We performed a second IVCY and renal function and LDH both improved

We performed a second IVCY and renal function and LDH both improved. whereas eculizumab did not show therapeutic effects. The patient further showed recurrent deterioration, and we initiated intravenous cyclophosphamide in addition to combination treatment and eventually succeeded in controlling the disease. Genome analysis by whole-exome STF-083010 sequencing revealed no particular gene mutation related to either complement disorders or type-1 interferon. Further elucidations concerning the pathogenic mechanisms causing juvenile-onset SLE are needed to establish an efficient treatment strategy for TMA with SLE. strong class=”kwd-title” Keywords: Thrombotic microangiopathy, Systemic lupus erythematosus, Kidney injury, Pediatric rheumatology, Pediatric nephrology Introduction Thrombotic microangiopathy (TMA) is usually characterized by microangiopathic hemolytic anemia, thrombocytopenia, and multiple organ injury [1, 2]. In particular, primary TMA occurs in case of complement disorderssuch as atypical hemolytic uremic syndrome, as well as in ADAMTS13 deficiency (also known as thrombotic thrombocytopenic purpura)whereas secondary TMA is brought on by various factors, such as drugs, bone marrow transplantations, and autoimmune diseases [2, 3]. TMA manifestations generally affect many organs, with the kidney being one of the main involved organs, both in primary and secondary TMA. Systemic lupus erythematosus (SLE) is an autoimmune disorder affecting multiple organs. SLE is usually reported to be accompanied by secondary TMA in 0.5C10.0% of the cases [4]; moreover, SLE with TMA has been described as a life-threatening condition [5, 6]. Nonetheless, TMA in pediatric SLE patients is usually a rather rare occurrence. Here, we report a case of a pediatric SLE patient presenting with a severe form of secondary TMA, successfully treated using a combination therapy of methylprednisolone pulse therapy, oral steroid, plasma exchange, mycophenolate mofetil, STF-083010 and intravenous cyclophosphamide. Case report Before being referred to our hospital, a 5-year-old lady presented to a former hospital complaining of fever and edema. Recent medical history reported periorbital edema and low fever 1?month prior, as well as diarrhea 1?week prior. Furthermore, she had a history of asthma which was treated with montelukast a few years prior, as reported during anamnesis; however, she was not on medication at the time of presentation. In addition, her paternal grandfather was diagnosed with SLE. When she frequented a former hospital, she had high fever and edema in the lower extremities, and her systolic blood pressure was 146?mmHg. Urinalysis revealed proteinuria and occult blood, whereas blood cell counts revealed anemia, thrombocytopenia, and schistocytosis. She was suspected of having developed TMA and subsequently admitted to our STF-083010 hospital. On admission, her body weight was 19.5?kg, which had increased 1.0?kg in the previous month, and her height was 104?cm. Her body temperature was 38.3?C, blood pressure was 128/98?mmHg with a heart rate of 100 beats per min, respiratory rate was 35 breaths per min, and SpO2 was 99% without oxygen supply. She showed significant edema around the eyes and lower extremities. Although her respiratory sounds were decreased, there were no abnormal heart sounds. Her belly was distended albeit non-tender, and she had no skin rash, joint swelling, or joint pain. She had no abnormalities related to the central nervous system. Although her general conditions were poor, she was fully conscious. Blood cell counts showed that peripheral leucocytes were 8200/L, with 2591?lymphocytes/L. The patients hematocrit was 19.9%, while STF-083010 red blood cell count was Rabbit Polyclonal to CCKAR 2,140,000/L, hemoglobin was 6.1?g/dL, and platelet count was 39,000/L. Laboratory serological findings were as follows: lactate dehydrogenase (LDH) 735?IU/L, total protein 4.3?g/dL, albumin 1.9?g/dL, creatinine 0.89?mg/dL, urea nitrogen 8.8?mg/dL, potassium 6.0?mEq/L, and brain natriuretic peptide 222.3?pg/mL. In addition, haptoglobin was below 10?mg/dL. Coagulation assessments reported that fibrin and fibrinogen degradation products were 321?mg/dL and that D-dimer was 3.65?g/mL. The serum IgG level was 420?mg/dL, and C3 and C4 were 36 and 5?mg/dL, respectively. CH50 was 13?U/mL, and anti-dsDNA IgG antibody level was 96?IU/mL; in addition, antinuclear antibody titer was 1:160, whereas SS-A antibody titer was 1:4. Other autoimmune antibodies, such as anti-Sm, anti-RNP, PR3-ANCA, and MPO-ANCA, were all unfavorable. Urinalysis showed proteinuria of 21.2?g/day with hematuria and increasing levels of 2-MG to 1418?g/L (Table ?(Table1).1). A chest X-ray showed pleural effusions and cardiomegaly (i.e., cardiothoracic ratio?=?57%), while cardiograms were normal. Ultimately, there were no abnormal findings related to either stool.