An unusual presentation of renal failure

(Section Editor: M. G. Zeier)

N. Khoury, G. Minot, C. Vigneau, F. Vincent, Y. Allory, M. Tligui, P. Callard, E. Rondeau and J. D. Sraer

Departments of Nephrology, Urology and Pathology, Tenon Hospital, Paris France

Case

A 20-year-old black man was admitted to the hospital for a first generalized tonico-clonic seizure. One month earlier, he had had a headache, which he treated with analgesics (paracetamol and dextropropoxyphene). His medical history was unremarkable, except for recurrent ‘vanishing’ left axillary nodes since the age of 5 years, in the absence of infections or fever. At physical examination, the pulse was regular at 100 beats per min, blood pressure was 205/106 mmHg, temperature was 37°C rectal. The patient had a small, palpable left inguinal lymph node and a small axillary lymph node. Fundoscopy revealed grade IV hypertensive retinopathy. The remainder of the physical examination, including the neurological examination, was otherwise unremarkable. Signs of left ventricular hypertrophy were found on the ECG. The laboratory tests were as follows: a macrocytic anaemia with mild haemolysis (Hb=8.4 g/dl; VGM=106 µ3; schizocytes=1%; haptoglobin=0.42 g/l for a normal value >0.6 g/l), platelets=185000/mm3, normal coagulation tests, serum urea=42 mmol/l, serum creatinine=2004 µmol/l, K=4.6 meq/l, Cl=96 meq/l, Na=139 meq/l. On 24-h urine collection, proteinuria was 4 g, creatinine clearance was measured as 8 ml/min/1.73 m2, and a sterile pyuria was found.

On admission, chest X-ray and brain CT were within normal limits. However, on the abdominal ultrasound, the right kidney measured 8.5x3 cm with cortical atrophy and hyperechogenic parenchymal masses. The left kidney was about 10x5.5 cm, with irregular limits and multiple intraparenchymal tissular lesions. There was no evidence of arterial stenosis. MRI of the abdominal region confirmed an atrophic right kidney and a bumpy left one with numerous tumour-like lesions which took up gadolinium on T2 sequences (Figures 1Go and 2Go). Haemodialysis was initiated through a femoral catheter.



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Fig. 1.  MRI (longitudinal section) showing an atrophic right kidney and hypertrophic left one with irregular limits and tumour-like appearance.

 


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Fig. 2.  MRI (transversal section) showing a bosselated left kidney with numerous tumour-like lesions taking-up guadolinium on T2 sequence.

 

Question

What is your diagnosis? What additional tests would be helpful?

Answer to quiz on preceding page

As a neoplastic aetiology was first suspected, percutaneous biopsy was judged inappropriate and a left lumboscopic nephrectomy was performed. Macroscopic examination of the left kidney (8.5x5.5x4.5 cm) showed an irregular subcapsular surface with fibrous scars. The cortex and medulla were punctuated by the white scattered pin-head size nodules (Figure 3Go). The calyces were not dilated and no tumour was present. Histology showed an intense interstitial nephritis with a dense infiltrate of lymphocytes, plasmocytes, mastocytes, and macrophages, clustering in 100–500 µm size granulomas. Epithelioid and multinucleate giant cells were noticed within some granulomas, but without caseation (Figure 4Go). The tubules were atrophic with flattened cells and dilated lumen. A fraction of them had totally disrupted the epithelial border, with an epithelioid granulomatous reaction in the vicinity. Glomeruli were obsolete within areas of fibrous scar and were ischaemic with focal and segmental glomerulosclerosis elsewhere. Arteries displayed a fibrous intimal thickening without necrosis and no sign of angeitis was noticed. A bone marrow biopsy was normal. A thoracic CT scan showed evidence of two left axillary lymph nodes without any mediastinal or parenchymal abnormalities or features of sarcoidosis. We noticed a positive tuberculin intradermal reaction and no evidence of Mycobacterium on sputum, urine and gastric aspiration cultures. After axillary node excision, histologic examination demonstrated multiple coalescing granulomas, with epithelioid and multinucleate giant cells. These granulomas were associated with large and irregular areas of caseation, strongly suggestive of tuberculosis. Special staining including periodic-acid-Schiff, Ziehl and May-Grunwald-Giemsa were performed both on renal and lymph node sections but did not reveal any micro-organisms. PCR for Mycobacterium on the tissue specimen was negative, but 4 weeks later, both tissue cultures confirmed the diagnosis of tuberculosis. Broad anti-mycobacterial therapy was begun, including rifadine, isoniazid, ethambutol and pirazinamide. The patient is now on chronic haemodialysis three times a week through a radial fistula. Hypertension is controlled by the association of ACE inhibitors, calcium inhibitors and beta-blockers.



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Fig. 3.  Macroscopic aspect of the kidney.

 


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Fig. 4.  Microscopic examination showing interstitial nephritis without caseous necrosis.

 

Discussion

We report an unusual clinical and radiological presentation of renal tuberculosis, manifested by malignant hypertension and bilateral tumour-like renal masses. Bilateral masses first suggest malignant lesions and not mycobacterial disease. The differential diagnosis includes renal carcinoma, Wilm's tumour, sarcoma, lymphoma and metastatic carcinoma, von Hippel Lindau or tuberous sclerosis [14]. Renal pseudo-tumours and end-stage renal failure are unusual manifestation of tuberculosis. The common presenting signs and symptoms, absent in our patient, include fever, weight loss, anorexia, pulmonary diseases, bone marrow involvement and Pott's disease and lymphadenitis. The involvement of the urinary tract can be insidious and progress slowly to caseation and kidney granuloma with ureteral strictions. Renal involvement is usually unilateral, occurring in 3% of infected patients [7,8], and generally without evolution to irreversible renal failure. The malignant hypertension in our patient is due to terminal renal failure, as other causes of secondary hypertension were absent (pheochromocytoma, Conn disease and vasculitis).

Our case depicts unusual clinical and radiological features of end-stage renal failure due to tuberculosis, which must be included in the differential diagnosis of tumour-like renal masses. A broad investigation is essential to confirm or exclude mycobacterial infection, since young patients with renal failure require adequate chemotherapy before undergoing kidney transplantation.

Notes

Supported by an educational grant from

References

  1. Robert J. Medical progress: Renal-cell carcinoma. N Engl J Med1996; 355: 865–875
  2. Hilton S. Imaging of renal cell carcinoma. Semin Oncol2000; 27: 150–159[ISI][Medline]
  3. Schrier RW. Diseases of the Kidney, 6th edn. Blackwell Science, Oxford: 1999; 400–600
  4. Shah DR. Von Hippel-Lindau disease with multiple malignant renal tumours: The importance of genetic testing. Clinical Nephrol2000; 53: 216–221[ISI]
  5. Bruce A. Renal lymphoma: CT patterns with emphasis on helical CT. Radiographics2000; 20: 197–212[Abstract/Free Full Text]
  6. Hartman DS, Davis CJ, Goldman SM Jr et al. Renal lymphoma: Radiologic-pathologic correlations of 21 cases. Radiology1982; 144: 759–766[Abstract]
  7. Brenner BM. Urinary tract obstructive disease. In: The Kidney. Saunders, Philadelphia: 1991; 1768–1808
  8. Brenner BM. The Kidney, 6th edn. Saunders, Philadelphia, 1999; 1844–1862




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