[ANCA vasculitis preceded by isolated joint manifestations. Report of two cases and literature review]. / Vascularites à ANCA précédées d'une atteinte articulaire isolée. À propos de deux cas et revue de la littérature.

INTRODUCTION: Joint manifestations of ANCA (antineutrophil cytoplasmic antibody) associated vasculitis (AAV) are extremely common in the progression of systemic damages. However, the joint involvement is rarely isolated. The diagnosis and the treatment are difficult in this particular situation and few data are available on the topic. CASE REPORT: We have observed two cases of joint manifestation revealing AAV we are going to describe here. The evolution toward a more severe disease raises the question of the treatment managing. The place of the ANCA research in relation to other immune tests is also discussed. CONCLUSION: Isolated joint manifestations of ANCA vasculitis are rare but can lead to a delay in diagnosis. ANCA vasculitis should be considered in seronegative symmetrical polyarthritis by looking for ANCA in a second line biological test. Methotrexate is the first treatment option to be considered. In case of insufficient response or failure, rituximab seems an interesting option in this context.

Effet indésirable rare d'un anti-IL-1RA.

INTRODUCTION: Anakinra is an anti-IL-1RA targeting IL-1ß with a central role in the occurrence of auto-inflammatory diseases. Its use is not without risk. CASE REPORT: We report a case of late onset auto-inflammatory syndrome treated with anti-IL-1RA whose progression was marked by deep isolated thrombocytopenia, rapidly regressive after discontinuation of anakinra. CONCLUSION: Immuno-allergic thrombocytopenia to anakinra is a rare, but serious adverse event.

[Severe alveolar haemorrhage related to a silicone embolism syndrome revealed by a chronic penis ulcer: A case report]. / Une hémorragie intra-alvéolaire sévère liée à un syndrome d'embolie siliconée révélée par une ulcération chronique du pénis : une observation.

INTRODUCTION: Silicone injections, in particular illegal injections, carried out in an aesthetic purpose, can cause serious complications, like silicone embolism syndrome. CASE REPORT: We present a 39-year-old man who presented with a severe acute respiratory distress syndrome related to an alveolar hemorrhage associated with a persistent penis ulcer and a genital lymphedema. It was the complications of silicone injections which revealed a severe personality disorder. Diagnosis of silicone embolism syndrome was made, a few years later, thanks to the histopathology study of a persistent penis ulcer with genital lymphedema. The outcome was favorable. CONCLUSION: A serious alveolar hemorrhage in a young patient should raise suspicion of silicone embolism syndrome, especially if there are cutaneous lesions compatible with injections.

Silica-associated connective tissue disease. A study of 24 cases.

We prospectively studied all patients hospitalized for connective tissue disease (CTD) in our French rheumatology clinic from January 1979 to December 1989. Our aims were 1) to determine if CTDs associated with occupational exposure to silica (Si) are currently observed in a rheumatology clinic, and, if so, 2) to describe the major features of Si-associated CTD, and 3) to specify which individuals are affected by Si-associated CTD. Patients were divided into 2 groups based on their responses to a questionnaire: those who had been exposed to Si, and those who had no occupational exposure to Si. Among the 764 patients with CTD studied, 24 (3%) were patients with Si-associated CTD and 740 (97%) were patients with non-Si-associated CTD. The sex ratio between the 2 groups was significantly different with a high frequency of men and of immigrants in the Si-associated CTD group. Two thirds of the patients exposed to Si were male miners or sandblasters, but the other third had more unusual exposures to Si, which may involve members of all socio-economics sectors and both sexes, such as sculpture or exposure to abrasive powders. Progressive systemic sclerosis (PSS) was significantly more prevalent in the Si-associated CTD group. This group also consisted of patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), dermatomyositis (DM), and other autoimmune diseases. Si-associated CTD was characterized by the frequency of radiologic lung fibrosis, impaired pulmonary function tests, secondary Sjögren syndrome, and antinuclear antibodies. The number of mineral particles and crystalline Si content were raised in all the bronchoalveolar lavage specimens of Si-exposed patients but in none of those of nonexposed patients. In some cases of Si-associated CTD, the disease was reversible after early cessation of Si exposure. Epidemiologic studies are required to confirm our hypothesis that not only PSS and RA but also SLE and DM are associated with occupational exposure to Si. Pending such results, exposure to Si should be sought in the history of any patient with CTD, especially in a male patient with pulmonary signs, and if present, exposure should be stopped. In the meantime, steps should be taken to ensure that workers exposed to Si in all environments have adequate protection.

[Occupational exposure to silicon and dermatopolymyositis. 3 cases]. / Exposition professionnelle à la silice et dermatopolymyosite. Trois observations.

After 5, 16 and 21 years of occupational exposure to silica, 2 men and 1 woman, respectively, were diagnosed as having dermatopolymyositis. Pulmonary impairment was always present and took the form of diffuse interstitial fibrosis in 2 patients, whose lung biopsies revealed high particulate silica contents. The role of silica in the induction of dermatopolymyositis is discussed in light of published information on silica-induced connective tissue diseases.

[Isolated muscular tuberculosis in dermatomyositis treated with corticosteroids. Developing outbreak of dermatomyositis under rifampicin treatment. 2 cases]. / Tuberculose musculaire isolée au cours d'une dermatomyosite traitée par corticothérapie. Poussée évolutive de la dermatomyosite sous rifampicine. Deux observations.

The authors report two cases of muscular tuberculosis in patients with dermatomyositis treated with steroids. Antituberculous therapy with Rifampicin led to a severe relapse of the dermatomyositis in both cases, suggesting enzymatic induction inhibiting the action of the steroids.