Cardiopulmonary involvement in adult-onset Still's disease is not as common as cutaneous and articular involvement. Pleuropericarditis is the most frequent thoracic manifestation. Although difficult, diagnosis of other thoracic manifestations, which may reveal the disease, is crucial, due to the high risk to life and the efficacy of new immunosuppressive agents. The pathophysiology involves essentially immunological factors, Still's disease being increasingly seen as an autoimmune inflammatory disease. Pro-inflammatory cytokines such as interleukine (IL) 1, 6 and 18 play a crucial role in macrophage activation, which is central in the pathophysiology of adult Still's disease. The classification of cardiopulmonary manifestations is based on anatomy. Cardiac lesions may involve all the tissues of the heart and the pulmonary arteries. Respiratory lesions may involve the pleura, the lung parenchyma (organizing pneumonitis, infiltrative lung disease, alveolar damage, amyloidosis), and the respiratory muscles, including the diaphragm. Finally, some manifestations may be provoked by the treatment itself. Steroids, the first-line treatment, are very effective in pleuropericarditis. Methotrexate used to be prescribed when steroids failed, but biotherapies such as IL1 and IL6 inhibitors have transformed the prognosis of forms resistant to these drugs.

Tocilizumab is a highly effective therapeutic agent for the treatment of rheumatoid arthritis and systemic juvenile idiopathic arthritis. Furthermore, a large amount of case study data reveals that tocilizumab can be an effective therapy for not only rheumatoid arthritis but also for other mostly rare inflammatory rheumatic diseases. By blocking the interleukin-6 pathway tocilizumab can be a useful therapeutic alternative when conventional treatment fails. It is successful in treating diseases such as the adult-onset Still's disease, amyloidosis, giant cell arteritis, multiple myeloma, polymyalgia rheumatica, relapsing polychondritis, remitting seronegative symmetrical synovitis with pitting edema-syndrome, systemic lupus erythematosus, systemic sclerosis, and Takayasu arteritis. Studies underway are now recruiting patients to acquire further data on treating patients with non-rheumatic arthritis, inflammatory diseases. This review focuses on tocilizumab as a promising agent for treating rare and orphan diseases in rheumatology for which no satisfactory treatment is yet available.

Abstract is missing (Letter).

Little is known about the interactions between adult-onset Still's disease (AOSD) and pregnancy. In an attempt to clarify the link between these 2 conditions, we retrospectively analyzed patients registered as suffering from AOSD seen in our university hospital. A total of 57 patients, among them 30 women, were diagnosed. Ten pregnancies in 8 women were identified. Three cases manifested AOSD in their first trimester, all treated with prednisone. Premature births and flares occurred in 2 patients. One patient developed a monocyclic AOSD during her second pregnancy's postpartum. In the 4 other cases, AOSD was known and quiescent before pregnancy. One patient had 2 pregnancies without any flare or complication. One patient experienced her first pregnancy while under treatment and presented a late flare 8 months after delivery. The third patient developed exacerbation in the first trimester of her second pregnancy which was treated with IgIV alone. The last one presented her first pregnancy 7 years after diagnosis. A prednisone-treated systemic flare occurred during the first trimester without later complication. Based on our own experience and the analysis of only two series of the literature, including, respectively, 4 and 5 patients, we suggest that two settings could be distinguished. First, AOSD can occur during pregnancy and can be responsible for obstetrical complications. Then, in patients with known AOSD, the second trimester and postpartum appear to be periods exposing to disease recurrence. Thus, we recommend a close multidisciplinary monitoring by a rheumatologist and an obstetrician prior to, during and after pregnancies.

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A traditional infectious disease vaccine is a preparation of live attenuated, inactivated or killed pathogen that stimulates immunity. Vaccine immunologic adjuvants are compounds incorporated into vaccines to enhance immunogenicity. Adjuvants have recently been implicated in the new syndrome named ASIA autoimmune/inflammatory syndrome induced by adjuvants. The objective describes the frequencies of post-vaccination clinical syndrome induced by adjuvants. We performed a cross-sectional study; adverse event following immunization was defined as any untoward medical occurrence that follows immunization 54 days prior to the event. Data on vaccinations and other risk factors were obtained from daily epidemiologic surveillance. Descriptive statistics were done using means and standard deviation, and odds ratio adjusted for potential confounding variables was calculated with SPSS 17 software. Forty-three out of 120 patients with moderate or severe manifestations following immunization were hospitalized from 2008 to 2011. All patients fulfilled at least 2 major and 1 minor criteria suggested by Shoenfeld and Agmon-Levin for ASIA diagnosis. The most frequent clinical findings were pyrexia 68 %, arthralgias 47 %, cutaneous disorders 33 %, muscle weakness 16 % and myalgias 14 %. Three patients had diagnosis of Guillain-Barre syndrome, one patient had Adult-Still's disease 3 days after vaccination. A total of 76 % of the events occurred in the first 3 days post-vaccination. Two patients with previous autoimmune disease showed severe adverse reactions with the reactivation of their illness. Minor local reactions were present in 49 % of patients. Vaccines containing adjuvants may be associated with an increased risk of autoimmune/inflammatory adverse events following immunization.

Atypical haemolytic uraemic syndrome is a rare disease associated which genetic or acquired factors those cause defective regulation of the alternative complement pathway. We report the case of a 46-year-old woman who presented with thrombotic microangiopathy coinciding with a monocyclic evolution of adult-onset Still's disease. Low C3 with decreased FB concentration, associated with normal C4 was present until the thrombotic microangiopathy's resolution, indicative of an excessive production of alternative C3 convertase. She responded to plasma exchange. This observation reinforces the hypothesis for a common pathway in the pathogenesis for both of the diseases, and suggests alternative complement pathway mediation.

Tissue granulomas formation in adult-onset Still's disease (AOSD) is extremely rare. We describe a case of AOSD associated with formation of granulomatous lesions in lymph nodes, liver and presumably spleen. The high dose steroid-dependent nature of our patient's illness, characterized by disease relapses when methylprednisolone dose was reduced below 10 mg/d, was overwhelmed with institution of anakinra (100 mg/d). The histologic finding of granulomas formation in lymph nodes, liver or spleen should not deter the consideration of AOSD as a potential diagnosis in a compatible clinical context; however, other more common etiologies of tissue granulomas formation should be first excluded.