| Clinicopathologic Characteristics of C1q nephropathy |
| Shin-young Ahn, M.D.1, Jae-yoon Park, M.D.1, Seong Woo Lee, M.D.1, Jong Cheol Jeong, M.D.1, Ji Yong Jung, M.D.3, Kook-Hwan Oh, M.D.1, Yon Su Kim, M.D.1, Curie Ahn, M.D.1, Jin Suk Han, M.D.1, Suhnggwon Kim, M.D.1, Kyung Chul Moon, M.D.2 and Kwon Wook Joo, |
Department of Internal Medicine1
Seoul National University College of Medicine, Seoul, Korea, Department of Pathology2
Seoul National University College of Medicine, Seoul, Korea, Department of Internal Medicine3
Gachon University of Medicine and Science Laboratory of Molecular Nephrology, Gachon University of Medicine and Science, Incheon, Korea |
| 원저 : C1q nephropathy의 임상병리학적 소견 |
| 안신영1, 박재윤1, 이성우1, 정종철1, 정지용3, 오국환1, 김연수1, 안규리1, 한진석1, 김성권1, 문경철2, 주권욱1 |
| 서울대학교 의과대학 내과학교실1 , 서울대학교 의과대학 병리학교실2, 가천의과학대학교 의학전문대학원 내과학교실3 |
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| Abstract |
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Purpose : C1q nephropathy (C1qN) is a rare glomerulonephritis characterized by mesangial deposits, predominantly C1q, without the evidence of systemic lupus erythematosus (SLE). It showed various clinical courses, however, the clinicopathologic features of C1qN have not been well defined as yet.
Methods : We retrospectively reviewed the clinicopathologic features of 11 patients (0.8%) diagnosed as C1qN among 1,403 patients aged ≥18 years who had undergone renal biopsy due to primary glomerular disease from Jan. 2000 to Jan. 2009. Diagnostic criteria of C1qN were as follows; 1) the presence of dominant or co-dominant immunofluorescence staining for C1q in the mesangium, 2) corresponding mesangial dense deposit by electron microscopy, and 3) lack of clinical evidence of SLE.
Results : The male-to-female ratio was 6:5 and their mean age was 41.1±22.6 yrs (range, 19-69 yrs). Eight patients presented with urinary abnormalities and three with nephrotic syndrome. At the time of biopsy, three patients had hypertension. The mean value of 24-hour urine protein was 4.4±5.5 g/day (range, 0.5-18.5 g/day). On light microscopy, normal glomerular architecture (4/11) and segmental sclerosis (7/11) were observed. Complete or partial remission was achieved in six of the seven patients treated with immunosuppressive agents (steroid and/or immunosuppressants). Among these patients, two using steroid monotherapy had relapsed. The mean follow-up duration was 14±11 months (range, 2-31 months) and renal function deterioration was observed in three patients.
Conclusion : C1qN showed various clinical manifestations and prognosis. Therefore, additional studies are needed to fully define the clinicopathologic features. |
| Key Words:
C1q, Glomerulonephritis, Nephrotic syndrome |
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