tacrolimus and Still-s-Disease--Adult-Onset

Patients on immunosuppression are at risk of unusual infections. We present a man diagnosed to have adult-onset Still's disease who, on high-dose oral steroid and tacrolimus, developed a cavitating pneumonia due to co-infection with Aspergillus flavus and Nocardia. Timely diagnosis and institution of appropriate therapy resulted in a favourable clinical outcome. Such co-infection in a patient receiving tacrolimus is rare in the published literature. This case serves to emphasise the need to be vigilant for unusual infections in patients who are immunosuppressed, either due to drugs or underlying disease condition.

Topics: Adult; Anti-Bacterial Agents; Antifungal Agents; Aspergillosis; Coinfection; Drug Therapy, Combination; Follow-Up Studies; Humans; Immunocompromised Host; Immunosuppressive Agents; Male; Nocardia Infections; Pneumonia; Radiography, Thoracic; Steroids; Still's Disease, Adult-Onset; Tacrolimus; Tomography, X-Ray Computed; Treatment Outcome

Adult-onset Still's disease (AOSD) sometimes demonstrates hematologic disorder, whereas acquired amegakaryocytic thrombocytopenia (AAT) involvement is extremely rare. We herein report a 67-year-old woman with relapse of AOSD who concomitantly developed AAT. Thrombocytopenia along with high disease activity of AOSD was resistant to high-dose prednisolone, even in combination with methotrexate and tacrolimus. However, alternative treatment with cyclosporine after administering tocilizumab resulted in the improvement of thrombocytopenia, ultimately demonstrating that combination therapy based on suppressing the intractable disease activity of AOSD and subsequently adding a reliable immunosuppressant was required to achieve remission.

Topics: Aged; Antibodies, Monoclonal, Humanized; Bone Marrow Diseases; Combined Modality Therapy; Cyclosporine; Female; Humans; Immunosuppressive Agents; Methotrexate; Prednisolone; Purpura, Thrombocytopenic; Still's Disease, Adult-Onset; Tacrolimus

Six patients with refractory adult-onset Still's disease (AOSD) were treated with tacrolimus (TAC). Patient 1 was pregnant, for whom high-dose corticosteroid (CS) monotherapy did not achieve clinical remission, whereas TAC concomitant with CS was successful, and her baby had no apparent abnormalities. Patient 2 had hemophagocytic syndrome (HPS), for whom high-dose CS monotherapy did not achieve clinical remission, whereas TAC improved HPS, and a complete clinical remission was achieved with concomitant administration of TAC and methotrexate (MTX) with CS. Cases 3-5 could not have reduced CS doses due to repeated recurrences and other disease-modifying antirheumatic drugs, including MTX, Cyclosporine A, and tumor necrosis factor alpha inhibitors, did not control disease activity. TAC administration allowed for reduced CS doses. Case 6 experienced adverse effects, and TAC was discontinued due to elevated serum creatinine and potassium levels. TAC was useful for five of six patients, which suggests it as an option for refractory AOSD.

Topics: Adrenal Cortex Hormones; Adult; Antirheumatic Agents; Cyclosporine; Drug Therapy, Combination; Female; Humans; Male; Methotrexate; Middle Aged; Pregnancy; Pregnancy Complications; Still's Disease, Adult-Onset; Tacrolimus

Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. The mainstays of treatment are glucocorticoids (GCs) and non-steroidal anti-inflammatory drugs, although most cases are refractory to these conventional therapies. Immunosuppressants,such as methotrexate (MTX), cyclosporine A, tumor necrosis factor-α blockers, an interleukin (IL)-1 blocker, and an IL-6, receptor blocker, have been suggested in previous reports for the treatment of steroid-resistant AOSD. We report herein the case of an AOSD patient who was successfully treated with tacrolimus, another immunosuppressant, in combination with GC and MTX. Blood concentrations of tacrolimus were monitored because of the narrow therapeutic window.

Topics: Adult; Aged, 80 and over; Drug Monitoring; Drug Therapy, Combination; Female; Glucocorticoids; Humans; Immunosuppressive Agents; Methotrexate; Still's Disease, Adult-Onset; Tacrolimus

Topics: Cyclosporine; Drug Resistance; Drug Substitution; Drug Therapy, Combination; Female; Humans; Immunosuppressive Agents; Middle Aged; Prednisolone; Still's Disease, Adult-Onset; Tacrolimus; Time Factors; Treatment Outcome

We report on a 64-year-old woman with multirefractory flare of adult-onset Still's disease successfully treated with six-month course of add-on anti-interleukin 6 receptor antibody, tocilizumab. Before administration of tocilizumab, the combination therapy with 80 mg/day of prednisolone and cyclosporine or tacrolimus for five weeks, two courses of pulse methylprednisolone, and high-dose intravenous immunoglobulin could not control the disease. Add-on tocilizumab dramatically improved her disease state and enabled tapering of corticosteroid and tacrolimus. Furthermore remission has been maintained on low-dose corticosteroid and tacrolimus after withdrawal of tocilizumab. This case report suggests that short-term add-on tocilizumab might be a useful therapeutic option for patients with multirefractory flare of polycyclic systemic adult-onset Still's disease.

Topics: Antibodies, Monoclonal, Humanized; Cyclosporine; Dose-Response Relationship, Drug; Drug Resistance, Multiple; Drug Therapy, Combination; Female; Humans; Immunoglobulins, Intravenous; Immunologic Factors; Methylprednisolone; Middle Aged; Prednisolone; Pulse Therapy, Drug; Recurrence; Remission Induction; Still's Disease, Adult-Onset; Tacrolimus

Topics: Adolescent; Calcineurin Inhibitors; Female; Humans; Immunosuppressive Agents; Pain, Intractable; Still's Disease, Adult-Onset; Tacrolimus

Adult Still's disease (ASD) is a systemic rheumatic disease characterized by high spiking fever, erythema, polyarthritis, and increased levels of C-reactive protein, ferritin, and interleukin (IL)-18. Recently, biological agents targeting proinflammatory cytokines such as tumor necrosis factor (TNF) alpha, IL-1, and IL-6 have been described as effective treatments for refractory ASD. Herein, we present a patient with ASD, who was successfully treated by tacrolimus concomitant with corticosteroid, while infliximab and etanercept were not effective. Tacrolimus may be one of the drugs for the ASD patients refractory to the conventional treatments including TNF inhibitors.

Topics: Adolescent; Adrenal Cortex Hormones; Antirheumatic Agents; C-Reactive Protein; Drug Therapy, Combination; Humans; Immunosuppressive Agents; Male; Still's Disease, Adult-Onset; Tacrolimus; Treatment Outcome; Tumor Necrosis Factor-alpha