NDT Advance Access originally published online on April 4, 2008
Nephrology Dialysis Transplantation 2008 23(7):2406-2408; doi:10.1093/ndt/gfn156
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No way in & no way out: a case of renal failure due to both pre- and post-renal obstruction
Department of Renal Medicine, York Hospital, Wiggington Road, York YO31 8HE, UK
Correspondence and offprint requests to: Oliver Monfredi, Department of Renal Medicine, York Hospital, Wiggington Road, York YO31 8HE, UK. Tel: +44-01904-631313 Ext 5374; Fax: +44-01904-726354; E-mail: oliver.monfredi{at}manchester.ac.uk
Keywords: Erdheim–Chester disease; histiocytosis; imaging; Langerhans cell histiocytosis; magnetic resonance angiography; renal failure
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Introduction |
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Causes of renal failure can be classified as pre-renal, intra-renal and post-renal. We discuss a case of combined pre-and post-renal failure due to an unusual presentation of a rare disease. The case illustrates the usefulness of combining imaging modalities in investigating complex renal disease.
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Case discussion |
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A 46-year–old lady presented to the renal physicians in 2002 with a biopsy diagnosis of Langerhans cell histiocytosis (LCH). Initial presentation had been with maxillary and retro-orbital disease. A CT scan of the thorax/abdomen/pelvis in 2002 had shown extensive irregular soft tissue around and extending into the sinuses of both kidneys (Figure 1), with renal calyceal but not pelvic dilatation, as well as disseminated involvement including bone, mediastinum and lungs. Her serum creatinine was normal and a MAG3 renogram showed no evidence of functional obstruction. She had no symptoms other than occasional loin discomfort.
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Three years later she developed worsening hypertension, a rising serum creatinine and pulmonary oedema. MR angiography showed bilateral tight renal artery stenosis. It again showed the unusual pattern of strikingly dilated calyces but collapsed renal pelves. Dynamic renography and antegrade pyelography (Figure 2) indicated obstruction at the level of the renal pelvis bilaterally. Consequently, percutaneous nephrostomies were placed, but there was no improvement in renal function.
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In view of the episode of pulmonary oedema, renal artery angioplasty was attempted. Digital subtraction angiography confirmed critically tight but focal renal artery stenoses (Figure 3). After intra-arterial heparin, the left-sided stenosis was traversed with a guide wire, but even this was sufficient to occlude the artery, and it was not possible to advance any catheter through the lesion. On removing the guide wire the artery remained occluded. Treatment on the right was not attempted. Despite maximal medical treatment including diuretics, ACE inhibitors and haemodiafiltration, the patient developed refractory episodes of pulmonary oedema and died. Some diagnostic uncertainty remained regarding whether the diagnosis was of true LCH or of the rarer Erdheim–Chester disease (ECD), since initial biopsies had not been exposed to the full panel of immunohistochemistry and electron microscopy required to differentiate these two closely related disorders (see discussion below).
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Discussion |
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LCH is a collection of disorders of unknown aetiology [1,2], having in common the pathological proliferation of specialized bone marrow–derived antigen-presenting Langerhans cells (LCs) and mature eosinophils. Initially described in 1868 by Paul Langerhans, the condition is now known to result from deficient presentation of antigen by the diseased LCs. Attempts at classification of LCH have been confusing. It is accepted that histiocytic disorders may be split into three different groups [3]:
- dendritic cell histiocytoses
- erythrophagocytic macrophage disorders
- malignant histiocytoses
LCH belongs to group 1 and spans a spectrum from limited and indolent disease, to acute and fulminating. Letterer–Siwe disease, Hand–Schüller–Christian disease, histiocytosis X and eosinophilic granuloma were names previously used to signify different forms of LCH, but are now largely redundant. The current preferred means of classifying LCH is based on whether disease involves single or multiple sites, and single or multiple organs. Prognosis and treatment depend on the extent of the disease when classified like this, and whether certain high-risk organs are involved (e.g. liver, spleen, lung, bone marrow). Common presenting complaints include skin rash, dyspnoea, painful bony lesions, lymphadenopathy, weight loss, proptosis and polydipsia with polyuria (diabetes insipidus). Diagnosis relies on histological demonstration of abnormal Langerhans cells (show pathognomonic Birbeck granules on electron microscopy, CD1a-, CD14+-, and CD52-positivity, and finally staining for antibodies to the granule protein ‘Langerin’). Effective treatment can be given in the form of combination chemotherapy, e.g. etoposide, vinblastine and prednisolone. Survival in disseminated disease is 
50% at 5 years.
Previous authors [4–9] have described renal artery stenosis and chronic kidney disease in ECD, a rare, class 2, non-LCH typified by extensive retroperitoneal infiltration with foamy histiocytes. This is often mistaken for LCH, and is differentiated by histological demonstration of absent Birbeck granules and absent S100 protein staining. In a case series and literature review reported by Haroche et al. [10] of 72 patients with cardiovascular complications of ECD, 8% had renovascular hypertension related to peri-renal artery stenosis. There are few reported cases of renal artery stenosis and severe renal impairment in true LCH. The above case illustrates well the need to employ all imaging modalities to fully assess extremely challenging cases of pre- and post-renal obstructive disease.
Conflict of interest statement. None declared.
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References |
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- Howarth DM, Gilchrist GS, Mullan BP, et al. Langerhans cell histiocytosis: diagnosis, natural history, management, and outcome. Cancer (1999) 85:2278–2290.[CrossRef][Web of Science][Medline]
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- www.histio.org.
- Leluc O, Andre M, Marciano S, et al. Retroperitoneal complications of Erdheim–Chester disease. J Radiol (2001) 82:580–582.[Web of Science][Medline]
- Chiang KS, Larson TS, Swee RG, et al. CT of Erdheim–Chester disease presenting as retroperitoneal xanthogranulomatosis. J Comput Assist Tomogr (1994) 18:503–505.[Web of Science][Medline]
- Veyssier-Belot C, Cacoub P, Caparros-Lefebvre D, et al. Erdheim–Chester disease. Clinical and radiologic characteristics of 59 cases. Medicine (Baltimore) (1996) 75:157–169.[Medline]
- Bancroft LW, Berquist TH. Erdheim–Chester disease: radiographic findings in five patients. Skeletal Radiol (1998) 27:127–132.[CrossRef][Web of Science][Medline]
- Kenn W, Stabler A, Zachoval R, et al. Erdheim–Chester disease: a case report and literature overview. Eur Radiol (1999) 9:153–158.[CrossRef][Web of Science][Medline]
- Ayuso JR, Garcia-Criado A, Caralt TM, et al. Atypical retroperitoneal fibrosis: MRI findings. Eur Radiol (1999) 9:937–939.[CrossRef][Web of Science][Medline]
- Haroche J, Amoura Z, Dion E, et al. Cardiovascular involvement, an overlooked feature of Erdheim–Chester disease: report of 6 new cases and a literature review. Medicine (Baltimore) (2004) 83:371–392.[CrossRef][Medline]
Accepted in revised form: 28. 2.08
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