Liposteroid and methylprednisolone combination therapy for a case of idiopathic lung hemosiderosis

Abstract

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease in children, with unknown etiology. The classical clinical triad is hemoptysis, hypochromic anemia and diffuse parenchymal infiltrations on chest X-ray. Liposteroid dexamethasone palmitate, which was developed in Japan, has shown good efficacy for IPH. We present the case of a patient with IPH, who suffered from a life-threatening respiratory dysfunction, and was rescued by a trial administration of liposteroid with methylprednisolone (mPSL).A 6-year-old girl was admitted to our hospital for repeated dyspnea and blood-stained sputum. She was diagnosed with IPH at the age of three-months by iron staining of gastric fluid and sputum studies. Her cumulative dose of steroids (equivalent to prednisolone (PSL)) was 1062 mg/kg. However, she could not achieve remission. We decided to initiate liposteroid therapy. We administered an infusion of liposteroid 0.8 mg/kg intravenously, for three consecutive days as a therapy for acute bleeding. After administration of liposteroid, she developed high fever with CRP elevation. We suspected that the inflammation was caused by palmitate, which is present as a lipo base in liposteroid. Hence, we added 2 mg/kg mPSL per day for 1 week. As a maintenance treatment, a single infusion of liposteroid was administered followed by mPSL administration for 6 days in every week. Her respiratory condition slowly improved. Tracheostomy was performed for airway management. She was shifted out of the ICU on the 34th day.Steroid is a key therapy for hemosiderosis. When IPH is diagnosed, oral prednisone therapy is initiated. Although this is effective, there are limitations due to significant adverse effects. Maintaining drug therapy is very important for IPH patients to keep the disease under control. Liposteroid has the same mechanism of action as dexamethasone. It has a Lipo-base, palmitate, which could induce pro-inflammatory cytokine activation. We used mPSL to inhibit the inflammation following liposteroid administration. This was effective. A combination of liposteroid and mPSL administration was useful method of treatment for the patient.

Successful Liposteroid Therapy for a Recurrent Idiopathic Pulmonary Hemosiderosis with Down Syndrome

“…Liposteroid has the same mechanism of action as dexamethasone, however, greater efficacy and lower frequency of systemic adverse effects than dexamethasone. In addition, liposteroid has lipo-based, palmitate, which is more harmful to non-adipose tissue, is easily taken up by macrophages, and induces strongly apoptosis of macrophages [24,25]. Doi et al suggested that low-dose liposteroid therapy accumulates effectively in the hemorrhagic inflamed sites of the lung, reduces the chance of high-dose corticosteroid therapy and prevents adverse effects [8].…”

Short Review of Liposteroid: A Novel Targeted Glucocorticoid Preparation for Treatment of Autoimmune and Inflammatory Diseases

“…Liposteroid has been used successfully in patients with IPH refractory to high dose conventional CS therapy (Sakurai et al, 1999;Doi et al, 2013;Sakamoto et al,…”

“…The incidence of acute toxicity is very low. Sakamoto et al (2018) reported the development of restlessness, irritability, hypertension, and altered mental status secondary to the development of posterior reversible encephalopathy in a 2-year-old girl after the first dose of liposteroid.…”