Granulomatosis Wegener dengan Antineutrophil Cytoplasmic Antibodies (ANCA) Negatif pada Laki-laki Usia 38 Tahun
DOI:
https://doi.org/10.56951/medicinus.v34i2.72Kata Kunci:
granulomatosis wegener, ANCA, autoimunAbstrak
Latar belakang : Granulomatosis Wegener atau granulomatosis dengan poliangiitis adalah vaskulitis nekrotikan sistemik yang menyerang pembuluh darah berukuran kecil dan sedang. Penyakit ini sering berkaitan dengan antineutrophil cytoplasmic antibodies (ANCA). Faktor pencetus terjadinya granulomatosis Wegener antara lain faktor lingkungan, infeksi, obat-obatan, dan genetik. Manifestasi klinis granulomatosis Wegener dapat berupa gejala tidak spesifik seperti nyeri otot, artralgia, demam, anoreksia, dan penurunan berat badan. Wujud kelainan kulit granulomatosis Wegener dapat berupa vaskulitis leukositoklastik, purpura, ulkus, dan nodul subkutan. Granulomatosis Wegener juga dapat menyerang berbagai organ.
Kasus : Seorang laki-laki berusia 38 tahun datang dengan keluhan muncul bercak kemerahan dan kaku pada wajah, tangan, dan kaki. Status dermatologi pada regio fasialis tampak adanya saddle nose dan pada ekstremitas superior dan inferior tampak purpura multipel diskret. Pada pemeriksaan ANCA pasien didapatkan hasil negatif. Pemeriksaan gambaran histopatologi pada lapisan epidermis menunjukkan proliferasi padat sel-sel limfosit, perivaskular infiltrat, dan sedikit sel neutrofil. Pada lapisan dermis didapatkan granuloma-granuloma yang terdiri atas banyak histiosit epiteloid dan makrofag berbuih, serta pembuluh darah yang rusak dengan infiltrasi neutrofil pada dinding serta ekstravasasi eritrosit.
Diskusi : Granulomatosis Wegener merupakan penyakit autoimun langka berupa inflamasi granulomatosis pada saluran pernapasan atas dan bawah serta vaskulitis sistemik yang berhubungan dengan ANCA. Gejala yang timbul pada granulomatosis Wegener berupa gejala ringan, namun dapat berkembang progresif dengan gambaran klinis yang lebih berat. Hasil pemeriksaan ANCA negatif tidak dapat langsung menyingkirkan diagnosis granulomatosis Wegener. Terapi awal granulomatosis Wegener berupa cyclophosphamide dan glucocorticoid yang diberikan selama 3–6 bulan. Pasien menunjukkan perbaikan klinis yang cukup bermakna dengan berkurangnya purpura pada seluruh tubuh setelah pemberian terapi selama lima minggu.
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Referensi
Comarmond C and Cacoub P. Granulomatosis with polyangiitis (Wegener): clinical aspects and treatment. Autoimmun Rev. 2014;13(11):1121-5. DOI: https://doi.org/10.1016/j.autrev.2014.08.017
Sudibyo TKAP, Mulya DP, Budiono E, Satiti S, Rosalia LP. Meningoensefalitis pada Wegener’s granulomatosis dengan ANCA negatif: laporan kasus. JPDI 2019;6(3):150-5. DOI: https://doi.org/10.7454/jpdi.v6i3.221
Lutalo PMK dan D’Cruz DP. Diagnosis and classification of granulomatosis with polyangiitiss (Wegener’s granulomatosis). J Autoimmun. 2014;48(1):94-8. DOI: https://doi.org/10.1016/j.jaut.2014.01.028
Moosig F, Lamprecht P, Gross WL. Wegener’s granulomatosis: the current view. Clin Rev Allerg Immunol. 2008;35(1):19-21. DOI: https://doi.org/10.1007/s12016-007-8067-5
Sarsat VW and Thieblemont N. Granulomatosis with polyangiitis (Wegener granulomatosis): a proteinase-3 driven disease? Joint Bone Spine. 2018;85(2):185-9. DOI: https://doi.org/10.1016/j.jbspin.2017.05.004
Guevara DL, Cerda F, Carreno MA, Piottante A, Bitar P. Update in the study of granulomatosis with polyangiitis (Wegener’s granulomatosis). Rev Chil Radiol. 2019;25(1):26-34. DOI: https://doi.org/10.4067/S0717-93082019000100026
Jokar M and Mirfeizi Z. Granulomatosis with polyangiitis (Wegener’s granulomatosis): An analysis of 59 patients. Rheum Resc. 2017;2(4):115-8. DOI: https://doi.org/10.22631/rr.2017.69997.1029
Mota A, Lotfi AS, Jamshidi AR, Najavand S. Alpha 1-antitrypsin activity is markedly decreased in Wegener’s granulomatosis. Rheumatol Int. 2014;34(4):553-8. DOI: https://doi.org/10.1007/s00296-013-2745-9
Comfere NI, Macaron NC, Gibson LE. Cutaneous manifestations of Wegener’s granulomatosis : a clinicopathologic study of 17 patients and correlation to antineutrophil cytoplasmic antibody status. J Cutan Pathol. 2007;34(1):739-47. DOI: https://doi.org/10.1111/j.1600-0560.2006.00699.x
Lima AM, Rocha SP, Ferraz FHRP, Torraca PFS, Santiago CMR. Granulomatosis with polyangiitis, a new nomenclature for Wegener’s granulomatosis - case report. An Bras Dermatol. 2015;90(3):101-3. DOI: https://doi.org/10.1590/abd1806-4841.20153455
Kim SH, Park J, Bae JH, Cho MS, Park KD, Jeong JH. ANCA-negative Wegener’s granulomatosis with multiple lower cranial nerve palsies. J Korean Med Sci. 2013;28(11):1690-6. DOI: https://doi.org/10.3346/jkms.2013.28.11.1690
Isa H, Lightman S, Luthert PJ, Rose GE, Verity DH, Taylor SRJ. Histopathological features predictive of a clinical diagnosis of ophtalmic granulomatosis with polyangiitis (GPA). Int J Clin Exp Pathol. 2012;5(7):684-9.
