[Autoimmune and inflammatory pathologies associated with systemic scleroderma: Clinical, serological and prognostic profiles. Bi-centric retrospective series in the PACA region]. / Pathologies auto-immunes et inflammatoires associées à la sclérodermie systémique : profils cliniques, sérologiques et pronostiques. Série rétrospective bi-centrique en région PACA.

INTRODUCTION: Systemic sclerosis (SSc) is a rare auto-immune disease, affecting principally women between 40 and 60 years old. It is caracterised by a cutaneous and visceral fibrosis, an alteration of the microvascular network and the presence of autoantibodies. SSc can be associated with another connectivite tissue disease or to other autoimmune diseases, thus defining the overlap syndrome. The goal of our study is to describe these overlap syndromes. METHODS: We have analysed the data of a retrospective and bicentrique cohort, from the internal medicine unit of Hôpital Nord in Marseille and from the internal medicine unit of the Hôpital Sainte-Anne in Toulon, of patients followed for a SSc between January 1st, 2019 and December 1st, 2021. We have collected clinical, imunological features, associated auto-immune and inflammatory diseases with its morbidity and mortality. RESULTS: The cohort included 151 patients including 134 limited cutaneous SSc. Fifty-two (34.4%) patients presented at least one associated auto-immune or inflammatory disease. The association of two connectivite tissue diseases including SSc was found in 24 patients (15.9%), a third with Sjögren's syndrome and a third with autoimmune myositis. The principal associated disease to SSc was the autoimmune thyroiditis found in 17 patients (11.3%). The occurrence of complications (hospitalization, long-term oxygene therapy, death) was not significantly different depending on the existence or not of an overlap syndrom. CONCLUSION: SSc is often associated with other autoimmune diseases. This interrelation between associated pathologies and SSc, modifying sometimes the evolution of SSc, enhances the need of a personalized follow-up.

[Oedematous polyarthritis and myopericarditis as presenting features of periarteritis nodosa]. / Polyarthrite oedémateuse et myopéricardite révélatrices d'une périartérite noueuse.

Remitting symmetrical seronegative synovitis with pitting edema (RS3PE) syndrome is a rare type of seronegative polyarthritis occurring in the elderly. It can be associated to various diseases. We report a case of RS3PE syndrome associated with myopericarditis, leading to the diagnosis of polyarteritis nodosa in a 71-year-old patient admitted to the hospital for a febrile acute polyarthritis with pitting edema of the hands associated with a marked inflammatory syndrome. On second day of hospitalization, a sustained chest pain led to the diagnosis of myopericarditis. Muscular biopsy showed necrotizing vasculitis, characteristic of polyarteritis nodosa. The coexistence of RS3PE and myopericarditis has never been described in the literature. Its association with polyarteritis nodosa is also very rare and only one case has been previously reported.

[Non-diseases: another field for the specialist of internal medicine]. / Les non-maladies: un autre domaine de linterniste.

PURPOSE: Non-diseases are a heterogeneous group of symptoms, preoccupations or phenomenon that are felt or interpreted as pathological and so justifiable of medical intervention. Most doctors easily recognize illnesses that require no medical act. However, as a result of a medical misinterpretation or wrong reasoning, physicians may diagnose a non-disease and prescribe a non adapted treatment. KEY POINTS: This non exhaustive literature review, gives examples of anatomical, clinical, investigational, iatrogenic, psychiatric and collective non-diseases. Health education of the population, initial and continuing education of general and specialist practitioners and continuous assessment of advertising by the pharmaceutical industry are probably useful to limit the provision of medical care of non-pathological problems, which excessively request the physicians. CONCLUSION: The specialist of internal medicine, because of a wide knowledge of the medical specialities, has to recognize and learn the frequent traps of non-diseases.

[Multi-organ failures during septic shock from Escherichia coli urinary tract infection: catastrophic antiphospholipid syndrome?]. / Défaillance multiviscérale au cours d'un choc septique d'origine urinaire à Escherichia coli: syndrome catastrophique des anticorps antiphospholipides?

