PMC5313424_01
A: A CT image showing bilateral hydronephrosis before steroid treatment. B: A retroperitoneal mass before steroid therapy. C: A CT image of the kidneys after three months of steroid therapy. D: The retroperitoneal mass after three months of steroid therapy. E: A pulmonary pseudotumor on the right lobe before steroid treatment. F: Pulmonary pseudotumor on the left lobe before steroid treatment. G: Gallium-67 scintigraphy taken on the fifth day of steroid therapy, showing the positive uptake in the left mandibular gland (arrow).
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A CT scan on admission revealed bilateral hydronephrosis and a periaortic mass (Fig. 1A and B, respectively). Small lung nodules that could be IgG4-related inflammatory pseudotumors were observed in both lobes (Fig. 1E and F). Gallium-67 scintigraphy on the fifth day of steroid therapy (Fig. 1G) was negative in the retroperitoneal area and kidneys but positive in the left mandibular gland. By the fourth month of steroid therapy, the retroperitoneal mass had diminished (Fig. 1D), and bilateral hydronephrosis and the swelling of the kidneys were completely relieved (Fig. 1C).
Upper panels: Sagittal T1-weighted images of the pituitary gland. A: After nine days of steroid therapy. B: After two months of steroid therapy. C: In the eighth month, when relapse occurred. D: Two weeks after the dose of prednisolone was increased. E: The time-course of urine volume and the serum TSH and IgG4 responses to prednisolone and oral desmopressin treatments. In the ninth month of steroid therapy, dexamethasone was temporarily used in place of PSL so that endocrinological tests could be performed.
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MRI after one week of steroid therapy revealed the significant enlargement of the pituitary stalk and body, with diminished intensity of the posterior "bright spot" on a T1-weighted image (Fig. 2A). In spite of the above-mentioned improvements to the patient's condition, his patient's urine volume markedly increased from approximately 2 L/day to 5-7 L/day after two weeks of prednisolone therapy (Fig. 2E). After steroid therapy, the patient's serum IgG4 concentration responded well, decreasing to 71.6 mg/dL by the third month, which was below the cut-off of 135 mg/dL (Fig. 2E). His TSH concentration fluctuated, with transient decreases as the steroid dose was reduced - it eventually returned to normal levels (Fig. 2E). A pituitary MR image taken after seven weeks of steroid therapy showed the clear amelioration of the pituitary and stalk swellings (Fig. 2B). Furthermore, the TSH level also decreased to 0.72 muU/mL the eighth month; however, it remained within the normal range, and follow-up MRI showed the apparent swelling of the pituitary body and stalk (Fig. 2C). The patient's prednisolone dosage was increased to 40 mg/day, and his serum IgG4, TSH, and pituitary enlargement rapidly improved (Fig. 2D).