Graves N. Wegener granulomatosis. Proc Bayl Univ Med Cent. 2006;19(4):342-4. DOI: https://doi.org/10.1080/08998280.2006.11928198
Guillevin L dan Mahr A. Wegener’s granulomatosis. Orphan Ency. 2004;3(1):1-5.
Panupattanapong S, Stwalley DL, White AJ, Olsen MA, French AR, Hartman ME. Epidemiology and outcomes of granulomatosis with polyangiitis (GPA) in pediatric and working-age adults populations in the United States: analysis of a large national claims database. Arthr Rheum. 2018;70(12):2067-76. DOI: https://doi.org/10.1002/art.40577
Kubaisi B, Samra KA, Foster CS. Granulomatosis with polyangiitis (Wegener’s disease): an updated review of ocular disease manifestations. Intractable Rare Dis Res. 2016;5(2):61-9. DOI: https://doi.org/10.5582/irdr.2016.01014
Pagnoux C. Granulomatosis with polyangiitis. Dalam: Sinico RA, Guillevin L, penyunting. Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis. Edisi ke-1. Switzerland : Springer;2020. pp.97-120. DOI: https://doi.org/10.1007/978-3-030-02239-6_7
Popa ER dan Tervaert JWC. The relation between Staphylococcus aureus and Wegener’s granulomatosis: current knowledge and future directions. Int Med. 2003;42(9):771-80. DOI: https://doi.org/10.2169/internalmedicine.42.771
Popa ER, Stegeman CA, Kallenberg CGM, Tervaert JWC. Staphylococcus aureus and Wegener’s granulomatosis. Arthritis Res. 2002;4(2):77-9. DOI: https://doi.org/10.1186/ar392
Carnevale C, Tagle DA, Echegaray PS, Til-Perez G, Barberan MT. Head and neck manifestations of granulomatosis with polyangiitis: a retrospective analysis of 19 patients and review of the literature. Int Arch Otorhinolaryngol. 2019;23(2):165-71. DOI: https://doi.org/10.1055/s-0038-1675759
Mohammad AJ dan Segelmark M. Association of cigarette smoking with organ damage in primary systemic vasculitis. Scand J Rheumatol. 2011;40(1):51-6. DOI: https://doi.org/10.3109/03009742.2010.487839
Haubitz M, Woywodt A, Groot K, Haller H, Goebel U. Smoking habits in patients diagnosed with ANCA associated small vessel vasculitis. Ann Rheum Dis. 2005;64:1500-2. DOI: https://doi.org/10.1136/ard.2004.033191
Tarabishy AB, Schulte M, Papaliodis GN, Hoffman GS. Wegener’s granulomatosis: clinical manifestations, differential diagnosis and management of ocular and systemic disease. Surv Ophthalmol. 2010;5(5):429-44. DOI: https://doi.org/10.1016/j.survophthal.2009.12.003
Ito O, Yano T, Ito M. Can a saddle nose deformity of granulomatosis with polyangiitis be repaired? Aesthetic Plast Surg. 2017;41(6):1463-4. DOI: https://doi.org/10.1007/s00266-017-0888-x
Kesel N, Kohler D, Herich L, Laudien M, Ulrich KH, Jungel A, dkk. Cartilage destruction in granulomatosis with polyangiitis (Wegener’s granulomatosis) is mediated by human fibroblasts after transplantation into immunodeficient mice. Am J Pathol. 2012;180(5):2144-55. DOI: https://doi.org/10.1016/j.ajpath.2012.01.021
Morillo MM, Grados D, Hans DN, Mateo L, Holgado S, Olive A. Granulomatosis with polyangiitis (Wegener): description of 15 cases. Reumatol Clin. 2012;8(1):15-9. DOI: https://doi.org/10.1016/j.reumae.2011.04.005
Viswanath V, Phiske MM, Gopalani VV. Systemic sclerosis: current concepts in pathogenesis and therapeutic aspects of dermatological manifestations. Indian J Dermatol. 2013;58(4):255-68. DOI: https://doi.org/10.4103/0019-5154.113930
Franco KR, Diaz AJM, Restrepo JDH, Melendez GL. Systemic scleroderma: an approach from plastic surgery. Rev Fac Med. 2018;66(2):237-45. DOI: https://doi.org/10.15446/revfacmed.v66n2.58618
Hughes M. Early diagnosis and management of systemic sclerosis. Prescriber. 2018;29(7):27-33. DOI: https://doi.org/10.1002/psb.1689
Hazrina S dan Mustofa S. Fenomena Raynaud dan pekerja dengan paparan getaran mekanik. J Agromedicine. 2018;5(1):489-93.
