A 23-year-old woman with asthma and normal vesicular development and regular menstrual cycles presented with a nephrotic syndrome and exanthema in sun-exposed areas.
Renal function was normal and serology for systemic lupus erythematosus (SLE) was negative.
Renal biopsy showed a lipoid nephrosis.
She was treated with prednisone with complete remission.
In the following 3 years she had several reactivations with corticoids 8 years.
Four years later she developed polyuria.
Although her parents had diabetes mellitus 2 (DM2) diagnosed before the age of 50, her pre- and post-load glycemia was normal.
Aqueous restriction test showed increased serum sodium and persistence of polyuria, normalizing when desmopressin was administered.
A turcica scan showed no abnormalities.
Insipidus diabetes (ID) was diagnosed and daily desmopressin was prescribed.
One year later, he presented cold intolerance, dry skin and weight gain, without palpable goiter.
Laboratory tests showed TSH: > 100 uUI/mL (VN: 0.8-4.4), T4: 1.9 ug/dL T3: 80.7 ng/mL (VN: 52-175), antibodies-antiperoxida.
Levothyroxine 100 ug/day was prescribed, improving symptoms and normalizing TSH.
Two years later the ID disappeared.
Combined hormonal contraceptives were prescribed.
A year later, a plaque of alopecia appeared, which became widespread and universal alopecia appeared.
Months later the hair reappeared, but white, giving the appearance of albinism.
Serum cortisol was 13.2 ug/dL (VN: 7-11) at 08:00 AM; TSH: 0.93 uUI/mL, T4: 8.4 ug/dL (without changes in dosage).
No ACTH stimulation test was performed.
At 34 years (11 years after nephrotic syndrome), she noticed amenorrhea when giving birth control.
There was a pattern of hypogonadotropic hypogonadism: FSH 0.8 mIU/mL (VN: 2.5-10), LH: 0.52 mIU/mL (VN: 1.9-2.5) with undetectable estradiol levels.
Prolactin was elevated (53.6 ng/mL) (VN: 2.11-25).
Cabergoline was not prescribed empirically and prolactin levels were normalized without recurrence of menstrual periods.
One year later cortisol AM levels were low (5.6 ug/dL) with normal-low ACTH (15.2 pg/mL, VN: 10-60), consistent with central adrenal insufficiency.
No ACTH stimulation test was performed and hydrolytic agents were administered in physiological doses.
Turcica MRI showed increased pituitary volume reported as macroadenoma with suprasellar extension.
The visual field was normal and showed no symptoms of ID.
Due to the progressive growth of the pituitary gland and imminent visual impairment, transsphenoidal resection was performed.
Biopsy showed a polyclonal hyperplasia of plasma cells.
It was prescribed, but due to progression of prednisone deficiency and deterioration of the visual field, despite treatment, a new trans-phenoidal resection was performed.
Histology was similar to that previously described, but subsequent immunohistochemistry showed the presence of IgG4 positive plasma cells.
Postoperatively, DI recurred and demopresin was prescribed.
External radiotherapy was performed to prevent pituitary growth.
Later, artificial hypolacrimia was treated.
One year later, in an outpatient follow-up, malar rash, cutaneous eczema in sun-exposed areas and elevated ESR at 102 mm/h were observed.
SLE was diagnosed and serology confirmed the diagnosis.
Antinuclear antibodies were (+) 1: 1,280, C’3 was low with normal C’4 and anti-ENA, anti-DNA and anti-phospholipid antibodies were (-).
Renal function and urine test were normal.
A few months later, hypertension and diabetes mellitus 2 were diagnosed, and loartan and metformin were prescribed.
Two years later, she presented with low back pain and deaf hypogastric pain, constipation, anemia and elevated creatinine to 1.4 mg/dL.
A scanner showed peri-aortic and perirenal tissue with bilateral hydronephrosis suggestive of PKF.
The biopsy confirmed the diagnosis.
Pigtail catheters were installed and 30 mg of prednisone and 0.5 mg of colchicine were prescribed every 12 h.
The symptoms disappeared rapidly with normal creatinine levels.
Plasma IgG4 concentration (measured under steroidal therapy) was normal.
The immunohistochemistry of the retroperitoneal tissue showed IgG4 producing plasma cells in the fibrous tissue.
Four months later the catheters were removed.
In the following years the retroperitoneal and perirenal tissue disappeared.
Twenty-five years after the first evaluation, the patient has no signs of hypophysis or PKF, maintains hormone supplementation and has adequate control of other chronic diseases.
