Letter to the Editor
ANCA Vasculitis in a Patient With Systemic Sclerosis
Vasculitis ANCA positivo en un paciente con esclerosis sistémica
Pablo Antonio Zurita Pradaa,
, Sonia Martín Rodríguezb, Claudia Lia Urrego Laurína, Manuel Heras Benitoc, Álvaro Molina Ordasc, Ana Saiz Gonzalezd
Corresponding author
a Servicio de Reumatología, Complejo Hospitalario de Segovia, Segovia, Spain
b Servicio de Medicina Interna, Complejo Hospitalario de Segovia, Segovia, Spain
c Servicio de Nefrología, Complejo Hospitalario de Segovia, Segovia, Spain
d Servicio de Anatomía Patológica, Hospital Universitario Ramón y Cajal, Madrid, Spain
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However, because they share similar clinical manifestations but a different etiopathogenic basis, the need to distinguish between inflammatory vasculitic damage or scleroderma vasculopathy can sometimes be a real dilemma that determines the clinical prognosis and therapeutic regimen for these patients.</p><p id="par0010" class="elsevierStylePara elsevierViewall">We report the case of a 63-year-old woman with a history of hypertension treated with inhibitors of the angiotensin converting enzyme (ACE) inhibitors and dyslipidemia. She was diagnosed 16 years before with limited cutaneous SS based on Raynaud's phenomenon, sclerodactyly, telangiectasias, esophageal dysfunction, and mild pulmonary fibrosis of both lung bases, with no functional impact on blood gases, with negative anticentromere, Jo1, Scl-70, and PM-1 antibodies. She had not received specific treatment except for oral corticosteroids for an acute episode of pleuropericarditis, presenting a secondary cardiac arrest one year prior to admission. She came to the clinic due to fatigue and malaise of a month's duration, without fever and no other data of interest. Physical examination showed BP 150/90<span class="elsevierStyleHsp" style=""></span>mmHg and typical stigmas of scleroderma, mucocutaneous pallor, small laterocervical lymphadenopathy, fine crackles, and mild edema of the lower extremities, without evidence of heart failure. Laboratory tests showed anemia with hemoglobin of 10.8<span class="elsevierStyleHsp" style=""></span>g/dl, (creatinine of 2.2<span class="elsevierStyleHsp" style=""></span>mg/dl, with no known impairment of renal function, having been previously normal) CRP of 48<span class="elsevierStyleHsp" style=""></span>mg/dl, and an ESR of 58<span class="elsevierStyleHsp" style=""></span>mm/h. Immunological tests showed positive ANA 1/160 with a nucleolar pattern, ENA (Ro, La, RNP, Sm) and negative anti-GBM antibody, normal C3 and C4 and positive 1/640ANCA p-ANCA, with positive anti-MPO ELISA 28<span class="elsevierStyleHsp" style=""></span>U/ml (negative 0–7) and negative anti-PR3. The urinalysis showed microhematuria and mild proteinuria of 21<span class="elsevierStyleHsp" style=""></span>mg/dl (0.6<span class="elsevierStyleHsp" style=""></span>g/d). The chest X-ray was normal, except for mild fibrosis in both lung bases. We performed a kidney biopsy. We appreciated 18 glomeruli, 6 of them completely sclerotic. Of the remaining 12, 7 had severe extracapillary proliferation forming circumferential crescents (<a class="elsevierStyleCrossRefs" href="#fig0005">Figs. 1 and 2</a>) that engulfed the glomerular tuft, and in 5 of these glomeruli were showed images of vascular plume fibrinoid necrosis (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>). The rest of the glomeruli showed no lesions. Direct immunofluorescence was negative for IgG, IgA, IgM, and C3. In the interstitium there was marked inflammatory infiltrates and vasculitis lesions were visible, especially in small and medium caliber vessels. This supported pauci-immune extracapillary glomerulonephritis. Treatment was initiated with 3 induction boluses of methylprednisolone 1<span class="elsevierStyleHsp" style=""></span>g/day and thereafter iv prednisone at 1<span class="elsevierStyleHsp" style=""></span>mg/kg/day orally with subsequent reduction, along with monthly cyclophosphamide iv boluses at a dose of 0.5<span class="elsevierStyleHsp" style=""></span>mg/m<span class="elsevierStyleSup">2</span>. The initial response was poor, with deterioration of the renal function reaching creatinine levels of 5.4<span class="elsevierStyleHsp" style=""></span>mg/dl. At 6 months, after 5 cycles of cyclophosphamide, creatinine stabilized at 2<span class="elsevierStyleHsp" style=""></span>mg/dl and negative ANCA, so we continued treatment with azathioprine 100<span class="elsevierStyleHsp" style=""></span>mg/day. At 2 months it had to be replaced due to moderate hepatotoxicity, using MMF 500<span class="elsevierStyleHsp" style=""></span>mg/12<span class="elsevierStyleHsp" style=""></span>h, finally suspendedshe has not undergone any more hospitalizations. However, residual impairment of renal function with creatinine of 2<span class="elsevierStyleHsp" style=""></span>mg/dl persists.