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Vol. 13. Issue 2.
Pages 267-274 (March - April 2007)
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Vol. 13. Issue 2.
Pages 267-274 (March - April 2007)
Caso Clínico / Clinical Case
Open Access
Vasculite e padrão de panbronquiolite difusa no lúpus eritematoso sistémico – Caso clínico
Vasculitis and diffuse panbronchiolitis-like in systemic lupus erythematosus – Case report
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Lina Carvalho1, Sara Freitas2
1 Professora de Anatomia Patológica. Faculdade de Medicina da Universidade de Coimbra
2 Pneumologista Eventual. Hospitais da Universidade de Coimbra
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Resumo

O compromisso visceral pelo lúpus eritematoso sistémico (LES) estende-se para além do rim e da pele. Lesões pleuropulmonares são reconhecidas e as for-mas de destruição alveolar difusa e hemorragia alveolar são as mais difíceis de controlar. O compromisso pulmonar na evolução clínica do LES difere nas crianças e nos adultos, tanto nos padrões morfológicos como nas apresentações clínicas, dependendo da imunoincompe-tência do doente e do tratamento instituído.

Um rapaz de 16 anos apresentou um quadro clínico de astenia, cansaço e pequenos gânglios linfáticos cer-vicais bilaterais e não dolorosos, entendido como in-fecção pelo EBV, com serologia concordante (IgG e IgM de EBV e EBNA positivos). Os sintomas persistiram durante oito meses e progressivamente insta-lou-se eritema nasal e malar, discreto e descamativo e também febre persistente, dispneia e estertores basais à auscultação. Foram efectuadas biópsia de um gânglio linfático cervical e biópsia cirúrgica pulmonar.

Observouse hiperplasia folicular no gânglio linfático e ausência de células LMP1 (EBV) positivas. Na biópsia pulmonar eram evidentes fenómenos de bronquiolite e vasculite à custa de células macrofágicas identificadas pelo marcador CD68. Os macrófagos dissociavam as paredes vasculares e bronquiolares e também estavam presentes nos septos interalveolares peribroncovascu-lares e nos espaços alveolares, observando-se assim um padrão de panbronquiolite difusa e vasculite. Não se identificaram células LMP1 (EBV) positivas.

O padrão pulmonar micronodular bilateral observado na TAC resolveu com corticoterapia. O diagnóstico de LES foi confirmado pela positividade dos anticorpos ANA, anti-dsDNA, anti-nDNA e anti-histonas.

Este é o primeiro caso divulgado na literatura médica de compromisso pulmonar sob a forma de vasculite e padão de panbronquiolite difusa como primeira ma-nifestação clínica do lúpus eritematosos sistémico.

Rev Port Pneumol 2007; XIII (2): 267-274

Palavras-chave:
LES
bronquiolite
vasculite
panbronquiolite difusa
Abstract

Visceral involvement in systemic lupus erythematosus (SLE) extends beyond renal and cutaneous management. Pleuro-pulmonary lesions have been recognised and diffuse alveolar damage and hemorrhage are the most difficult patterns to control. Pulmonary compromise in clinical evolution of SLE differs from children to adults, both in morphological patterns and in clinical presentation, depending on immunocompetence and the treatment prescribed.

A 16-year-old boy presented asthenia, malaise and bilateral cervical painless adenopathies understood as EBV infection as serological EBV IgG, IgM and EBNA were positive. The symptoms persisted for eight months when discrete erythematous and desquamative nasal and malar rash expressed together with persistent fever, dispnoea and bibasilar crackles. Lymph node and pulmonary biopsi-ses were performed.

Lymph node presented follicular hyperplasia and LMP1 (EBV) immunostaining was negative. In lung biopsy bronchovascular lesions were consistent with vasculitis and bronchiolitis due to intense macrophage infiltration, validated with CD68 antibody and intra-alveolar macrophages were also present with septal compromise; LMP1 (EBV) positive cells were not visualized.

