Nasal oral fistula revealing Langerhans´ cell histiocytosis in adult patient: case report.

Langerhans cell histiocytosis (LCH) is a rare systemic disease caused by proliferation of mature histiocytes; its association to histiocyto fibroma is rarely reported. It rarely affects adults. We report a case of systemic LCH, in an adult patient with osteolytic lesion causing a fistula between the left nasal cavity and hard palate, involving the bone, lung, lymph node and associated to multiple histiocyto fibroma. The patient was operating for a fistula, and he was treated by chemotherapy and corticosteroids. Langerhans´ cell histiocytosis is a rare case, especially in adult patient. The diagnosis was based on histological and immunohistochemical analyses. This patient was treated by steroids and chemotherapy.

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.

Takayasu's arteritis occurring under TNF blockers in a patient with spondyloarthritis: is it an association or a paradoxical effect?

Coexistence of spondyloarthritis (SpA) and Takayasu's arteritis is not a common finding, but such cases have been discussed, particularly in the context of choice of therapy. Inhibition of inflammation by tumor necrosis factor inhibitors (TNFi) is a key aspect of the treatment of SpA and also positive effects of such treatment in concomitant large vessel vasculitis have been reported. However, TNFi is also associated with the possibility of initiating vasculitis. The present article based on a case study and the available literature is an attempt to discuss coexistence of these two diseases and the impact of treatment with biological drugs from the anti-TNF group in the course of SpA with Takayasu's arteritis.

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.