Refractory sarcoidosis-like systemic granulomatosis responding to ruxolitinib.

In 2012, a 51-year-old woman presented with a 2-year history of painful hepatomegaly, chronic asthenia and recurrent fevers. Her medical history comprised augmentation mammoplasty 6 years prior to the onset of her symptoms and she received fenofibrate as a treatment for dyslipidaemia. Physical examination found an increased liver span and splenomegaly, cervical lymphadenopathy, and peritoneal and pleural effusion outbreaks. There was neither rash nor joint pain. Plasma inflammatory markers were constantly raised with C reactive protein (CRP) levels of 80–100 mg/L, increasing when fever and effusions occurred. Subsequently, in the course of the disease, biochemical analysis found hypercalcaemia peaking at 3.81 mmol/L with hypercalciuria. The blood cells count, serum angiotensin conversion enzyme, parathormone (PTH), PTH-rp and plasma protein electrophoresis were constantly unremarkable. Fludeoxyglucose F18 positron-emission tomography (FDG-PET) showed heterogeneous hypermetabolism of the liver, spleen, entire bone frame and several mediastinal, thoracic and abdominal lymph nodes (figure 1). Repetitive pleural …