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.