Krieg T dan Takehara K. Skin disease: a cardinal feature of systemic sclerosis. Rheumatology. 2009;48(3):14-8 DOI: https://doi.org/10.1093/rheumatology/kep108
Degang Y, Nakamura K, Akama T, Ishido Y, Luo Y, Ishii N, dkk. Leprosy as a model of immunity. Future Microbiol. 2014;9(1): 43-54. DOI: https://doi.org/10.2217/fmb.13.140
Kumar B, Uprety S, Dogra S. Clinical diagnosis of leprosy. Dalam: Scollard DM, Gillis TP, penyunting. International Textbook of Leprosy. Edisi ke-1. USA: American Leprosy Mission; 2017. pp.1-24.
Junior IAR, Gresta LT, Noviello MM, Cartelle CT, Lyon S, Arantes RME. Leprosy classification methods: a comparative study in a referral center in Brazil. Int J Infect Dis. 2016;45(1):118-22. DOI: https://doi.org/10.1016/j.ijid.2016.02.018
Lastoria JC. Leprosy: review of the epidemiological, clinical, and etiopathogenic aspects. An Bras Dermatol. 2014;89(2):205-18. DOI: https://doi.org/10.1590/abd1806-4841.20142450
Salgado CG, de Brito AC, Salgado UI, Spencer JC. Leprosy. Dalam: Kang S, Amagai M, Bruckner AL, Enk AH, Margolis DJ, McMichael AJ, Orringer JS, penyunting. Fitzpatrick’s Dermatology. Edisi ke-9. United States: McGraw-Hill; 2019. pp.2892-919.
Muller K dan Lin JH. Orbital granulomatosis with polyangiitis (Wegener granulomatosis): clinical and pathologic findings. Arch Pathol Lab Med. 2014;138(8):1110-4. DOI: https://doi.org/10.5858/arpa.2013-0006-RS
Congdon D, Sherris DA, Specks U, McDonald T. Long-term follow-up of repair of externa nasal deformities in patients with Wegener’s granulomatosis. Laryngoscope. 2002;112(4):731-7. DOI: https://doi.org/10.1097/00005537-200204000-00025
Kashiwagi T, Hayama N, Fujita E, Hara K, Mii A, Masuda Y, dkk. A case of double ANCA-negative granulomatosis with polyangiitis (Wegener’s). CEN Case Rep. 2012;1:104-11. DOI: https://doi.org/10.1007/s13730-012-0023-4
Sichani MM, Hadi M, Talebi A, Khalighinejad P. Renal solid mass as a rare presentation of Wegener’s granulomatosis: a case report. Urol Case Rep. 2012;79(3):1-3. DOI: https://doi.org/10.1155/2012/793014
Leung N, Ytterberg SR, Blute ML, Lager DJ, Specks U, Fervenza FC. Wegener’s granulomatosis presenting as multiple bilateral renal masses. Nephrol Dial Trans. 2004;19(1):984-7. DOI: https://doi.org/10.1093/ndt/gfh058
Langford CA. Cyclophosphamide as induction therapy for Wegener’s granulomatosis and microscopic polyangiitis. Clin Exp Immunol. 2011;164(1):31-4. DOI: https://doi.org/10.1111/j.1365-2249.2011.04364.x
Keller ER, Schmitt WH, Gross WL. Azathioprine toxicity mimicking a relapse of Wegener’s granulomatosis. Rheumatology. 2001;40(7):831-2. DOI: https://doi.org/10.1093/rheumatology/40.7.831
Pagnoux C, Mahr A, Hamidou MA, Boffa JJ. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med. 2008;359(1):2790-803. DOI: https://doi.org/10.1056/NEJMoa0802311
Geetha D, Kallenberg C, Stone JH, Salama AD, Appel GB, Duna G, Brunetta P, Jayne D. Current therapy of granulomatosis with polyangiitis and microscopic polyangiitis: the role of rituximab. J Nephrol. 2015;28(2):17-27. DOI: https://doi.org/10.1007/s40620-014-0135-3
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