The lung pattern seen in CAT as diffuse micronodules all over the lung parenchyme resolved after corticos-teroid therapy. The diagnosis of SLE was confirmed by ANA, anti-dsDNA, anti-nDNA and anti-histones positivity.

To the best of our knowledge this is the first reported case of pulmonary SLE involvement with vasculitis and diffuse panbronchiolitis – like pattern as the first clinical sign of the disease.

Rev Port Pneumol 2007; XIII (2): 267-274

Key-words:
SLE
bronchiolitis
vasculitis
diffuse pan-bronchiolitis
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Bibliografia
[1.]
S. Sahn.
The Pleura.
Am Rev Respir Dis, (1988), pp. 184-234
[2.]
A. Mattay, I. Schwarz, L. Petty.
Pulmonary manifestations of systemic lupus erythematosus: review of twelve cases with acute lupus pneumonitis.
Medicine, 54 (1974), pp. 397-409
[3.]
J. Myers, A. Katzenstein.
Microangiitis in lupus-induced pulmonary hemorrhage.
Am J Clin Pahol, 85 (1986), pp. 553-556
[4.]
R. Gammon, T. Bridges, H. Al-Nezir.
Bronchiolitis obliterans organizing pneumonia associated with systemic lupus erythematosus.
Chest, 202 (1992), pp. 1171-1174
[5.]
H. Eisenberg, E. Dubois, R. Sherwin, O. Balchum.
Diffuse interstitial lung disease in systemic lupus erythema-tosus.
Ann Intern Med, 79 (1973), pp. 37-45
[6.]
A. Askari, P. Thompson, C. Barnes.
Sarcoidosis: atypical presentation associated with features of systemic lupus erythematosus.
J Rheumatol, 15 (1988), pp. 1578-1579
[7.]
A. Sandrini, M. Balter, K. Chapman.
Diffuse panbronchiolitis in a caucasian man in Canada.
Can Respir J, 10 (2003), pp. 449-451
[8.]
V. Poletti, M. Chilosi, R. Trisolini, A. Cancellieri, M. Zompatori, L. Agli, M. Boaron, W. Schulte, D. Theegarten, J. Guzman, U. Costabel.
Idiopathic bronchiolitis mimi-cking diffuse panbronchiolitis.
Sarcoidosis Vasc Diffuse Lung Dis, 20 (2003), pp. 62-68
[9.]
M. Fischer, W. Rush, M. Rosado-de-Christenson, E. Gol-dstein, S. Tomski, J. Wempe, W. Travis.
Diffuse pan-bronchiolitis: histologic diagnosis in unsuspected cases involving north American residents of Asian descent.
Arch Pathol Lab Med, 122 (1998), pp. 156-160
[10.]
V. Poletti, M. Patelli, G. Poletti, T. Bertanti, L. Spiga.
Diffuse panbronchiolitis observed in an Italian male.
Sar-coidois, 9 (1992), pp. 67-69
[11.]
G. Mueller, A. Pickoff.
Pediatric lymphocytic interstitial pneumonitis in an HIV-negative child with pulmonary Epstein-Barr vírus infection.
Pediatric Pulmonology, 36 (2003), pp. 447-449
[12.]
M. Shimakage, T. Sasagawa, M. Kimura, T. Shimakage, S. Seto, K. Kodama, H. Sakamoto.
Expression of Eps-tein-Barr virus in Langerhans’ cell histiocytosis.
Human Pathology, 35 (2004), pp. 862-868
[13.]
X. Hu, E. Selbs, S. Drexler.
An 18-year-old man with persistent cough and bilateral lower lung infiltration –Eps-tein-Barr virus-positive lymphoproliferative disorder consistent with lymphomatoid granulomatosis.
Archives of Pathology & Laboratory Medicine, 130 (2006), pp. 44-46
[14.]
G. Stopyra, H. Multhaupt, L. Alexa, M. Husson, J. Stern, M. Warhol.
