A 57-year-old woman presented with fever and cellulitis of the right leg. Urinalysis and kidney function were normal on admission. Cellulitis remitted but fever persisted for six weeks. X-ray imaging, cultures, serological assays for viruses and autoimmunity did not reveal the cause of fever. Unexpectedly anti-Scl 70 (anti-topoisomerase I) antibodies were positive. A skin biopsy ruled out scleroderma. On the fifth hospitalization week kidney function declined in association with hematuria, leucocyturia, and proteinuria. Prednisone was administered due to clinical suspicion of drug-induced interstitial nephritis. Fever declined in 24 h, but renal failure became rapidly worse requiring hemodialysis. Kidney biopsy revealed extensive crescentic glomerulonephritis (CGN), with much glomerular destruction, with an IgG-positive linear pattern on immunofluorescence microscopy. No overtly active microangiopathy or vasculitis were present. There was no pulmonary involvement and anti-glomerular basement membrane antibodies were not detected in the serum. After one year anti-Scl 70 antibodies were still positive without scleroderma manifestations and 17 months later the patient received a kidney transplant with excellent results. Presentation of type 1 CGN as a fever of unknown origin (FUO) is exceptional. Anti-Scl 70 antibodies are highly specific for scleroderma and are seldom present in other diseases. As far as we are aware there are no published cases of the association of type 1 CGN with anti-Scl 70 antibodies.
Urothelial cancer and other cancers directly invading to urinary tract can cause hematuria. Hematuria is sometimes difficult to control and lowers patient's quality of life. Radiation therapy (RT) has been used to alleviate hematuria in a palliative setting, but its safety and efficacy data are limited. Therefore, we aimed to assess the effectiveness of palliative RT on hematuria caused by advanced cancer. Materials/Methods: Patients who received palliative RT to control gross hematuria from August 2006 to October 2015 in our hospital were retrospectively reviewed. Patients who underwent RT for curative intent were excluded. Hematuria was evaluated by treating physicians. Transfusion record was reviewed from 3 months prior to RT through the follow-up period. The rate of blood transfusion and gross hematuria before and after RT was compared using chi-square test. Kaplan-Meier method was used to analyze gross hematuria free survival and overall survival. Results: Twenty seven cases of 26 patients received RT to control gross hematuria from advanced cancers. One patient received RT twice. Median follow-up duration was 158 days (range, 9-989) and median age was 79 years (36-96). Primary sites of cancers were bladder (nZ14, 52%), prostate (nZ6, 22%), ureter (nZ3, 11%), rectum (nZ3, 11%) and colon (nZ1, 4%). RT dose ranged from 10 Gy to 45 Gy. Most commonly used regimen was 20 Gy in 5 fractions (8 patients), followed by 36 Gy in 12 fractions (6 patients). Median overall survival was 167 days. To date 21 patients died and 20 of them died of cancer. Fourteen patients (52%) required blood transfusion before RT, and 11 of them (79%) became transfusion free post RT. Median gross hematuria free survival was 121 days. Eighteen cases (67%) achieved gross hematuria free after RT. Median duration of gross hematuria free in the 18 cases was 178 days. Two of 7 patients who died within 1 month after RT achieved gross hematuria free whereas 16 of 20 cases who survived for longer than 1 month achieved gross hematuria free (PZ0.013). Patients who received 30 Gy or higher had better gross hematuria free survival (PZ0.045, log-rank). There was no statistically significant difference in gross hematuria free survival among different primary sites. There were only 3 grade 1 adverse events (2 diarrheas and 1 urinary frequency). Conclusion: RT can safely control hematuria and reduce transfusion requirement regardless of primary cancer sites. Patients whose life expectancy of shorter than 1 month may not benefit. Higher than 30 Gy may be associated with better hematuria control.
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