QJM Advance Access originally published online on February 12, 2008
QJM 2008 101(4):327-329; doi:10.1093/qjmed/hcn009
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Ureteral nephrogenic adenoma in antiphospholipid antibody syndrome
Department of Internal Medicine
Department of Pathologic
Histology and Cytology
Department of Urology,
Rouen University Hospital,
76031 Rouen Cedex,
France
email: isabelle.marie{at}chu-rouen.fr
Sir,
Primary antiphospholipid antibody syndrome (PAPS) is characterized by recurrent venous and arterial thrombosis, or both, or repeated fetal loss, associated with the presence of anticardiolipin (acL) or anti-β2 glycoprotein 1 (anti-β2 GP1) antibody or lupus anticoagulant (LAC) on two samples at least 8 weeks apart, in the absence of other connective-tissue disorders.1 Systemic manifestations are well recognized complications of PAPS. Renal involvement is not an uncommon complication of the disease, occurring in as many as 2.7–30% of patients.2,3 In PAPS patients, renal impairment may indeed lead to: renal artery stenosis and/or malignant hypertension, renal infarction, renal vein thrombosis, thrombotic microangiopathy, increased allograft vascular thrombosis and reduced survival of renal allografts and non-thrombotic conditions like glomerulonephritis.2,3 Urologic impairment has not been described in PAPS patients; only Fernandez-Rosado et al.4 have, in fact, reported the case of a patient with neurogenic bladder related to spinal cord thrombosis in a patient with PAPS. We recently observed a patient with systemic lupus erythematosus (SLE) and antiphospholipid antibody syndrome (APS) who developed bilateral ureterohydronephrosis related to ureteral nephrogenic adenoma.
A 24-year-old man presented with a 1-month history of asthenia and abdominal pain. He had a previous medical history of SLE and associated APS evolving for 2 years; systemic manifestations of SLE and APS included: (i) cardiac impairment, characterized by pericarditis and heart valve dysfunction involving the mitral valve; (ii) renal impairment, i.e. focal mesangioproliferative glomerulonephritis and (iii) stroke. He received combined therapy of prednisone (20 mg daily) and warfarin (target INR: 2–3). On admission, the patient was non-febrile. Physical examination showed tender abdomen within the left iliac area; general examination was otherwise normal. Laboratory findings disclosed the following: erythrocyte sedimentation rate: 26 mm/h, C-reactive protein: 5 mg/l, haemoglobin: 7.1 mmol/l, white blood cell count: 6.4 x 109/l, platelet count: 164 x 109/l, urea: 11.1 mmol/l, creatinine: 128 µmol/l. Blood protein electrophoresis showed: total protein: 65 g/l, albumin: 34 g/l. Proteinuria was found to be 0.7 g/24 h. Urinalysis showed neither infection nor hematuria and abnormal cells. Autoantibody screening tests revealed antinuclear antibodies (1:1000), anti-DNA antibody (10%), acL IgG: 120 UGPL/l and IgM: 216 UGPL/l, positive LAC (LAC was quantified with the dilute thromboplastin time inhibitor test, which showed a high dilution of thromboplastin: 1/300); it was negative for complement profile, cryoglobulin and antineutrophil cytoplasmic antibodies. Abdominal ultrasound examination and computed tomography showed bilateral ureterohydronephrosis, predominating on the left ureter (Figure 1). Retrograde ureteropyelography revealed that both ureters were stenosed in the middle ureteral segment. Prednisone regimen was increased to a dose of 50 mg daily. Because of corticosteroid refractory ureteral involvement, surgical biopsies of the left ureter were performed. Histological analyses of stenosed ureteral biopsy specimens demonstrated damage consistent with nephrogenic adenoma (Figure 2), showing: fine papillary structures and papillal covered by a single layer of columnar cells (i.e. with tubular structures common to the renal unit).
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Nephrogenic adenoma is a rare tumor-like lesion composed of small tubules resembling renal structure along the urinary system (pelvis, ureter, bladder and urethra).5–8 Although the pathogenesis of nephrogenic adenoma is not clearly understood, the condition has usually been associated with irritative stimuli to the urothelial epithelium leading to metaplastic changes of the multipotent urothelium, including: genitourinary surgery, permanent/repeated catheterization, lithiasis, infection, chemotherapy/radiotherapy or drug abuse (analgesic agents).5–10
We report, to the best of our knowledge, the first case of nephrogenic adenoma in a patient with APS. The pathological mechanisms of nephrogenic adenoma in our patient with APS remain unclear, which raises the question of whether the condition arose through a causal association (as part as a continuum) or by chance. Nevertheless, in our patient, the diagnosis of nephrogenic adenoma related to infection, trauma, surgery, catheterization as well as drugs (i.e. analgesic abuse) could be excluded; ureteral involvement related to SLE was also unlikely as histology did not demonstrate associated damage consistent with SLE vasculitis in the ureteral vessels.11 Taken together, we suggest that one pathological mechanism may explain nephrogenic adenoma onset in our patient with APS-associated vaso-occlusive disease. In essence, Mazal et al.7 have suggested that nephrogenic adenoma may be, in fact, derived from detached renal tubular cells along the urothelial tract in previously injured areas; in turn, high incidence of detachment of viable renal tubular cells has been reported in patients with renal disease in hypoxic conditions.7 In this instance, tubular ischemia related to APS may have been responsible for detachment of renal tubular cells, resulting in ureteral nephrogenic adenoma.
References
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2. Amigo MC. Kidney disease in antiphospholipid syndrome. Rheum Dis Clin North Am (2006) 32:509–22.[CrossRef][Web of Science][Medline]
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4. Fernandez-Rosado E, Sanchez Rodriguez-Losada J, Alvarez Castelo L, Blanco Diez A, Barbagelata Lopez A, Ponce Diaz-Reixa J, et al. Urologic damage of the primary antiphospholipid syndrome. Arch Esp Urol (2004) 57:707–23.[Medline]
5. Ducrocq S, Bruniau A, Cordonnier C, Demailly M, Petit J, Saint F. Bladder nephrogenic metaplasia: circumstances of discovery, predisposing factors, and clinical course in 7 cases diagnosed between 1988 and 2000. Prog Urol (2003) 13:613–7.[Web of Science][Medline]
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7. Mazal PR, Schaufler R, Altenhuber-Müller R, Haitel A, Watschinger B, Kratzik C, et al. Derivation of nephrogenic adenomas from renal tubular cells in kidney-transplant recipients. N Engl J Med (2002) 347:653–9.
8. Scelzi S, Giubilei G, Bartoletti R, Di Loro F, Mondaini N, Crisci A. Nephrogenic adenoma of bladder after ibuprofen abuse. Urology (2004) 64:1030.e13–4.
9. Bozkurt SU, Erbarut I, Yazici C, Kaya H, Turkeri L. Nephrogenic adenoma of the ureter: case report. Int Urol Nephrol (2007) 39:65–9.[CrossRef][Web of Science][Medline]
10. Fernandez-Jimenez I, de Diego Garcia E, Sandoval Gonzalez F. Nephrogenic adenoma of the urethra. Report of a case. Cir Pediatr (2003) 16:203–4.[Medline]
11. Kobayashi K, Obara K, Watanabe R, Hara N, Katagiri A, Takahashi K. Obstruction of the ileal ureter by mesenteric vessels occurring 5 years after total ureteral substitution for bilateral ureteral stenosis due to systemic lupus erythematosus. Int J Urol (2006) 13:80–3.[CrossRef][Web of Science][Medline]
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