Lesões destrutivas da linha média induzidas por cocaína com ANCA positivo mimetizando a granulomatose de Wegener / Cocaine-induced midline destruction lesions with positive ANCA test mimicking Wegener's granulomatosis

O uso crônico de cocaína por inalação pode causar lesões destrutivas de linha média (LDLMIC), que podem ser difíceis de distinguir das lesões da granulomatose de Wegener (GW) nos ouvidos, nariz e garganta. Descrevemos o caso de uma paciente de 43 anos admitida com história de dois anos de obstrução nasal e rinorreia. Ela havia recebido o diagnóstico de GW há cinco meses e estava em tratamento com prednisona e ciclofosfamida. Ao exame físico apresentava perfuração de septo nasal e palato. Exames de laboratório mostraram elevação das proteínas de fase aguda e teste p-ANCA positivo. Ensaios ELISA antiproteinase 3 e mieloperoxidase foram negativos. Tomografia computadorizada (TC) dos seios paranasais mostrou destruição de septo nasal e palato, bem como sinusite maxilar bilateral. TC de tórax resultou normal. Biópsia da mucosa nasal revelou infiltrado inflamatório sem granuloma ou vasculite. Quando questionada, admitiu ser usuária de cocaína há cinco anos. Os imunossupressores foram suspensos e a paciente não mais fez uso da droga. Ela está sendo monitorada há seis meses e não desenvolveu novas lesões ou sintomas de outros órgãos. O diagnóstico diferencial em pacientes com LDLMIC pode ser desafiador. A avaliação deve incluir pesquisa de uso intranasal de cocaína. Embora o teste de ANCA não diferencie claramente o ANCA encontrado em alguns pacientes com LDLMIC daqueles em pacientes com GW, o envolvimento localizado e os achados de biópsia não típicos de vasculite granulomatosa de pequenos vasos devem ser reconhecidos como características das lesões induzidas por cocaína.

Chronic use of cocaine by inhalation may induce midline destructive lesions (CIMDL), which can sometimes be difficult to distinguish from the ear, nose and throat lesions of Wegener's Granulomatosis (WG). We describe the case of a 43-year-old female patient admitted with a two-year history of nasal obstruction and rhinorrhea. She had been diagnosed with WG for five months, being on prednisone and cyclophosphamide. On her physical examination, perforation of her nasal septum and palate was observed. Laboratory tests showed elevated acute phase proteins and a positive p-ANCA test. ELISA assays anti-proteinase 3 and myeloperoxidase were negative. The paranasal sinus computed tomography (CT) showed destruction of the nasal septum and palate, in addition to bilateral maxillary sinusitis. Chest CT was normal. Nasal mucosal biopsy revealed an inflammatory infiltrate, with neither granuloma nor vasculitis. When questioned, she admitted being a cocaine user for five years. Medical therapy and cocaine use were withdrawn. She has been followed up for six months and no other lesion or other organ symptoms occurred. Differential diagnosis in patients with midline destructive lesions can be very challenging. Evaluation should include enquiry about intranasal use of cocaine. Although ANCA testing does not clearly differentiate the ANCA found in some patients with CIMDL from those found in WG patients, the localized involvement and the biopsy findings non-characteristic of small vessel granulomatous vasculitis should be recognized as features for cocaine-induced lesions.

Two Cases of ANCA-associated Pauci-immune Glomerulonephritis with Rheumatoid Arthritis / 대한류마티스학회지

Renal involvement is one of the extra-articular manifestations found in patients with rheumatoid arthritis (RA). Membranous glomerulonephopathy, membranoproliferative glomeruonophritis, secondary amyloidosis, and focal segmental glomerulosclerosis are reported as pathologic diagnoses of renal involvement. However, reports of renal involvement in patients with RA and antineutrophil cytoplasmic autoantibody (ANCA)-associated pauci-immune glomerulonephritis are rare. Recently, we experienced two patients with RA who developed azotemia and were finally diagnosed with ANCA-associated pauci-immune glomerulonephritis. Because of the rarity of these cases, we report two cases in patients with RA with a literature review.

A Case of Rapidly Progressive Glomerulonephitis Associated with Anti-Glomerular Basement Membrane Antibody and p-Antineutrophilic Cytoplasmic Antibody / 대한신장학회잡지

Rapidly progressive glomerulonephritis(RPGN) is clinical syndrome characterized by rapid loss of renal function within several weeks to months, with histologic finding of extensive crescent formation. We report a case of RPGN associated with anti-glomerular basement membrane antibody(anti-GBM Ab) and perinuclear-antineutrophilic cytoplasmic antibody(p- ANCA), which rapidly progressed to chronic renal failure. A 44-year-old male was referred to our hospital for evaluation of pitting edema and proteinuria. Both anti-GBM Ab and p-ANCA were detected in serum. Percutaneous renal biopsy showed many crescents with some fibrinoid materials and heavy deposits of IgG. He was treated with pulse methylprednisolone, followed by oral corticosteroid and cyclophosphamide. In spite of immunosuppressive therapy, his renal function deteriorated rapidly and uremic symptoms including pulmonary edema were aggravated. He was started on hemodialysis and he has received regular hemodialysis without recovery of renal function. Further studies will be needed to determine the clinical significance of combined anti- GBM Ab and ANCA.