The Second Stage
From May 8, her appetite and general state worsened again with frequent nausea and fatigue, but still with no fever, cough, expectoration, or hemoptysis. Most laboratory indices were improving except for plasma complement levels (C3: 53.6 mg/dl and C4: 1.66 mg/dl). The SCr concentration decreased to 241.6 μmol/L, ESR was 16 mm/h, and the CRP concentration was normal. Owing to the development of leukopenia, the discordance between the patient’s subjective symptoms and objective indicators was initially interpreted as a possible adverse reaction to the CTX, and CTX was discontinued. Nevertheless, her condition continued to worsen. On May 12, she developed dark urine again and a fever, with a temperature as high as 38.5°C. The moist rales in both lung bases worsened significantly, with increased patchy shadows on chest X-rays (Fig. 2). SCr continued to decrease (226.9 μmol/L), which was also confusing, and there was minimal evidence of infectious complications, with normal CRP and procalcitonin concentrations. Intravenous immunoglobulin (IVIG) was prescribed (20 g per day, for 5 days). However, there was no clinical response, and her fever increased to 39°C; antibiotics were re-prescribed (cefoperazone-sulbactam 0.5 g every 12 hours for 3 days, followed by meropenem 0.25 g every 12 hours). In the absence of immediate improvement, ANCA was reanalyzed. The MPO antibody concentration had increased to 136.9 units/ml, and the methylprednisolone dosage was increased to 80 mg per day again. Unfortunately, the patient showed no improvement, with a continuous fever, decreased urine volume (approximately 600 ml per day), and exacerbated renal function (SCr: 408.7 μmol/L on May 17). Peripheral blood (1-3)-β-D-glucan, cytomegalovirus DNA, and Pneumocystis carinii antigen and antibody were all negative. Dialysis was initiated with high-dose intravenous methylprednisolone pulse therapy (320 mg per day for 3 days, followed by 40 mg per day). Five days later (May 22), her temperature returned to normal with decreased MPO antibody concentration (86.3 units/ml) and improved urine color. Although the plasma C4 concentration improved (3.11 mg/dl), C3 concentrations decreased further (36.3 mg/dl), and her urine volume showed no significant increase. All of these findings implied on-going disease activity. According to the leukopenia and for economic reasons, cyclosporine A (CsA) was added to the glucocorticoids, with a plasma concentration of 80–90 ng/ml. The patient’s alimentary symptoms and fatigue decreased in response to this therapy.