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ANCA-Associated Rapidly Progressive Glomerulonephritis in a Patient with Iga Nephropathy, Case Report

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ANCA-Associated Rapidly Progressive Glomerulonephritis in a Patient with Iga Nephropathy, Case Report Rev. Colomb. Nefrol. 2017;4(2): 200 - 209 http://www.revistanefrologia.org Case report doi: http://dx.doi.org/10.22265/acnef.4.2.272 ANCA-associated rapidly progressive glomerulonephritis in a patient with IgA nephropathy, case report Glomerulonefritis rápidamente progresiva asociada a ANCA en un paciente con nefropatía por IgA, reporte de caso Adalberto Peña Wilches1, José de Jesús Arias Agudelo2, Luis Javier Ossa Gómez3, Diana Gutiérrez González4, Jorge Enrique Echeverri Sarmiento5, Carolina Larrarte Arenas6,* 1Internist, Nephrology Resident, Universidad Militar Nueva Granada, Bogotá, Colombia 2Pathologist, Hospital Militar Central, Bogotá, Colombia 3Pathologist, Associate Professor of Pathology, Universidad Militar Nueva Granada, Bogotá, Colombia 4Pathology Resident, Universidad Militar Nueva Granada, Bogotá, Colombia 5Internist and Nephrologist, RTS Hospital Militar Central, Bogotá, Colombia 6Internist and Nephrologist, RTS Hospital Militar Central, Bogotá, Colombia Abstract IgA nephropathy is the most common glomerulonephritis, in which less than 10% of patients have a rapid decline of renal function. The histological findings of this group resemble those of vasculitis, with presence of crescents and fibrinoid necrosis. The coexistence of IgA nephropathy and neutrophil anti-cytoplasmic antibodies is infrequent, and the pathogenic role of these antibodies in IgA nephropathy is unclear. Here we describe a case of a patient with IgA nephropathy, rapidly progressive glomerulonephritis and neutrophil positive an- ti-cytoplasmic antibodies, and literature review is presented. Key words: IgA nephropathy, rapidly progressive glomerulonephritis, vasculitis, ANCA, crescents. doi: http://dx.doi.org/10.22265/acnef.4.2.272 Resumen La nefropatía por IgA es la glomerulonefritis más frecuente, en la cual menos del 10% de los pacientes cursan con deterioro rápido de la función renal. Los hallazgos histológicos de este grupo semejan los de vasculitis, con presencia de semilunas y necrosis fibrinoide. La coexistencia de nefropatía por IgA y anticuerpos anticitoplasma de neutrófilo es infrecuente y no está claro el rol patogénico de estos anti- cuerpos en la nefropatía por IgA. A continuación, se describe un caso de un paciente con nefropatía por IgA, glomerulonefritis rápidamente progresiva y anticuerpos anticitoplasma de neutrófilo positivos y se realiza una revisión de la literatura referente al tema. Palabras clave: nefropatía IgA, glomerulonefritis rápidamente progresiva, vasculitis, ANCA, semilunas. doi: http://dx.doi.org/10.22265/acnef.4.2.272 How to cite this article: Peña Wilches A, Arias Agudelo J, Ossa Gómez LJ, Gutiérrez González D, Echeverri Sarmiento JE, Larrarte Arenas C. Glomerulonefritis rápidamente progresiva asociada a ANCA en un paciente con nefropatía por IgA, reporte de caso. Rev. Colomb. Nefrol. 2017;4(2): 200-209 doi: http://dx.doi.org/10.22265/acnef.4.2.272 Correspondence Author: *Carolina Larrarte Arenas, [email protected] Received: 11-10-16 • Accepted: 06-30-17 • Published online: 07-27-17 200 Rev. Colomb. Nefrol. 2017;4(2): 200 - 209 http://www.revistanefrologia.org Introduction taste, and asthenia. He reported foamy urine, wi- thout macroscopic hematuria, edema, or any other apidly progressiveglomerulonephritis symptom. In addition, he had mixed dyslipidemia, (RPGN) is a feared manifestation of glo- hypothyroidism and grade 1 obesity. IgAN was Rmerular disease, since it involves an acce- diagnosed 12 years ago and its last assessment by lerated deterioration of renal function that marks nephrology was one year ago, finding serum crea- the prognosis of patients. Less than 10% of patients tinine of 1.48 mg/dL, glomerular filtration rate with IgA nephropathy (IgAN) have an aggressive (CKD-EPI) at 55.4 mL/min/1.73 m2, and proteinu- course, some of them with crescents in the renal ria of 250 mg/day. He received enalapril and ator- biopsy and other findings that may resemble vas- vastatin, without immunosuppressants. culitis. The presence of antineutrophil cytoplasmic antibodies (ANCA) has been reported in patients Upon assessment, he was hypertensive (155/90 with IgAN; however, the role of these antibodies mmHg), without edema or other relevant fin- in both the pathophysiology of the disease and the dings. The paraclinical testsevidenced creatinine at prognosis of patients is not clear. 