphopenia. The serum creatine kinase and serum lactate dehydrogenase concentration were normal. IRM muscle and electromyography were normal. Antinuclear antibody was positive 1:80 and anti-Jo-1 antibody and other autoantibodies to specific antigens were all negative. High resolution computed tomographic chest scans also revealed diffuse ground-glass opacities in both lungs with basilar predominance. Arterial blood gas analysis revealed hypoxia and hypocapnia. LBA was not performed because of the deterioration of respiratory symptoms. There was no neoplasm associated. The diagnosis of ADM complicated with ADM rapidly progressive interstitial pneumonia was made. Despite of IV methylprednisolone pulse therapy (1g*day-1 for 3 days) and cyclophosphamide, she died by respiratory failure.

[Giant insulinoma]. / Un insulinome géant : à propos d'un cas.

Islet-cell tumors are the most common neuroendocrine tumors that arise from the endocrine pancreas. They are typically benign and sporadic. Diagnosis is generally established late because clinical signs lack specificity. The insulinoma is difficult to localize since it is very small in size, often not exceeding 2cm. We report an exceptional case of giant insulinoma initially revealed by a pseudo-polycythemia in an 80-year-old man. He had been treated for hypertension for a few months. Routine biological investigations showed elevated hematocrit and haemoglobin, suggesting Vaquez disease. History taking revealed recent episodes of nocturnal agitation. On admission, he had reddish skin with a suspected enlarged spleen, but total blood volume was normal. Imaging studies showed a voluminous tumor located between the pancreas and the spleen. The presence of an insulinoma was confirmed on the basis of an elevated level of proinsulin at the time of an asymptomatic episode of hypoglycemia. Spleno-pancreatectomy was performed. Histopathological examination revealed a malignant, well-differentiated neuroendocrine malignant tumor.

[Clinical spectrum of cobalamin deficiency in Tunisia]. / Le spectre clinique des déficits en cobalamines en Tunisie.

PURPOSE: the aim of this study was to determine the prevalence of cobalamin (vitamin B12) deficiency in different populations of patients with clinical manifestations associated or secondary to cobalamin or folates deficiency and to analyse the demographic, clinical, paraclinical investigations in cobalamin deficient patients in Tunisia. METHODS: it was a prospective (1999-2001) multicenter study of 604 patients divided into four groups. The first group is composed of 478 consecutive patients with anaemia and/or macrocytosis with megaloblastic haemopoiesis on bone marrow examination without myelodyslasic or malignancy signs. The second group is made up of 34 patients with unexplained neurological symptoms without the presence of anaemia. The third group was composed of 82 invidious with isolated psychiatric disorders and the 10 patients with Hashimoto thyroïditis constituted the last group. RESULTS: serum cobalamin level was low in 98 %, 23%, 14% of cases, respectively, in the first three groups. Only one case of patients with Hashimoto thyroiditis has serum cobalamin deficiency. Pernicious anaemia (Biermer's disease) was established by dual isotope schilling examination in 103 patients among a sample of 120 serum cobalamin deficient patients (86%). The median age at presentation was 45.5 years. Severe chronic atrophic gastritis was diagnosed in 97.5% of patients with Biermer's disease. Serum antibodies against intrinsic factor and gastric parietal cells were detected in (42.5%) and (60.6%) patients, respectively; (25.5%) patients had the both types of antibodies. 23.4% patients were positive for antithyroid antibodies. Anti-nuclear antibodies were detected in 3% patients. CONCLUSION: an interesting finding of our study was the high frequency of cobalamin deficiency in Tunisia, particularly in relative young patients. Our patients had classic features of florid cobalamin deficiency (severe haematological manifestations and neuro-psychiatric disorders). The main underlying causes of such deficiencies were Biermer's disease. Subtle clinical manifestations should be recognized and investigated even in young patients at risk.

[Pituitary and ovarian mass syndrome in primary hypothyroidism]. / Syndrome de masse hypophysaire et kystes ovariens dans un cas d'hypothyroïdie périphérique.

INTRODUCTION: Hyperprolactinemia and a pituitary mass syndrome can occur in patient with primary hypoyhroidism. In young women, hypothyroidism can be associated with ovarian cysts. EXEGESIS: We report a case of a 30 year-old women who was treated for an anemia. She had clinical and biological signs of primary hypothyroidism with hyperprolactinemia at the biology. Pituitary MRI showed a pituitary mass. The pelvic ultrasound examination and CT scan showed a right ovarian cyst. The subsequent thyroid hormone replacement was associated with a clinical, a biological and radiological resolution. It was a pituitary and ovarian mass syndrome secondary to primary hypothyroidism. CONCLUSION: The recognition of these associations may eliminate unnecessary surgery and lead to the choice of hormone replacement therapy.

[Estrogen use in systemic lupus erythematosus]. / Rôle des estrogènes dans le lupus érythémateux systémique.

The role of exogenous estrogen in the initiation and maintenance of human systemic lupus erythematosus (SLE) remains very controversial. To review the current literature of the safety of using exogenous estrogens in patients with SLE, a Medline search for articles published between 1970 et 2004 regarding this relationship was performed. Although cohort studies suggest an increase in the incidence of SLE with both oral contraceptives and hormone replacement therapy, recent retrospective studies suggest that the risk of flares is not increased with hormone replacement therapy. Large prospective double blind placebo controlled studies inclusive of all ethnic groups such as the Safety of Estrogen in Lupus Erythematosus National Assessment (SELENA) trial had to provide the basis for definitive recommendations but it had been interrupted after WHI study results.