Epstein-Barr virus-associated adult respiratory distress syndrome in a patient with AIDS – case report and review.
Modern Pathology, 12 (1999), pp. 984-989
[15.]
S. Sriskandan, L. Labrecque, J. Schofield.
Diffuse pneumonia associated with infectious mononucleosis – detection of Epstein-Barr virus in lung tissue by in situ hybridi-zation.
Clinical Infectious Disease, 22 (1996), pp. 578-579
[16.]
A. Pfleger, E. Eber, H. Popper, M. Zach.
Chronic interstitial lung disease due to Epstein-Barr virus infection in two infants.
European Respiratory Journal, 15 (2000), pp. 803-806
[17.]
T. Ankermann, A. Claviez, H. Wagner, M. Krams, F. Rie-del.
Chronic interstitial lung disease with lung fibrosis in a girl – uncommon sequelae of Epstein-Barr virus infection.
Pediatric Pulmonology, 35 (2003), pp. 234-238
[18.]
S. Lok, J. Stewart, B. Kelly, P. Hashleton, J. Egan.
Eps-tein-Barr virus and wild p53 in idiopathic pulmonary fibrosis.
Respiratory Medicine, 95 (2001), pp. 787-791
[19.]
H. Adachi, I. Saito, M. Horiuchi, J. Ishii, Y. Nagata, F. Mi-zuno, H. Nakamura, H. Yagyu, K. Takahashi, T. Matsuoka.
Infection of human lung fibroblasts with Epstein-Barr virus causes increased IL-1 beta and bFGF production.
Experimental Lung Research, 27 (2001), pp. 157-158
[20.]
M. Yamasaki, R. Kitamura, S. Kusano, H. Eda, S. Sato, M. Okawa-Takatsuji, S. Aotsuka, K. Yanagi.
Elevated immunoglobulin G antibodies to the proline-rich amino-terminal region of Epstein-Barr virus nuclear antigen-2 in sera from patients with systemic connective tissue diseases and from a subgroup of Sjogren’s syndrome patients with pulmonary involvements.
Clinical & Experimental Immunology, 139 (2005), pp. 558-568
[21.]
P. Chu, L. Cerilli, Y. Chen, S. Mills, L. Weiss.
Epstein-Barr vírus plays no role in the tumorigenesis of small-cell carci-noma of the lung.
Modern Pathology, 17 (2004), pp. 158-164
[22.]
M. Felizardo, M. Aguiar, A. Mendes, D. Moniz, R. Sotto-Mayor, A. Almeida.
Collagen vascular diseases and lung – characterization of the outpatients with intertitial lung disease.
Rev Port Pneumol, 11 (2005), pp. 26-27
[23.]
V. Lilleby, T. Aalokken, B. Johansen, O. Forre.
Pulmonary involvement in patients with childhood-onset systemic lupus erythematosus.
Clin Exp Rheumatol, 24 (2006), pp. 203-208
[24.]
R. Singh, W. Huang, Y. Menon, L. Espinoza.
Shrinking lung syndrome in systemic lupus erythematosus and Sjo-gren´ s syndrome.
J Clin Rheumatol, 8 (2002), pp. 340-345
[25.]
P. Ferguson, M. Weinberger.
Shrinking lung syndrome in a 14-year-old boy with systemic lupus erythemato-sus.
Pediatric Pulmonology, 41 (2006), pp. 194-197
[26.]
Y. Makino, S. Ogawa, U. Ohto.
CT appearance of diffuse alveolar hemorrhage in a patient with systemic lupus erythematosus.
Acta Radiologica, 34 (1993), pp. 634-635
[27.]
D. Boumpas, H. Austin, B. Fessler, J. Balow, J. Klippel, M. Lockshin.
Systemic lupus erythematosus – emerging concepts.
Ann Intern Med, 122 (1995), pp. 940-950
[28.]
D. Huang, A. Yang, Y. Tsai, B. Lin, C. Tsai, S. Wang.