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Renal involvement in SS is characterized by obliterative vascular disease of the cortical arteries due to endothelial damage, vascular wall thickening, and renal hypoperfusion juxtaglomerular hyperplasia, leading to a state of hyperreninemia. The most serious clinical consequences, the scleroderma crisis (SC), appears in 10%–15% of patients with SS, being more frequent in diffuse forms and after the 3 or 4 first years from disease onset. Some risk factors have been associated as risk factors, such as anemia, the use of corticosteroids (>15<span class="elsevierStyleHsp" style=""></span>mg prednisone), black ethnicity, and topoisomerase III antibodies, but not anticentromere or anti-Scl70 antibodies. It can classically be accompanied by malignant hypertension, acute kidney injury, and microangiopathic hemolytic anemia. Although the prognosis has improved with treatment with ACE inhibitors, estimated mortality is still approximately 19% per year and 40% at 5 years.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">However, our case presented an atypical nephropathy in the biopsy. ANCA associated vasculitis (AAV) is the most frequent type of vasculitis seen in SS, with large and medium caliber vasculitis being even more exceptional.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> The incidence of ANCA in patients with SS varies between 7% and 13%, but clinical impact is rareç; unknown pathophysiological mechanism trigger the development of vasculitis, even on previous sclerotic lesions. Patients with MPO-ANCA vasculitis are more susceptible to present clinical features and PR3-ANCA rarely have clinical consequences, with involvement of upper airway or granulomatous lesions being more common. Rho et al., in a review of 50 AAV cases associated to SS<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> describe female predominance (84%), with a mean age of 57 years and a mean duration of SS of 9.5 years before the onset of vasculitis. It has been related to the previous use of <span class="elsevierStyleSmallCaps">d</span>-penicillamine. Renal involvement is the most frequent (82%), followed by gastrointestinal (58%) and alveolar hemorrhage (22%). Only 28% present hypertension. Distal ischemia or cutaneous vasculitis are less common manifestations. No differences were found between diffuse and limited forms, with anti-Scl70 being clearly prevalent (70%) compared to anti-centromere antibodies (14%). Seventy-two percent of cases are associated with anti-MPO compared with 24% of anti-PR-3 positive patients, most with a p-ANCA pattern by IF. High titers of p-ANCA appear to correlate with a rapid deterioration of renal function.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Arad et al. added prognostic information such as improvement in 51% of cases and evolution to ESRD or death in 14% and 34%, respectively. Mortality occurs mostly during the first year from the onset of vasculitis, mainly due to secondary causes as infectious disease and pulmonary hemorrhage. Mortality contrasts with the SC, which is mainly caused by late complications of chronic dialysis.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Success in treating the etiopathogenesis is less of a focusthan avoiding the likely irreversible damage. Suggestive manifestations for differential diagnosis are SC hypertension (88%), thrombocytopenia and microangiopathic hemolytic anemia (50%), typically appearing early in the SC and with rare presentations of elevation of the RFA or fever. If associated to pulmonary vasculitis, severe worsening of anemia without evidence of hemolysis with elevated RFA may be key to the interpretation of a radiological pattern of alveolar hemorrhage on pulmonary fibrosis or cardiac edema.</p><p id="par0030" class="elsevierStylePara elsevierViewall">There are no specific recommendations for AAV treatment associated to SS. The common trend is to follow the standard recommendations of AAV treatment with steroids and cyclophosphamide boluses. High-dose corticosteroid therapy has proven effective and renal crisis did not appear in the case described. Plasma exchange has also been used, and more recently rituximab, with favorable<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> results.