Elderly-onset adult Still's disease.

Adult-onset Still's disease is a rare inflammatory disorder usually affecting young adults. Elderly-onset Still's disease (EOSD) is reported in some cases, commonly in Japan, the USA and Europe. One of the most commonly used criteria for diagnosing EOSD is Yamaguci criteria. In elderly patients more severe course of the disease and more complications may be expected than in the younger group of patients with Still's disease. The lungs involvement is rather rare manifestation of this disease. In our article we discuss the problem of both the development of Still's disease in the elderly and interstitial lung changes in the course of the disease, based on available literature and own cases from one centre.

Systemic lupus erythematosus, antiphospholipid syndrome and Hashimoto thyroiditis occurring in a patient with Niemann-Pick disease: a second case.

Lysosomial diseases and autoimmune diseases are systemic disorders. Their clinical manifestations can overlap with the broad spectrum of one another. Their association has been rarely reported. We report a new case of systemic lupus erythematous (SLE) associated to antiphospholipid syndrome (APS) and Hashimoto thyroiditis occurring in Niemann-Pick disease (NPD) type B patient. A 42-year-old woman with a familial history of NPD was diagnosed with a NPD type B at the age of ten. Twenty years later (2008), she complained of inflammatory arthralgia with acute dyspnea. She was diagnosed with SLE (according to ACR criteria) and Hashimoto disease with positive IgG anti-cardiolipin and IgA anti-beta2 glycoprotein. In 2018, she presented a left segmental pulmonary embolism. Antiphospholipid syndrome was retained. She was treated with steroids, hydroxychloroquine, anticoagulation therapy and levothyroxine. Her SLE treatment was re-enforced by cyclophosphamide because of corticosteroid dependence and recurrent hemolytic crises.

[Multifocal tuberculosis in immunocompetent patients]. / Tuberculose multifocale chez les immunocompétents.

Multifocal tuberculosis is defined as the presence of lesions affecting at least two extrapulmonary sites, with or without pulmonary involvement. This retrospective study of 10 cases aims to investigate the clinical and evolutionary characteristics of multifocal tuberculosis. It included 41 cases with tuberculosis collected between 1999 and 2013. Ten patients had multifocal tuberculosis (24%): 9 women and 1 man, the average age was 50 years (30-68 years). Our patients were correctly BCG vaccinated. The evaluation of immunodepression was negative in all patients. 7 cases had lymph node tuberculosis, 3 cases digestive tuberculosis, 2 cases pericardial tuberculosis, 2 cases osteoarticular tuberculosis, 1 case brain tuberculosis, 2 cases urinary tuberculosis, 4 cases urogenital tuberculosis, 1 case adrenal tuberculosis, 1 case cutaneous and 1 case muscle tuberculosis. All patients received anti-tuberculosis treatment for a mean duration of 10 months, with good evolution. Multifocal tuberculosis is difficult to diagnose. It can affect immunocompetent patients but often has good prognosis. Anti-tuberculosis therapy must be initiated as soon as possible to avoid sequelae.

Amyloïdosis, sarcoidosis and systemic lupus erythematosus.

The occurrence of renal and multiple organ Amyloïdosis is currently considered exceptional in the course of systemic lupus erythematosus. We report a case of a concomitant SLE and Amyloïdosis in a 57 year old female patient with hypothyroidism history, who presented with erythema nodosum, fever, arthralgia and sicca syndrome. Biological findings showed an inflammatory syndrome, renal failure, proteinuria (1g / 24h), positive auto antibodies and anti DNA. Lung radiology revealed medistinal lymphadenopathy, pleural nodules, ground glass infiltrates and pleuritis. Bronchial biopsy showed non specific inflammation. The salivary gland biopsy showed amyloïd deposits. This case report reminds us that lupus and Amyloïdosis association, although exceptional remains possible. The occurrence of Lofgren syndrome in this situation make the originality of this report.

A Seropositive Nodular Rheumatoid Polyarthritis without Arthritis: Does It Exist?

