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+The 36-year-old male patient in this case had a 6-year history of diffuse cutaneous SSc (Fig.1) and had not received regular follow-up or medication therapy.
+He had experienced malaise, poor appetite, and progressive shortness of breath for 1 month and oligouria for 3 days.
+He was admitted to our hospital with renal failure and pulmonary edema.
+In the emergency department, his blood pressure, pulse rate, and respiratory rate were 174/127 mm Hg, 88/min, and 22/min, respectively.
+Laboratory analysis revealed the following values: white blood cell count, 7730/μL (3500–11,000/μL); hemoglobin, 8.6 g/dL (12–16 g/dL): platelet count, 87000/μL (150,000–400,000/μL); blood urea nitrogen, 78 mg/dL (6–21 mg/dL); creatinine, 8.9 mg/dL (1.1–1.5 mg/dL); calcium, 8.2 mg/dL (8.8–10.3 mg/dL); phosphorus, 5.4 mg/dL (2.7–4.5 mg/dL); haptoglobin, <6.56 mg/dL (30–200 mg/dL); and lactate dehydrogenase, 547 U/L (106–211 U/L).
+A routine urinalysis revealed a proteinuria score of 2+ (200 mg/dL), white blood cell count of 3–5/high power field, and red blood cell count of 25–50/high power field.
+The autoimmune profile indicated an antinuclear antibody level of 1:320 (speckles; normal, <1:40).
+Tests for anti-Scl70, anti-double-stranded DNA, anti-Ro, anti-La, and anticardiolipin antibodies were all negative.
+Kidney echogram showed a decreased bilateral kidney size without hydronephrosis.
+Despite the chronic changes visible on the echogram, the patient had developed acute pulmonary edema and oligouria only 3 days before admission.
+Accordingly, a clinical diagnosis of acute-on-chronic renal failure was made.
+A further diagnosis of SRC was supported by the presence of renal failure with microangiopathic hemolytic anemia and hypertension, and the patient was started on captopril therapy.
+The sustained deterioration in renal function and anuria had led to a requirement for regular hemodialysis from admission.
+After 3 days, captopril was changed to amlodipine because the patient developed a severe, intolerable cough thought to be associated with captopril.
+His systolic blood pressure was controlled between 140 and 180 mm Hg.
+At approximately 3 weeks after the initiation of maintenance hemodialysis, the patient newly developed a generalized tonic-clonic seizure disorder.
+A brain computed tomography (CT) scan revealed a small lacunar infarct over the left basal ganglion without intracranial hemorrhage or large infarct.
+The seizure resolved spontaneously without anticonvulsants, and the patient was finally discharged home without incident and scheduled for regular hemodialysis thrice weekly.
+However, at 1 week after discharge, the patient developed a sudden-onset headache and vomiting with confusion and recurrence of the generalized tonic-clonic seizure.
+He presented at the emergency department with a blood pressure of 183/100 mm Hg, platelet count of 149,000/μL, serum LDH of 332 U/L, and a peripheral blood smear containing 2–3 schizocytes/HPF (Fig.2).
+Brain CT revealed no interval change, and lumbar puncture revealed traumatic tapping only.
+Cerebrospinal fluid cultures were negative for bacteria, mycobacteria, and viruses.
+Brain magnetic resonance imaging (MRI) showed bilateral hyperintensity in the occipital and parietal lobes on a fluid-attenuated inversion recovery (FLAIR) sequence (Fig.3).
+Finally, the patient was diagnosed with PRES.
+We resumed captopril therapy at a dose of 25 mg thrice daily.
+As it was difficult to differentiate SRC from TTP, we also considered plasma exchange treatment.
+However, the patient's mental status returned to normal within 3 days in the absence of plasma exchange, and a follow-up MRI of the brain 2 months later showed complete resolution of cerebral edema (Fig.4).
+Therefore, SRC-related PRES was confirmed.
+Although the patient experienced a full neurologic recovery, his renal function did not improve and he remained dialysis dependent.