</p><p id="par0035" class="elsevierStylePara elsevierViewall">The association of AAV in patients with SS is uncommon. The most common clinical presentation is often long standing renal p-ANCA in positive Scl70 patients. It constitutes a differential diagnostic challenge with a scleroderma renal crisis and especially in unusual normotensive presentations. Immunosuppressive therapy is effective, but with significant mortality or progression to ESRD.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara">Please, cite this article as: Zurita Prada PA, et al. Vasculitis ANCA positivo en un paciente con esclerosis sistémica. Reumatol Clin. 2013;<span class="elsevierStyleBold">9</span>:72–74.</p>" ] ] "multimedia" => array:3 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1281 "Ancho" => 1707 "Tamanyo" => 492269 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Hematoxylin and eosin (×40) showing a circumferential crescent.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1281 "Ancho" => 1707 "Tamanyo" => 432613 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">PAS positive slide ×40 with a circumferential crescent.</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Fig. 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 1292 "Ancho" => 1709 "Tamanyo" => 658431 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Hematoxylin and eosin (×20) with fibrinoid arteriole necrosis.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:6 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Scleroderma renal crisis: patient characteristics and long-term outcomes" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "H. Penn" 1 => "A.J. Howie" 2 => "E.J. Kingdon" 3 => "C.C. Bunn" 4 => "R.J. Stratton" 5 => "C.M. Black" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1093/qjmed/hcm052" "Revista" => array:6 [ "tituloSerie" => "QJM" "fecha" => "2007" "volumen" => "100" "paginaInicial" => "485" "paginaFinal" => "494" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/17601770" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0010" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Vasculitis en systemic sclerosis" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "L. Kao" 1 => "C. Weyand" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1155/2010/385938" "Revista" => array:5 [ "tituloSerie" => "Int J Rheumatol" "fecha" => "2010" "volumen" => "2010" "paginaInicial" => "385938" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/20976300" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0015" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Scleroderma associated with ANCA-associated vasculitis" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "Y.H. Rho" 1 => "S.J. Choi" 2 => "Y.H. Lee" 3 => "J.D. Ji" 4 => "G.G. Song" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1007/s00296-005-0011-5" "Revista" => array:6 [ "tituloSerie" => "Rheumatology International" "fecha" => "2006" "volumen" => "26" "paginaInicial" => "369" "paginaFinal" => "375" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/16032390" "web" => "Medline" ] ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0020" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Antineutrophil cytoplasmic autoantibodies in 6 patients with renal failure and systemic sclerosis" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "H. Endo" 1 => "T. Hosono" 2 => "H. Kondo" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Journal of Rheumatology" "fecha" => "1994" "volumen" => "21" "paginaInicial" => "864" "paginaFinal" => "870" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/8064727" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0025" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Anti-neutrophil antibody associated vasculitis en systemic sclerosis" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "U. Arad" 1 => "A. Balbir-Gurman" 2 => "K. Doenyas-Barak" 3 => "M. Amit-Vazina" 4 => "D. Caspi" 5 => "O. Elkayam" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.semarthrit.2010.11.001" "Revista" => array:6 [ "tituloSerie" => "Seminars in Arthritis and Rheumatism" "fecha" => "2011" "volumen" => "41" "paginaInicial" => "223" "paginaFinal" => "229" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/21277620" "web" => "Medline" ] ] ] ] ] ] ] ] 5 => array:3 [ "identificador" => "bib0030" "etiqueta" => "6" "referencia" => array:1 [ 0 => array:1 [ "referenciaCompleta" => "Omair MA, Mohamed N, Johnson SR, Ahmad Z, Lee P. ANCA-associated vasculitis in systemic sclerosis report of 3 cases. Rheumatol Int. In press 2012. <a class="elsevierStyleInterRef" href="http://dx.doi.org/10.1007/s00296-011-2359-z">http://dx.doi.org/10.1007/s00296-011-2359-z</a>." ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/21735743/0000000900000001/v1_201305061542/S2173574312001578/v1_201305061542/en/main.assets" "Apartado" => array:4 [ "identificador" => "8400" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Letters to the Editor" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/21735743/0000000900000001/v1_201305061542/S2173574312001578/v1_201305061542/en/main.pdf?idApp=UINPBA00004M&text.app=https://reumatologiaclinica.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2173574312001578?idApp=UINPBA00004M" ]
| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 October | 61 | 34 | 95 |
| 2024 September | 75 | 19 | 94 |
| 2024 August | 73 | 29 | 102 |
| 2024 July | 104 | 36 | 140 |
| 2024 June | 64 | 38 | 102 |
| 2024 May | 65 | 24 | 89 |
| 2024 April | 61 | 15 | 76 |
| 2024 March | 89 | 34 | 123 |
| 2024 February | 51 | 25 | 76 |
| 2024 January | 41 | 31 | 72 |
| 2023 December | 56 | 21 | 77 |
| 2023 November | 58 | 35 | 93 |
| 2023 October | 70 | 31 | 101 |
| 2023 September | 65 | 35 | 100 |
| 2023 August | 44 | 18 | 62 |
| 2023 July | 33 | 24 | 57 |
| 2023 June | 43 | 25 | 68 |
| 2023 May | 49 | 9 | 58 |
| 2023 April | 38 | 15 | 53 |
| 2023 March | 56 | 23 | 79 |
| 2023 February | 44 | 28 | 72 |
| 2023 January | 35 | 16 | 51 |
| 2022 December | 46 | 34 | 80 |
| 2022 November | 70 | 36 | 106 |
| 2022 October | 52 | 35 | 87 |
| 2022 September | 41 | 29 | 70 |
| 2022 August | 48 | 33 | 81 |
| 2022 July | 41 | 48 | 89 |
| 2022 June | 58 | 43 | 101 |
| 2022 May | 59 | 40 | 99 |
| 2022 April | 57 | 45 | 102 |
| 2022 March | 60 | 75 | 135 |
| 2022 February | 57 | 36 | 93 |
| 2022 January | 45 | 26 | 71 |
| 2021 December | 36 | 37 | 73 |
| 2021 November | 45 | 38 | 83 |
| 2021 October | 62 | 54 | 116 |
| 2021 September | 57 | 39 | 96 |
| 2021 August | 43 | 36 | 79 |
| 2021 July | 45 | 23 | 68 |
| 2021 June | 63 | 37 | 100 |
| 2021 May | 57 | 36 | 93 |
| 2021 April | 105 | 86 | 191 |
| 2021 March | 77 | 34 | 111 |
| 2021 February | 52 | 22 | 74 |
| 2021 January | 36 | 25 | 61 |
| 2020 December | 31 | 13 | 44 |
| 2020 November | 21 | 22 | 43 |
| 2020 October | 24 | 15 | 39 |
| 2020 September | 45 | 28 | 73 |
| 2020 August | 55 | 16 | 71 |
| 2020 July | 22 | 13 | 35 |
| 2020 June | 45 | 19 | 64 |
| 2020 May | 43 | 16 | 59 |
| 2020 April | 30 | 11 | 41 |
| 2020 March | 15 | 8 | 23 |
| 2020 February | 2 | 0 | 2 |
| 2019 January | 1 | 0 | 1 |
| 2018 May | 5 | 0 | 5 |
| 2018 April | 73 | 10 | 83 |
| 2018 March | 100 | 6 | 106 |
| 2018 February | 46 | 3 | 49 |
| 2018 January | 70 | 4 | 74 |
| 2017 December | 51 | 8 | 59 |
| 2017 November | 52 | 3 | 55 |
| 2017 October | 30 | 3 | 33 |
| 2017 September | 46 | 6 | 52 |
| 2017 August | 62 | 11 | 73 |
| 2017 July | 63 | 7 | 70 |
| 2017 June | 82 | 5 | 87 |
| 2017 May | 81 | 6 | 87 |
| 2017 April | 57 | 7 | 64 |
| 2017 March | 68 | 4 | 72 |
| 2017 February | 41 | 4 | 45 |
| 2017 January | 27 | 2 | 29 |
| 2016 December | 76 | 14 | 90 |
| 2016 November | 86 | 6 | 92 |
| 2016 October | 114 | 9 | 123 |
| 2016 September | 113 | 11 | 124 |
| 2016 August | 109 | 8 | 117 |
| 2016 July | 51 | 5 | 56 |
| 2016 June | 0 | 8 | 8 |
| 2016 May | 0 | 7 | 7 |
| 2016 April | 0 | 5 | 5 |
| 2016 March | 0 | 5 | 5 |
| 2016 February | 0 | 16 | 16 |
| 2015 December | 2 | 0 | 2 |
| 2015 October | 1 | 0 | 1 |
| 2015 September | 0 | 15 | 15 |
| 2015 August | 2 | 0 | 2 |
| 2015 July | 42 | 6 | 48 |
| 2015 June | 55 | 9 | 64 |
| 2015 May | 117 | 12 | 129 |
| 2015 April | 90 | 9 | 99 |
| 2015 March | 76 | 4 | 80 |
| 2015 February | 78 | 13 | 91 |
| 2015 January | 96 | 11 | 107 |
| 2014 December | 74 | 13 | 87 |
| 2014 November | 80 | 7 | 87 |
| 2014 October | 66 | 8 | 74 |
| 2014 September | 69 | 5 | 74 |
| 2014 August | 56 | 9 | 65 |
| 2014 July | 85 | 12 | 97 |
| 2014 June | 106 | 9 | 115 |
| 2014 May | 94 | 10 | 104 |
| 2014 April | 70 | 10 | 80 |
| 2014 March | 72 | 11 | 83 |
| 2014 February | 57 | 12 | 69 |
| 2014 January | 65 | 15 | 80 |
| 2013 December | 59 | 9 | 68 |
| 2013 November | 40 | 5 | 45 |
| 2013 October | 63 | 15 | 78 |
| 2013 September | 66 | 11 | 77 |
| 2013 August | 63 | 16 | 79 |
| 2013 July | 70 | 6 | 76 |
| 2013 June | 59 | 7 | 66 |
| 2013 May | 57 | 11 | 68 |
| 2013 April | 79 | 18 | 97 |
| 2013 March | 75 | 32 | 107 |
| 2013 February | 27 | 9 | 36 |
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