A difficult diagnosis

Abstract

Ajda Rowshani and colleagues (March 6, p 782)1Rowshani AT Schot LJ ten Berge IJM c-ANCA as a serological pitfall.Lancet. 2004; 363: 782Summary Full Text Full Text PDF PubMed Scopus (29) Google Scholar describe a patient with midline destructive disease and c-ANCA (classic antineutrophil cytoplasmic autoantibodies) but no Wegener's granulomatosis. In their Case Report, the authors state that the referring doctors suspected the patient to have progressive Wegener's granulomatosis and that cocaine use had been overlooked. This statement is incorrect. Moreover, as the referring doctors, we have serious doubts about whether the reported case is indeed a serological pitfall. The patient was referred to us in July, 1999. In December, 1998, he was admitted to hospital elsewhere with a perforation of the nasal septum. Before being admitted he had had arthralgias, purpura, and conjunctivitis for 6 months. Proteinase-3 (PR3)-ANCA was detectable, and the results of his kidney biopsy indicated a necrotising crescentic glomerulonephritis and a mild mesangioproliferative glomerulonephritis with IgA, IgG, and C3 deposits. After treatment with cyclophosphamide and steroids, the patient developed fever, for which he was referred to us. On presentation, the ANCA test was negative and X-sinus disclosed sinusitis with air fluid levels. Postnasal drip was present and cultures were positive for Candida albicans, Pseudomonas aeruginosa, and Staphylococcus aureus. Despite treatment with antibiotics and fluconazole, perforation of his nasal septum increased and he developed a defect in the velum palatum. Treatment with cyclophosphamide was replaced with methotrexate. His dose of steroids was temporally increased and fever disappeared. After discharge the patient again developed a fever, however, for which he was readmitted in November, 1999. Despite treatment with ceftazidime results of tests indicated the presence of P aeruginosa for which he was now treated with tobramycin. The patient confessed he had used cocaine in the past but denied current use. However, a urine test was positive. The individual's temperature returned to normal within 48 h during daily endonasal dissolution of his sinus and discontinuation of cocaine use. The dose of steroids prescribed was reduced and the patient referred to a hospital in Amsterdam close to where he lives. Rowshani and colleagues suggest that our patient had destructive midline disease due to cocaine use after an initial presentation with Henoch Schönlein purpura. Cocaine use can indeed mimic Wegener's granulomatosis. However, in such cases, elastase ANCA are nearly always detectable and are suggestive of cocaine-induced midline destructive disease.2Trimarchi M Gregorini G Facchetti F et al.Cocaine-induced midline destructive lesions: clinical, radiographic histopathologic, and serologic features and their differentiation from Wegener granulomatosis.Medicine. 2001; 80: 391-404Crossref PubMed Scopus (109) Google Scholar, 3Wiesner O, Russell KA, Lee AS, et al. ANCA reacting with human neutrophil Elastase: a diagnostic marker for cocaine induced midline destructive lesions but not autoimmune vasculitis. Arthritis Rheum (in press).Google Scholar There are, however, also patients who have used cocaine who have no elastase ANCA, but who have PR3-ANCA, such as the patient described. In these individuals, diagnosis is more difficult and a case of biopsy-proven PR3-ANCA-associated Wegener's granulomatosis in a cocaine user has been documented.3Wiesner O, Russell KA, Lee AS, et al. ANCA reacting with human neutrophil Elastase: a diagnostic marker for cocaine induced midline destructive lesions but not autoimmune vasculitis. Arthritis Rheum (in press).Google Scholar Because elastase ANCA was repeatedly tested for4Cohen Tervaert JW Mulder L Stegeman C et al.Occurrence of autoantibodies to human leucocyte elastase in Wegener's granulomatosis and other inflammatory disorders.Ann Rheum Dis. 1993; 52: 115-120Crossref PubMed Scopus (114) Google Scholar but not detected in our patient and because his PR3-ANCA-associated midline destructive disease was associated with arthralgias, purpura, and necrotising crescentic glomerulonephritis, we believe Wegener's granulomatosis cannot be ruled out. The presence of IgA-associated mesangioproliferative disease does not prove that he had Henoch Schönlein purpura. In fact, the combination of Wegener's granulomatosis and IgA nephropathy has been described in up to 20% of patients with Wegener's granulomatosis.5Andrassy K Waldherr R Erb A Ritz E De novo glomerulonephritis in patients during remission from Wegener's granulomatosis.Clin Nephrol. 1992; 38: 295-298PubMed Google Scholar