Abstract
Sjögren Sendromu SjS öncelikle gözyaşı ve tükrük bezleri olmak üzere tüm ekzokrin bezlerin lenfositit infiltrasyonu ile karakterize kronik, sistemik ve otoimmün bir hastalıktır. Kserostomi ve kseroftalmi tablosu hastalığın ana belirtisi olmakla ile birlikte kas iskelet, pulmoner, gastrointestinal, hematolojik, dermatolojik, böbrek ve sinir sistemine uzanan geniş bir yelpazede bozukluğa neden olur. Lökositoklastik vaskülit SjS de sık görülen kutanöz vaskülit tipidir.Granülamatöz polianjitis GPA nedeni belli olmayan, genellikle küçük veya orta boy damarları tuttuğu gibi sistemik tutulum da yapabilen, nekrotizan granülomatöz tipte bir vaskülittir. GPA, alt-üst solunum yolları, böbrek ve diğer organları tutabilmektedir. Özellikle deri lezyonları önemli bir tutulum şeklidir. Hastalık seyrinde optik sinir iskemisine bağlı retroorbital psödotümör gelişebilmektedir. Burada SjS tanısı ile takip edilirken lökositoklastik vaskülit gelişen beyin görüntülemesinde psödotümör orbita saptanmışolan 48 yaşında bir kadın hasta tartışılmıştır. SjS ve GPA birlikteliğine çok sık rastlanılmamaktadır. Bu olgu, bu nadir birlikteliğe değinilmek için sunulmuştur. Psödotümör orbita ile prezente olan hastalarda GPA‘nın ayırıcı tanıda akılda tutulması, erken teşhis, tedavi ve komplikasyonların önlenmesi açısından önemlidir
References
- Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of sys- temic vasculitides. Proposal of an International Consensus Conference. Arthritis Rheum 1994; 37: 187-92.
- Seo P, Stone JH. The antineutrophil cytoplasmic anti- body-associated vasculitides. Am J Med 2004; 117:39.
- Macias JD, Wackym PA, McCabe BF. Early diagnosis of oto- logic Wegener’s granulomatosis using the serologic marker C-ANCA. Ann Otol Rhinol Laryngol 1993; 102:337-41.
- Hoffman GS, Kerr GS, Leavitt RY,et al. Wegener granu- lomatosis: an analysis of 158 patients. Ann Intern Med 1992;116:488-98.
- Schmidt J, Pulido JS, Matteson EL. Ocular manifestations of systemic disease: antineutrophil cytoplasmic antibody-as- sociated vasculitis. Curr Opin Ophthalmol 2011; 22:489.
- Yeşildağ A, Adanır E, Köroğlu M, ve ark.Orbital psödotü- mör ve hipofiz makroadenom birlikteliği: MRG bulguları. SDÜ Tıp Fak Derg 2004; 11: 18-20.
- Leavitt RY, Fauci AS, Bloch DA, et al. The American Collage of Rheumatology 1990 criteria for theclassification of We- gener’s granulomatosis. Arthritis Rheum 1990; 33: 1101-7.
- Carol AL, Gary SH. Wegener’s granulomatosis. Thorax 1999; 54: 629-37.
- Fraser RS, Miller NL, Colman N,et al. Diagnosis of diseases of thechest. 4th ed. USA: Saunders Company; 1999. 1489- 532.
- Düzgün N. Wegener Granülomatozu. Romatoloji Kitabı. Ankara: Ankara Üniversitesi; 2014: 348-52.
- Nölle B, Specks U, Lüdemann J,et al.Anticytoplasmatic au- toantibodies: their immunodiagnostic value in Wegener’s granulomatosis. Ann Intern Med 1989; 111: 28-40.
- DeRemee R. Pulmonary vasculitis. Fishman’s Pulmonary Diseases and Disorders New-York: Mac-Graw-Hill Com- panies 1998:1357-74.
- Ramos-Casals M, Tzioufas AG, Font J. Primary Sjögren's syndrome: new clinical and therapeutic concepts. Ann Rheum Dis 2005; 64:347.
- Vitali C, Bombardieri S, Jonsson R, et al; European Study Group on Classification Criteria for Sjogren's Syndro- me.Classification criteria for Sjogren’s syndrome: a re- vised versionof the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002;61:554–8.
- Ramos-Casals M, Anaya JM, Garcia-Carrasco M, et al. Cutaneous vasculitis in primary Sjogren’s syndrome: clas- sification and clinical significance of 52 patients. Medicine 2004;83:96–106.
- Garcia-Carrasco M, Ramos-Casals M, Rosas J, et al. Pri- mary Sjogren syndrome: clinical and immunologic disease patterns in a cohort of 400 patients. Medicine (Baltimore) 2002;81: 270–80.
- Scofield RH. Vasculitis in Sjogren’s syndrome. Curr Rheu- matol Rep 2011;13:482–8.
- Prajs K, Bobrowska-Snarska D, SkaΠa M,et al. Polyarteritis nodosa and Sjogren's syndrome: overlap syndrome. Rheu- matol Int 2012;32:4019–21.
- Guillot X, Solau-Gervais E, Coulon A,et al.Sjogren's sy- ndrome with ANCA-associated crescentic extramembra- nousglomerulonephritis. Joint Bone Spine 2009;76:188–9
- Guellec D, Cornec-Le Gall E, Groh M, et al. ANCA-associ- ated vasculitis in patients with primary Sjögren's syndrome: detailed analysis of 7 new cases and systematic literature re- view. Autoimmun Rev. 2015;14:742-50.
