NDT Advance Access originally published online on January 8, 2007
Nephrology Dialysis Transplantation 2007 22(3):949-951; doi:10.1093/ndt/gfl792
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© The Author [2007]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org
(Section Editor: M. G. Zeier)
Acute renal failure and febrile rash—infectious or not?
Department of Nephrology
Hopital Necker-Enfants Malades
AP-HP
Paris
France
Correspondence and offprint requests to: Aurélie Hummel. Email: hummel{at}necker.fr
Keywords: acute renal failure; adult kawasaki disease
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A previously healthy 45-year-old African-American man was admitted for acute renal failure. Recent history revealed cervical lymphadenopathy swelling, followed by apparition of fever and erythematous maculopapular rash on the abdomen and extremities. On admission, temperature was 38.6°C, blood pressure 119/79 mmHg. He had an erythematous rash on the arms and legs, with indurative oedema of the palms and soles, and bilateral non-suppurative conjunctivitis. Pharynx was injected. The patient also presented bilateral knee and ankle arthritis. Abdominal, cardiovascular and neurological examinations were normal.
Laboratory studies showed: white cells count 15.9 x 109/l, neutrophils 13 x 109/l, eosinophils 1.1 x 109/l, CRP 275 mg/l, serum creatinine 215 µmol/l. SGOT, SGPT, total bilirubin, creatine kinase and lacticodeshydrogenase were normal, but 
-glutamyl transferase was 4 times the normal level and alkaline phosphatases 1.5 times the normal level. Urinalysis showed haematuria (80 cells/µl), leucocyturia (70 cells/µl), and only traces of proteinuria.
ANCA, antinuclear antibodies, anti-dsDNA antibodies, rheumatoid factor and cryoglobulinaemia were negative. Complement was normal.
Blood and urine bacterial cultures were negative. Serological tests were negative for Rickettsia, Coxiella, Chlamydia, Mycoplasma, Salmonella, Campylobacter jejuni, Bartonella, Leptospira, Hepatitis B, C, human immunodeficiency virus (HIV), parvovirus B19 and toxoplasmosis. There were cytomegalovirus and Epstein–Barr virus-specific IgG, with no IgM.
Renal and abdominal ultrasonography showed two normal-sized kidneys, with mild hepatomegaly.
Electrocardiogram, chest X-ray and two-dimensional echocardiogram were normal.
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What is your diagnosis?
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Our patient had adult Kawasaki disease (KD). Several differential diagnoses were possible, such as drug reaction, other vasculitis (which may benefit from steroids therapy) or infectious aetiology, but extensive explorations ruled out these hypotheses. The patient fulfilled the clinical criteria of KD (fever, lymphadenopathy, polymorphous exanthema, changes of extremities, conjunctivitis and oral cavity injection) [1]. Bilateral peeling of the skin of the palms, beginning in the hyponychium, developed secondarily (Figure 1).
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He was treated with intravenous immunoglobulin. No aspirin therapy was given, because of an allergy.
Temperature decreased and he gradually improved. Creatinine was 117 µmol/l the day of discharge, with normal urinalysis; CRP was 28mg/l.
Two months after hospitalization, he had recovered normal renal function, and had not presented any complication.
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KD, also known as mucocutaneous lymph node syndrome, is a multisystemic vasculitis of the medium-sized vessels, most commonly diagnosed in young children (89% are under 5 years of age). The disease is characterized by high fever, rash, erythema and oedema of the extremities, lymphadenopathy, mucositis and non-purulent conjunctivitis. The main complication is the development of coronary aneurysms [1]. Clinical criteria for the diagnosis in children are established [1]. Treatment with intravenous immunoglobulin and aspirin has shown a significant reduction in the rate of cardiac aneurysms [2].
KD is unusual in adults. A high proportion of HIV infection is found in adults, especially in recurrent forms. Clinical features are similar in adults and children, but articular involvement and lymphadenopathy occur more frequently in adults, as well as elevated liver enzymes levels and eosinophilia. Coronary artery aneurysms seem to be less frequent than in children [3]. Treatment should be started in the acute phase of the illness, before skin desquamation, in order to obtain significant benefits from the therapy [3]. Steroid therapy is controversial, because of its potential role in coronary aneurysms formation [4].
Renal involvement is rare. Few cases of KD with acute renal failure have been described, and only in children. These cases include three tubulointerstitial nephritis [5], and one mesangial hypercellularity with interstitial infiltrate and immune deposits within glomeruli [6]. Rhabdomyolysis or heart failure are the other aetiologies of acute renal failure in this setting. A case of secondary renal artery stenosis [7] and a case of pelviureteric junction obstruction have also been described [8]. No worsening of renal function with intravenous immunoglobulin treatment has been reported in adult KD.
To our knowledge, this is the first report of acute renal failure in an adult KD. The clinical and biological findings were consistent with tubulo-interstitial involvement, which began to improve spontaneously and while treated by intravenous immunoglobulin.
This case shows that adult KD should be considered as a cause of acute renal failure with multisystemic features, underscoring the role of aspirin and early intravenous immunoglobulin treatment, and the potential worsening effect of steroid therapy.
Conflict of interest statement. None declared.
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- Newburger JW, Takahashi M, Gerber MA, et al. (2004) Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Circulation 110:2747–2771.
- Newburger JW, Takahashi M, Burns JC, et al. (1986) The treatment of Kawasaki syndrome with intravenous gamma globulin. N Engl J Med 315:341–347.[Abstract]
- Seve P, Stankovic K, Smail A, Durand DV, Marchand G, Broussolle C. (2005) Adult Kawasaki disease: report of two cases and literature review. Semin Arthritis Rheum 34:785–792.[CrossRef][Web of Science][Medline]
- Shulman ST. (2003) Is there a role for corticosteroids in Kawasaki disease? J Pediatr 142:601–603.[CrossRef][Web of Science][Medline]
- Bonany PJ, Bilkis MD, Gallo G, et al. (2002) Acute renal failure in typical Kawasaki disease. Pediatr Nephrol 17:329–331.[CrossRef][Web of Science][Medline]
- Salcedo JR, Greenberg L, Kapur S. (1988) Renal histology of mucocutaneous lymph node syndrome (Kawasaki disease). Clin Nephrol 29:47–51.[Web of Science][Medline]
- Foster BJ, Bernard C, Drummond KN. (2000) Kawasaki disease complicated by renal artery stenosis. Arch Dis Child 83:253–255.
[Abstract/Free Full Text] - Subramaniam R, Lama T, Chong CY. (2004) Pelviureteric junction obstruction as sequelae of Kawasaki disease. Pediatr Surg Int 20:553–555.[Web of Science][Medline]
Accepted in revised form: 5.12.06
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