The rheumatoid polyarthritis is the most frequent chronic polyarthritis. It affects essentially the woman between 40 and 60 years. Rheumatic subcutaneous nodules and tenosynovitis are usually associated with seropositive symptomatic rheumatoid polyarthritis. It is, however, rare that they constitute the essential clinical expression of the disease. In this case, it makes dispute another exceptional form of rheumatoid arthritis such as rheumatoid nodulosis. A 60-year-old woman was hospitalized for tumefaction of the dorsal face of the right hand evolving two months before. The clinical examination found subcutaneous nodules from which the exploration ended in rheumatoid nodules with tenosynovitis. The evolution after four years was favourable under corticosteroid therapy, methotrexate, and colchicine.

Epidemiological and clinical features of giant cell arteritis in Tunisia.

BACKGROUND: Giant cell arteritis (GCA) is a systemic vasculitis of the elderly that could result in vision loss or even be life threatening. Unlike western countries, this disease is considered exceptional in Tunisia. OBJECTIVE: The aims of this study were to determine epidemiological and clinical features of GCA in Tunisian population and to identify management difficulties. PATIENTS AND METHODS: A multicentric study of 96 patients in whom GCA was diagnosed between 1986 and 2003. All patients fulfilled the ACR criteria for classification of GCA. RESULTS: The majority of cases (77%) were diagnosed since 1994. The male/female ratio was 0.88 and the mean age at the time of diagnosis was 70.8+/-7.7 years. Clinical features were characterized by gradual onset in 64.4% of cases. The most frequent clinical manifestations were headache (91.7%), abnormalities in temporal arteries (85.4%), severe ischemic manifestations (80.2%), constitutional symptoms (75%), and polymyalgia rheumatica (56.3%). Biological inflammatory syndrome was noted in all patients. Temporal artery biopsy established histological diagnosis in 73% of cases. All patients were treated by corticosteroids. Remission was obtained in 45.6%. Relapses occurred in 40.4% of cases and 30 patients were still receiving corticosteroids at the time of study. Four patients died and irreversible ischemic complications were noted in 15.6% of cases. Steroid adverse effects occurred in 56 patients. CONCLUSION: GCA is not exceptional to Tunisia. It occurs amongst elderly patients with no female predominance noticed. Clinical features are similar to those reported in other series.

Isotopic exploration of hepatic hydrothorax: ten cases.

OBJECTIVE: The aim of this retrospective study was to evaluate the performance of peritoneal scintigraphy for the diagnosis of peritoneopleural communication in patients with cirrhosis and to discuss its role in therapeutic management. PATIENTS AND METHODS: Ten patients with cirrhosis and pleural effusion were included in this study. Cirrhosis was due to viral hepatitis in eight patients, auto-immune disease in one patient and of unknown origin in one. The pleural effusion was right-sided in nine patients and bilateral in one. 99m-technetium sulfur colloid peritoneal scintigraphy was performed in all patients. RESULTS: Scintigraphy revealed peritoneopleural communication in nine patients. In four patients, radioactivity appeared in the pleural cavity within a few minutes after injection of the radiotracer. In three of them, a large diaphragmatic defect was demonstrated by ultrasonography, magnetic resonance imaging or thoracoscopy. Complete response to medical treatment was observed in four patients. Scintigraphy revealed rapid radioactivity migration in four patients; diuretic treatment led to resolution of the hydrothorax in one of them. Three patients whose hydrothorax was refractory to medical treatment were treated by pleurodesis with talc. Resolution of the hydrothorax was achieved in one of them. CONCLUSION: Peritoneal scintigraphy is a simple non-invasive method enabling confirmation of peritoneopleural communication in cirrhotic patients. The importance of the diaphragmatic defect can also be evaluated, providing a significant contribution to therapeutic decision-making.

Large diaphragmatic defect as the cause of hydrothorax in a cirrhotic patient: demonstration with peritoneal scintigraphy and magnetic resonance imaging.

A 52-year-old man with history of post-hepatitic cirrhosis presented with ascitis and respiratory distress. Chest X-ray on admission showed a large right hydrothorax. Thoracentesis yielded a large volume of a clear transudate fluid. Peritoneal scintigraphy showed rapid migration of radiotracer into the right pleural cavity, confirming the abdominal origin of the pleural fluid and suspecting a large diaphragmatic defect. MR imaging study using ultrafast sequences confirmed the large diaphragmatic defect.