INTRODUCTION: Although the presence of antiphospholipid antibodies is not an uncommon fact during infection, their responsibility in serious manifestations can still be debated and questions the existence of an actual catastrophic antiphospholipid syndrome or multivisceral faintings, triggered by the infection only, since the presence of antiphospholipid antibodies is not pathogenous. CASE: A 68-year-old man presented during an Escherichia coli urinary tract infection a septic shock with renal and cardiac insufficiencies, hepatic cytolysis and cholestasis and disseminated intravascular coagulation. There was a significant titer of antiphospholipid antibodies IgG (50 UGPL/ml) with an antibêta2-glycoprotein-1 positivity. The patient quickly recovered with antibiotherapy and intravenous immunoglobulins. DISCUSSION: Diagnosing the pathogeny of multivisceral faintings is founded of the clinical manifestations published during the catastrophic antiphospholipid syndrome, the evolution and the persistence of post recovery antibodies and the comparison with the visceral bouts that the sepsis exclusively as revealed.

[Diabetes insipidus revealing chronic myelomonocytic leukemia]. / Diabète insipide central inaugural d'une leucémie myélomonocytaire chronique.

INTRODUCTION: Central diabetes insipidus is most frequently reported to occur after a trauma from surgery or accident. However, between 30 and 50% of cases are considered idiopathic. It's a rare complication of myelodysplastic syndrome. CASE REPORT: A 61-year-old patient presented central diabetes insipidus revealing, 17 months before, chronic myelomonocytic leukemia. Cytogenetics studies revealed monosomy 7. Acute myeloid leukemia appears 3 months after training rapid patient's death. DISCUSSION: Blood examination is necessary before to conclude idiopathic central diabetes insipidus. The discovery of chronic myelomonocytic leukemia implicates a rapid managing before its possible acute myeloid leukemia transformation. Indeed, prognosis of central diabetes insipidus and acute myeloid leukemia associated, in presence of monosomy 7, is very poor.

[Pulmonary eosinophilia in France: possible toxocariasis]. / Poumon éosinophile en métropole, ne pas oublier la toxocarose.

INTRODUCTION: In France, except in the overseas departments and territories, pulmonary eosinophilia rarely has a parasitic cause except among subjects who have traveled to tropic areas. CASE: A 19-year-old man was hospitalized for incidentally discovered hypereosinophilia. The thoracic CT scan showed several hyperdense nodules that suggested bilateral interstitial alveolar lesions. Diagnosis was finally based on serologic results positive for toxocariasis. A 14-day course of albendazole led to regression of the eosinophilia and of the radiographic images. DISCUSSION: The frequency of toxocariasis or visceral larva migrans syndrome is probably underestimated. In adults, the symptoms can be atypical and possibly serious. Albendazole, for 14 days, is the reference treatment.

[Current treatment of rheumatoid arthritis]. / Traitement actuel de la polyarthrite rhumatoïde.

Over the past 10 years, the management of rheumatoid arthritis has been revolutionized. Early diagnosis is essential and should allow an early initiation of disease modifying anti-rheumatic drugs (DMARD), if possible within the first 3 three months after disease onset, aiming at disease remission and the best long-term prognosis. Recommendations for the prescription of synthetic and biologic DMARD (mainly anti-TNFalpha agents) are available since September 2007 [6] by HAS in France. The great efficacy of these drugs has been established from many clinical trials including tens of thousands of patients. However, severe adverse side effects may occur (allergy, tuberculosis, opportunistic infections, demyelination) and rheumatologists should remain vigilant. Global care of the patient includes prescription of pharmacologic and non-pharmacologic treatments (education, physical treatment, ergotherapy, psychotherapy, surgery). A good coordination between all specialists is required. Screening and treatment of extra-articular manifestations, prevention of infections, osteoporosis and cardiovascular complications are essential to allow a better long-term prognosis, and reduce disability and mortality of rheumatoid arthritis.