A previously healthy 30-year-old Chinese man reported fever and anorexia for 1 week; he had to stop playing football because of marked lethargy. Laboratory evaluation showed acute impairment of renal function with an increase in serum creatinine level from 0.97 mg/dL (86 μmol/L; glomerular filtration rate estimated by using the Modification of Diet in Renal Disease Study equation > 60 mL/min/1.73 m2 [>1.0 mL/s/1.73 m2]) 5 months previously to 3.7 mg/dL (327 μmol/L). Urine examination showed trace proteinuria and few leukocytes. Urine protein excretion was less than 0.1 g/d. A renal biopsy (Fig 1A) was performed. His fever persisted, accompanied by increased levels of inflammatory markers, C-reactive protein level of 57 mg/L, and erythrocyte sedimentation rate of 103 mm/h. After 1 month, bilateral anterior uveitis developed. Chest radiograph and echocardiography findings were normal. Repeated blood and urine cultures showed no growth. Antinuclear antibody, anti–double-stranded DNA, and antineutrophil cytoplasmic antibody test results were negative. Features of systemic lupus erythematosus or Behçet disease were not present. A bone marrow biopsy (Fig 1B) was performed.
Figure 1B. Representative bone marrow biopsy specimen.
■What do you observe on this image of the patient’s renal biopsy?
■What abnormality do you see on this bone marrow biopsy?
■What is your diagnosis?
Discussion
What do you observe on this image of the patient’s renal biopsy?
Figure 1A shows heavy infiltration in the tubulointerstitium by inflammatory cells, mostly lymphocytes and plasma cells.
What abnormality do you see on this bone marrow biopsy?
Figure 1B shows giant cells and surrounding eosinophils.
What is your diagnosis?
The patient had an acute systemic inflammatory process including acute interstitial nephritis and ocular involvement with uveitis. Histological findings in the kidney specimen include inflammatory cell infiltration, eosinophils, and frequently noncaseating granulomas. This distinctive syndrome of tubulointerstitial nephritis, anterior uveitis, and bone marrow granulomas was first described more than 30 years ago.1 Tubulointerstitial nephritis and uveitis (TINU syndrome), initially reported in children and adolescents, increasingly is being recognized in adult patients as well, and it is believed to represent a lymphocyte-mediated immune disorder.
Our patient subsequently showed resolution of renal disease after systemic corticosteroid therapy, although the uveitis had a characteristic chronic relapsing course. On the whole, the renal component of TINU syndrome seems to have excellent prognosis, with mild residual renal insufficiency in a small percentage of patients.2 Relapse of uveitis is common, as in our patient, and does not predict recurrence of the renal disease.2, 3
Final Diagnosis
TINU syndrome.
References
1. 1Dobrin RS, Vernier RL, Fish AL. Acute interstitial nephritis and renal failure with bone marrow-lymph node granulomas and anterior uveitis: A new syndrome. Am J Med. 1975;59:325–333. Abstract |
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2. 2Kadanoff R, Lipps B, Khanna A, Hou S. Tubulointerstitial nephritis with uveitis (TINU): A syndrome rheumatologists should recognize: A case report and review of the literature. J Clin Rheumatol. 2004;10:25–27.
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3. 3Mackensen F, Smitth JR, Rosenbaum JT. Enhanced recognition, treatment and prognosis of tubulointerstitial nephritis and uveitis syndrome. Ophthalmology. 2007;114:995–999. Abstract | Full Text |
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Case provided and authored byKai Ming Chow, MBChB, MRCP,1 Fernand Mac-Moune Lai, FRCPA, MD,2 Cheuk Chun Szeto, MBChB, FRCP, MD,1 Natalie Pui Ha Chan, MRCP,2 Edward Ho Chung Wong, MBChB, MRCP,1 and Philip Kam-tao Li, FRCP, FACP, MD,1 Departments of 1Medicine and Therapeutics and 2Anatomical and Cellular Pathology, Prince of Wales Hospital, The Chinese University of Hong Kong, Shatin, Hong Kong, SAR, China
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