Hiroyuki Kamiya 1), Soichiro Ikushima 1), Tetsu Sakamoto 1), Kozo Morimoto 1), Tsunehiro Ando 1), Masaru Oritsu 1), Atsuo Goto 2), Tamiko Takemura 3)

Transcription

1 ACE39.7IU/l X TBLB Langhans [ ] A Case of Granulomatous Interstitial Nephritis with Progressive Renal Impairment Due to Sarcoidosis in the Course of Spontaneous Improvement of Pulmonary Lesions Hiroyuki Kamiya 1), Soichiro Ikushima 1), Tetsu Sakamoto 1), Kozo Morimoto 1), Tsunehiro Ando 1), Masaru Oritsu 1), Atsuo Goto 2), Tamiko Takemura 3) ABSTRACT A 29-year old man was admitted to our hospital due to low-grade fever and abnormal pulmonary shadows in May Laboratory findings revealed an elevated serum ACE up to 39.7 IU/l and uveitis was also noted. Chest X-ray film showed bilateral lymphadenopathy and patchy shadows scattered in the bilateral lung fields. A transbronchial lung biopsy (TBLB) demonstrated epithelioid cell granulomas, compatible with sarcoidosis. Serum creatinine gradually elevated although pulmonary lesions almost disappeared without treatment. A specimen of percutaneous renal biopsy demonstrated epithelioid cell granulomas with Langhans giant cells, tubular atrophy and fibrosis in the interstitium, corresponding with granulomatous interstitial nephritis. Renal function improved after corticosteroid therapy, but was not completely recovered. It should be kept in mind that granulomatous interstitial nephritis due to sarcoidosis could cause renal failure unless treatment is started at an early stage. [ ] keywords ; Sarcoidosis Renal impairment Granulomatous interstitial nephritis 1) 2) 3) TEL FAX mlb04194@nifty.com The Department of 1) Respiratory Medicine 2) Nephrology 3) Pathology, Japanese Red Cross Medical Center 57

2 cm 74.5kg /55mmHg 70/ 1.1mg/dl 10.4mg/dl ACE39.0 IU/l IL-2R 4650 U/ml 14 mm X CT 67 Ga-citrate CD4/8 2.1 TBLB Table 1. Laboratory findings on admission Hematology WBC St Seg Ly Mono RBC Hb Ht Plt 8100 / l / l 15.3 g/dl / l Chemistry TP Alb GOT GPT LDH ALP BUN Cr Na K Cl Ca 7.6 g/dl 4.1 g/dl 26 IU/l 26 IU/l 426 IU/l 172 IU/l 17 mg/dl 1.1 mg/dl 140 meq/l 4.5 meq/l 102 meq/l 10.5 mg/dl Serology CRP 1.9 mg/dl sil-2r 4650 U/ml ACE 39.0 IU/l BGA (room air) ph PaCO2 41 Torr PaO2 98 Torr HCO meq/l BE 1.5 meq/l A-aDO Torr PPD skin test 14 8 mm (positive) ECG Normal sinus rhythm ST-T change ( ) Pulmonary function test VC 4.86 L VC 109 FEV L FEV DLco/VA 5.78 ml/min/mmhg/l 58

3 X 2+ 2MG NAG16511 g/g U/g mg/dl ( ) Langhans 2.4mg/dl m-psl g/ PSL 40mg/ 2.5mg/dl 1.8mg/dl ACE 24.5IU/l 6.5IU/l PSL40mg/ 10mg/dl A Figure 1A. Figure 1B. Chest X-ray film on admission shows diffuse patchy shadows with bilateral hilar lymphadenopathy (BHL). Chest X-ray film several months later shows a marked improvement of diffuse patchy shadows and hilar lymphadenopathy (BHL). B Figure 2. CT scan of the chest on admission shows cotton-like lesions on bilateral lung fields. Figure Ga-citrate scintigraph shows accumulation of 67 Ga-citrate in the mediastinum, hila, and bilateral lungs, besides other lesions including bilateral lacrimal glands, parotid gland, and kidney. 59

4 Figure 4. specimen of transbronchial lung biopsy (TBLB) shows epithelioid cell granulomas with multinucleated giant cells (arrow heads) and lymphocytic alveolitis (arrows). (HE stain, 20) Figure 5. A specimen of percutaneous renal biopsy shows epithelioid cell granulomas (G), tubular atrophy and fibrosis (F) in the interstitium, corresponding with granulomatous interstitial nephritis. Calcification was not seen in any part of the interstitium. (Periodic acid silver methenamine (PAM) stain, 10) TBLB Renal biopsy mpsl pulse PSL Figure 6. Clinical course 1963 Mayok ) 2003 Bergner ) 3-7) 60

5 Hannedouche 51 8) 3-7) 1963 Mayok ) 2000 Sharma 63 9) ) 3-5,8) 3-5,8) 10mg/dl 13mg/dl 11) -27 8) 40 3) 13 12) 40 5) 3-5,7,8,13) 2-8,13-15) 4,5,8,13) 8) PSL mg/kg/ 2,4,6,13) 4,5,7,13) 4) 2MG NAG 13-15) 67 Ga-citrate 15) 14) ACE BHL 16) ) 61

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