[Interstitial lung diseases from autoimmune origins: A prognostic and diagnostic challenge for the clinician]. / Pneumopathies interstitielles diffuses aiguës d'origine auto-immune : un défi diagnostique et pronostique pour le clinicien.

Acute or subacute interstitial lung diseases from autoimmune origins are especially hard to diagnose but have to be detected promptly. We illustrate this necessity with three case reports. One case of paraneoplasic polymyositis, one case of interstitial lung disease caused by a connectivite and one case of interstitial lung disease related to an anti-synthetase syndrome. The subject is to alert the practitioners to the early search of extra pulmonary signs, autoantibodies analysis in the objective to set up quickly the right treatment.

[Thymoma and autoimmune diseases]. / Thymomes et maladies auto-immunes.

The association between thymoma and autoimmunity is well known. Besides myasthenia gravis, which is found in 15 to 20% of patients with thymoma, other autoimmune diseases have been reported: erythroblastopenia, systemic lupus erythematosus, inflammatory myopathies, thyroid disorders, Isaac's syndrome or Good's syndrome. More anecdotally, Morvan's syndrome, limbic encephalitis, other autoimmune cytopenias, autoimmune hepatitis, and bullous skin diseases (pemphigus, lichen) have been reported. Autoimmune diseases occur most often before thymectomy, but they can be discovered at the time of surgery or later. Two situations require the systematic investigation of a thymoma: the occurrence of myasthenia gravis or autoimmune erythroblastopenia. Nevertheless, the late onset of systemic lupus erythematosus or the association of several autoimmune manifestations should lead to look for a thymoma. Neither the characteristics of the patients nor the pathological data can predict the occurrence of an autoimmune disease after thymectomy. Thus, thymectomy usefulness in the course of the autoimmune disease, except myasthenia gravis, has not been demonstrated. This seems to indicate the preponderant role of self-reactive T lymphocytes distributed in the peripheral immune system prior to surgery. Given the high infectious morbidity in patients with thymoma, immunoglobulin replacement therapy should be considered in patients with hypogammaglobulinemia who receive immunosuppressive therapy, even in the absence of prior infection.

The hand in systemic diseases other than rheumatoid arthritis.

This review outlines the skin, vascular and musculoskeletal symptoms affecting the hand during systemic inflammatory diseases other than rheumatoid arthritis. Skin lesions are diagnosed clinically and their symptomatology is documented through an extensive series of photographs. These conditions may require specific care before a surgical procedure can be performed. Vascular lesions are also diagnosed clinically and their symptomatology is described in detail. It is important to recognize that acrocyanosis is always benign. The surgeon should be able to distinguish between primary, but benign Raynaud's disease and secondary Raynaud's syndrome, which has a high risk of finger necrosis. Current preventative and curative treatments for finger necrosis are described. The clinical, radiological, progressive and therapeutic features of musculoskeletal lesions are reviewed, namely those associated with psoriatic arthritis, systemic sclerosis and lupus.

[Therapeutic strategy in inflammatory myopathies (polymyositis, dermatomyositis, overlap myositis, and immune-mediated necrotizing myopathy)]. / Prise en charge thérapeutique des polymyosites, dermatomyosites, myosites de chevauchement et myopathies nécrosantes auto-immunes.

Inflammatory myopathies (IM) are a heterogeneous group of autoimmune muscle disorders of unknown origin that share clinical symptoms such as muscle weakness and histological features with the presence in muscle of inflammatory infiltrate. Based on clinical, histological and serological characteristics, IM can be divided into polymyositis, dermatomyositis, overlap myositis, cancer-associated myositis, immune-mediated necrotizing myopathy, and inclusion-body myositis. Because of their resistance to corticosteroids and immunosuppressive drugs, inclusion-body myositis will be treated separately in this issue. Major obstacles in conducting high quality randomized controlled trials in inflammatory myopathies include the low prevalence and the heterogeneity of these diseases as well as the lack of international consensus on the outcome measures. In the absence of adequate controlled therapeutic trials, treatment of these disorders remains largely empirical. Corticosteroids are the cornerstone therapy. Due to the chronic course of the disease, there is a frequent need to use additional immunosuppressive treatment both to improve the disease response and to reduce the side effects of corticosteroids. Intravenous immunoglobulin infusion is a costly treatment option that is reserved in the presence of refractory dermatomyositis based on a trial showing superior efficacy against control in patients with impaired swallowing or with contraindications to immunosuppressive drugs. In patients who fail second-line therapy, which usually consists of methotrexate plus corticosteroids, the diagnosis should be carefully reassessed before considering other treatment options including methotrexate plus azathioprine or biological agents such as rituximab.