Acute massive pulmonary haemorrhage, pulmonary embolism and deep vein thrombosis in a patient with systemic lupus erythe-matosus and varicella.
Respir Med, 90 (1996), pp. 239-241
[29.]
M. Liu, F. Chen, T. Hsiue, C. Liu.
Disseminated zygomycosis simulating cerebrovascular disease and pulmonary alveolar hemorrhage in a patient with systemic lupus erythematosus.
Clinical Rheumatology, 19 (2000), pp. 11-14
[30.]
M. Hughson, Z. He, J. Heneger, R. McMurray.
Alveolar hemorrhage and renal microangiopathy in systemic lupus erythematosus.
Archives of Pathology & Laboratory Medicine, 125 (2001), pp. 475-483
[31.]
A. Santos-Ocampo, B. Mandell, B. Fessler.
Alveolar hemorrhage in systemic lupus erythematosus.
Chest, 120 (2001), pp. 323-327
[32.]
G. Zandman-Goddard, M. Ehrenfeld, Y. Levy, S. Tal.
Diffuse alveolar hemorrhage in systemic lupus erythe-matosus.
IMAJ, 4 (2002), pp. 470-474
[33.]
K. Kaneko, M. Matsuda, Y. Sekijima, W. Hosoda, T. Gono, K. Hoshi, H. Shimojo, S. Ikeda.
Acute respiratory distress syndrome due to systemic lupus erythematosus with hemaphagocytic syndrome – an autopsy report.
Clinical Rheumatology, 24 (2005), pp. 158-161
[34.]
J. Kreindler, D. Ellis, A. Vats, G. Kurland, S. Ranganathan, M. Moritz.
Infantile systemic lupus erythematosus presenting with pulmonary hemorrhage.
Pediatric Nephrology, 20 (2005), pp. 522-525
[35.]
M. Beresford, A. Cleary, J. Sills, J. Couriel, J. Davidson.
Cardio-pulmonary involvement in juvenile systemic lupus erythematosus.
Lupus, 14 (2005), pp. 152-158
[36.]
A. Traynor, T. Corbridge, A. Eagan, W. Barr, Q. Liu, Y. Oya-ma, R. Burt.
Prevalence and reversibility of pulmonary dysfunction in refractory systemic lupus – improvement correlates with disease remission following hematopoietic stem cell transplantation.
Chest, 127 (2005), pp. 1680-1689
[37.]
M. Irfan, Zubairia, S. Husain.
Bronchiolitis obliterans organizing pneumonia associated with cytomegalovirus infection in a patient with systemic lupus erythematosus.
JPMA, 54 (2004), pp. 328-330
[38.]
G. Contreras, D. Green, V. Pardo, D. Schultz, J. Bourgoignie.
Systemic lupus erythematosus in two adults with human immunodeficiency virus infection.
Am J kidney Dis, 28 (1996), pp. 292-295
[39.]
E. Kocakoc, S. Ozgocmen, A. Kiris, L. Ozcakar, Y. Bo-ztosun, N. Yildirim.
An overwhelming pulmonary fungus ball in a systemic lupus erythematosus patient.
Zeits-chrift fur Rheumatologie, 62 (2003), pp. 570-573
[40.]
A. Al-Abad, D. Cabral, S. Sanatani, G. Sandor, M. Seear, R. Petty, P. Malleson.
Echocardiography and pulmonary function testing in childhood onset systemic lupus erythe-matosus.
Lupus, 10 (2001), pp. 32-37
[41.]
L. Chumbley, E. Harrison, R. DeRemee.
Allergic granulomatosis and angiitis (Churg – Strauss syndrome) – report and analysis of 30 cases.
Mayo Clin Proc, 52 (1977), pp. 477-485
[42.]
O. Erdogan, A. Oner, G. Demircin, M. Bulbul, L. Memis, C. Uner, N. Kiper.
A boy with consecutive development of SLE and Wegener granulomatosis.
Pediatric Nephrology, 19 (2004), pp. 438-441
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