4.77 mg/dL, hematuria, nephrotic proteinuria and hypoalbuminemia (Table 1). KDIGO 3 acute kid- Description of the case ney injury is considered, probably associated with upper respiratory infection, and behavior sugges- A 49-year-old male patient, with a history of IgAN, tive of rapidly progressive glomerulonephritis. He presented a 4-week clinical picture of rhinorrhea was hospitalized, and methylprednisolone 500 mg/ and hacking cough, followed by nausea, metallic day for 3 days was initiated. Peña Wilches A, Arias Agudelo J, Ossa Gómez LJ, Gutiérrez 201 González D, Echeverri Sarmiento JE, et al Rev. Colomb. Nefrol. 2017;4(2): 200 - 209 http://www.revistanefrologia.org The testsevidenced anti-nuclear antibodies (ANA), endovenous cyclophosphamide (1 gr/m2) was ini- anti-DNA negative, normal C3 and C4 and nega- tiated. A renal biopsy was performed, which preli- tive serologies for hepatitis B, C, HIV and syphi- minary report confirmed the presence of crescents. lis (Table 2). Results of ANCA and ENA were sti- Creatinine dropped slowly to 2.76 mg/dL (Figure ll pending. Considering the deterioration of renal 1). He was discharged from hospital and treatment function and the suspicion of rapidly progressive with oral steroidswas continued with pending re- glomerulonephritis (RPGN),management with sults. ANA antinuclear antibodies, ENA extractable nucleus antibodies, ANCA neutrophil cytoplasmic antibodies, IFI indirect immunofluorescence, p-ANCA perinuclear pattern, c-ANCA cytoplasmic pattern, ELISA enzyme-linked immunosorbent assay, MPO myeloperoxidase, PR3 proteinase 3, HIV human immunodeficiency virus Figure 1. Evolution of serum creatinine over time and key events during patient follow-up. 202 ANCA-associated rapidly progressive glomerulonephritis in a patient with IgA nephropathy, case report Rev. Colomb. Nefrol. 2017;4(2): 200 - 209 http://www.revistanefrologia.org Four days later, he attended an appointment with Considering the histological findings and renal an elevation of creatinine at 4.01 mg/dL and po- deterioration, despite having received a dose of sitive p-ANCAS, confirmed by ELISA (Myelope- cyclophosphamide, we decided to continue with roxidase, MPO 11.6) (Table 2). A renal biopsy was cytostatic and steroids, and initiate plasmapheresis. performed, documenting 6 glomeruli upon light Seven sessions of plasmapheresis were performed, microscopy, with mild endocapillary proliferation showing a decrease in creatinine to 2.74 mg/dL and (polymorphonuclear), cellular extracapillary proli- resulting in hospital discharge (Figure 1). feration in more than half of the glomeruli, inflam- matory cells (lymphocytes and plasma cells) in the The patient changed his country of residence and interstitium, and mild tubular atrophy. No mesan- therefore his treating doctor. Information was recei- gial or vascular changes were evident (Figure 2). ved from the patient about the continuity of cyclo- It was not possible to evaluate immunofluorescence phosphamide and steroids. One month after hospital due to lack of material, but immunohistochemistry discharge, tests were performed to document sta- was performed confirming IgA deposits in the me- bility of renal function, but persistence of hypoal- sangium (Figure 3). No electronic microscopy was buminemia, proteinuria and hematuria (creatinine available. The patient was considered to have AN- 2.39 mg/dL, BUN 36 mg/dL, albumin 2.9 gr/dL and CA-associated RPGN, without extrarenal systemic urinalysis with 3+ of proteins and hematuria). manifestations of vasculitis. Figura 2. Image of kidney biopsy by light microscopy Peña Wilches A, Arias Agudelo J, Ossa Gómez LJ, Gutiérrez 203 González D, Echeverri Sarmiento JE, et al Rev. Colomb. Nefrol. 2017;4(2): 200 - 209 http://www.revistanefrologia.org (100x), positive for presence of IgA in the mesangium (ocher color) Figure 3. Image of kidney biopsy, immunohistochemistry. Literature review ported8-10. Although the clinical behavior of patients with IgAN and ANCA has been evaluated, the pa- IgAN is the most frequent primary glomerulo- thogenic role thereof is still unclear, since it could nephritis, characterized by mesangial deposit of be the hyperactivity of antibody producing cells or IgA. The rate of progression of the disease is varia- another concomitant disease3. It is even less clear ble; however, at 25 years of follow-up, 30-50% of if the presence of IgG or IgAANCA has any diffe- patients progress to stage 5 chronic kidney disease rential effect. (CKD5).2 Rapid progression has been reported in less than 10% of patients, with histological findings Several reports and case series of patients with suggestive of vasculitis such as crescents, fibrinoid IgAN and positive ANCA have been published11-16. necrosis, and arteriolar lesion in some of them3. Bantis et al. evaluated the behavior of 8 patients with crescentic IgAN and positive ANCA and com- The coexistence of antineutrophil cytoplasmic an- pared them with 26 patients with more than 10 % of tibodies (ANCA) and IgAN is rare, being reported glomeruli with crescents, but negative ANCA. All in 0.2-2 % of cases4. ANCA, mainly ANCA-mye- patients with ANCA presented rapidly progressive loperoxidase
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