Abstract
Sjögren Syndrome SjS is a chronic, systemic and autoimmune disease characterized by lymphocytic infiltration of all exocrine glands, primarily lacrimal and salivary glands. Beside Xerostomy and xerophthalmia being the main symptoms of the disease, it causes a wide range of disorders including musculoskeletal, pulmonary, gastrointestinal, hematological, dermatological, kidney and nervous systems. Leukocytoclastic vasculitis is a common type of cutaneous vasculitis in SjS.Granulomatous polyangiitis GPA is a necrotizing granulomatous type of vasculitis, withunclear etiology, usually capable of systemic involvement as well as affecting small or medium-sized vessels. GPA can involve lower and upper airways, kidneys and other organs. Skin lesions are an important form of involvement.Retroorbital pseudotumor due to optic nerve ischemia may develop during the disease course. In our case we discussed a 48-year-old female patient diagnosed with pseudotumor orbita and developed leukocytoclastic vasculitis during her follow-up period. The association of SjS and GPA is not very common. This case is presented to address this rare association.In patients presenting with pseudotumor orbita, it is important to keep GPA in mind in the differential diagnosis, early diagnosis, treatment and prevention of complications
References
- Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of sys- temic vasculitides. Proposal of an International Consensus Conference. Arthritis Rheum 1994; 37: 187-92.
- Seo P, Stone JH. The antineutrophil cytoplasmic anti- body-associated vasculitides. Am J Med 2004; 117:39.
- Macias JD, Wackym PA, McCabe BF. Early diagnosis of oto- logic Wegener’s granulomatosis using the serologic marker C-ANCA. Ann Otol Rhinol Laryngol 1993; 102:337-41.
- Hoffman GS, Kerr GS, Leavitt RY,et al. Wegener granu- lomatosis: an analysis of 158 patients. Ann Intern Med 1992;116:488-98.
- Schmidt J, Pulido JS, Matteson EL. Ocular manifestations of systemic disease: antineutrophil cytoplasmic antibody-as- sociated vasculitis. Curr Opin Ophthalmol 2011; 22:489.
- Yeşildağ A, Adanır E, Köroğlu M, ve ark.Orbital psödotü- mör ve hipofiz makroadenom birlikteliği: MRG bulguları. SDÜ Tıp Fak Derg 2004; 11: 18-20.
- Leavitt RY, Fauci AS, Bloch DA, et al. The American Collage of Rheumatology 1990 criteria for theclassification of We- gener’s granulomatosis. Arthritis Rheum 1990; 33: 1101-7.
- Carol AL, Gary SH. Wegener’s granulomatosis. Thorax 1999; 54: 629-37.
- Fraser RS, Miller NL, Colman N,et al. Diagnosis of diseases of thechest. 4th ed. USA: Saunders Company; 1999. 1489- 532.
- Düzgün N. Wegener Granülomatozu. Romatoloji Kitabı. Ankara: Ankara Üniversitesi; 2014: 348-52.
- Nölle B, Specks U, Lüdemann J,et al.Anticytoplasmatic au- toantibodies: their immunodiagnostic value in Wegener’s granulomatosis. Ann Intern Med 1989; 111: 28-40.
- DeRemee R. Pulmonary vasculitis. Fishman’s Pulmonary Diseases and Disorders New-York: Mac-Graw-Hill Com- panies 1998:1357-74.
- Ramos-Casals M, Tzioufas AG, Font J. Primary Sjögren's syndrome: new clinical and therapeutic concepts. Ann Rheum Dis 2005; 64:347.
- Vitali C, Bombardieri S, Jonsson R, et al; European Study Group on Classification Criteria for Sjogren's Syndro- me.Classification criteria for Sjogren’s syndrome: a re- vised versionof the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002;61:554–8.
- Ramos-Casals M, Anaya JM, Garcia-Carrasco M, et al. Cutaneous vasculitis in primary Sjogren’s syndrome: clas- sification and clinical significance of 52 patients. Medicine 2004;83:96–106.
- Garcia-Carrasco M, Ramos-Casals M, Rosas J, et al. Pri- mary Sjogren syndrome: clinical and immunologic disease patterns in a cohort of 400 patients. Medicine (Baltimore) 2002;81: 270–80.
- Scofield RH. Vasculitis in Sjogren’s syndrome. Curr Rheu- matol Rep 2011;13:482–8.
- Prajs K, Bobrowska-Snarska D, SkaΠa M,et al. Polyarteritis nodosa and Sjogren's syndrome: overlap syndrome. Rheu- matol Int 2012;32:4019–21.
- Guillot X, Solau-Gervais E, Coulon A,et al.Sjogren's sy- ndrome with ANCA-associated crescentic extramembra- nousglomerulonephritis. Joint Bone Spine 2009;76:188–9
- Guellec D, Cornec-Le Gall E, Groh M, et al. ANCA-associ- ated vasculitis in patients with primary Sjögren's syndrome: detailed analysis of 7 new cases and systematic literature re- view. Autoimmun Rev. 2015;14:742-50.
Details
| Primary Language | Turkish |
|---|---|
| Journal Section | Case Report |
| Authors | |
| Publication Date | December 1, 2019 |
| Published in Issue | Year 2019 Volume: 29 Issue: 4 |
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