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Fulminant macrophage activation syndrome and no improvement: case report A 67-year-old man developed fulminant macrophage activation syndrome (MAS) during immunosuppressive treatment with azathioprine and methylprednisolone for anti-synthetase syndrome. Later, he exhibited no improvement during treatment with ganciclovir for MAS. The man was previously diagnosed with anti-synthetase syndrome based on the presence of Raynaud’s phenomenon, interstitial lung disease, myositis and anti-Jo-1 and anti-Ro52 autoantibodies. He had been treated with methylprednisolone (glucocorticoid therapy) 32 mg/day, which was tapered to zero, along with azathioprine 100 mg/day, under which he remained stable for 2 years. Then, at the age of 67 years, he experienced a relapse in the disease activity (elevated creatine kinase levels and weight loss). Therefore, the dose of azathioprine was increased to 150 mg/day, and he started receiving methylprednisolone 4mg. However, he experienced worsening of his condition. The man was hospitalised with increased dyspnoea, fever, general malaise and muscle weakness. Physical examination revealed tachycardia and fever. Pancytopenia, hypertriglyceridaemia, extremely high ferritin level, high transaminase levels, low fibrinogen, elevated creatine kinase and elevated lactate dehydrogenase were noted on laboratory investigations. A CT scan of the lungs demonstrated deterioration of the existing interstitial lung disease (fibrotic nonspecific interstitial pneumonia) along with a discrete pericardial and pleural effusion. Shortly after hospitalisation, he developed haemodynamic instability, and he was therefore moved to the ICU. He had 2014 HScore of 241, due to which he started receiving methylprednisolone. Based on the presence of CD68-positive histiocytes on a bone marrow biopsy, the diagnosis of MAS was confirmed. A PET CT scan showed splenomegaly and active myositis. PCR assay for CMV was found to be positive, both in the serum and bronchoalveolar lavage fluid. Therefore, he started receiving IV ganciclovir 350mg twice a day. After 4 days, the therapy was changed to dexamethasone and ciclosporin since there was no improvement in the laboratory findings and clinical status with ganciclovir. He had persistently elevated ferritin levels. An initial 3-day decline was noted, after which the serum ferritin levels increased again. Therefore, he started receiving etoposide (cytostatic drug therapy) along with immune-globulin [IVIG]. Subsequently, an immediate clinical improvement was observed. Also, the ferritin levels and other laboratory abnormalities normalised gradually. During the period following the completion of treatment with etoposide, he developed pneumonia, catheter-related bacteraemia, disseminated fusariosis with fungaemia and pulmonary infection [aetiologies unknown], which was evident on a chest CT. He was treated with appropriate unspecified antibacterials and voriconazole (antifungal drug). Three weeks later, he died due to a massive intracranial bleed following an accidental fall. At t