A case of an SLE patient with febrile neutropenia who experienced exacerbation of cutaneous manifestations after the administration of G-CSF.

Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease. Although hematological anomalies are commonly found in SLE, severe polymorphonuclear leukocyte depletion is rare. Most episodes of severe granulocytopenia in SLE patients tend to occur as part of drug toxicity-induced medullar hypoplasia, and recombinant human (rh) granulocyte colony-stimulating factor (G-CSF) has been shown to be effective for treating neutropenia associated with SLE. However, flares of some autoimmune diseases, including flares in six SLE patients, have been reported after G-CSF therapy. This report presents the case of a patient with SLE who experienced exacerbation of skin symptoms after G-CSF therapy. There is sufficient evidence to suggest that G-CSF can exacerbate inflammatory disease. Furthermore, the possibility that several factors other than rhG-CSF may affect the disease activity of SLE should be considered when SLE patients with neutropenia require the administration of rhG-CSF. Therefore rhG-CSF should be used with considerable caution in neutropenic patients with SLE.

Adult-onset Still's disease with macrophage activation syndrome successfully treated with a combination of methotrexate and etanercept.

We report a 16-year-old female case of intractable adult-onset Still's disease accompanied by macrophage activation syndrome, who went into full remission after switching from infliximab to etanercept. Although the disease promptly relapsed when etanercept was discontinued, she again responded fully upon the reintroduction of etanercept. Furthermore, the effect of etanercept was apparently enhanced by combining it with a sufficient dose of methotrexate. This combination therapy should be considered as one of treatment options for the disease.

Effectiveness of subcutaneous tocilizumab in refractory adult Still's disease: report of three cases and a review of the literature.

Adult Still's disease (ASD) is a systemic inflammatory disorder characterised by spiking fever, skin rash, arthritis, hepatosplenomegaly, and elevated inflammatory markers. Several proinflammatory cytokines, including interleukin (IL)-6, contribute to its pathogenesis. There have been some recent reports on the efficacy of tocilizumab (TCZ), a humanised anti-IL-6 receptor antibody, in the treatment of ASD refractory to conventional therapy. However, most of the evidence is for intravenous administration of TCZ, whereas subcutaneous injection is often preferred in terms of efficiency in cost and labour. We have experienced three patients whose ASD was refractory to corticosteroid and immunosuppressant therapy but showed a marked response to off-label use of subcutaneous TCZ (TCZ-SC). Patient 1 received TCZ-SC 162 mg on days 0 and 14 and every week thereafter. Patients 2 and 3 received TCZ-SC every 2 weeks. At the time of initiation of TCZ-SC, all three patients had elevated inflammatory markers and two had fever despite previous therapy. After the first TCZ-SC injection, the patients became afebrile within one day and inflammatory parameters (i.e. C-reactive protein and erythrocyte sedimentation rate) returned to normal within 2 weeks. None of the patients developed severe infection or other serious side effects during 104 weeks of follow-up. There have been only a limited number of case reports showing that TCZ-SC significantly improves refractory ASD during its active phase. Our experience with these patients suggests that TCZ-SC could, as well as offering cost efficiency in clinical practice, be a potent treatment option for refractory ASD.