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Katastrofik antifosfolipid sendromu; nadir görülen hızlı seyirli mortalitesi yüksek antifosfolipid antikor sendromunun ciddi bir formudur. Sendrom, yaygın mikro trombozlara bağlı olarak multiorgan yetmezliğine neden olur. Bu olguda; 58 yaşında kadın hasta da alt extremite distal bölgeden başlayan ekimozlar üst bacak bölgesine ve üst extremiteye yayılan lezyonlar hızlı seyir göstererek nekrotik alanlara dönüştü. Histopatolojik incelemede dermiste intravasküler trombüsler tespit edilen hastanın laboratuvar bulgularında IgM anti-kardiyolipin antikor ve Lupus antikoagülan’ı pozitifti. Klinik seyir çok hızlı ilerleyen hastada akciğer ve böbrek tutulumu da eşlik etti. Mevcut bulgularla hastaya katastrofik antifosfolipid sendromu tanısı konuldu. Katastrofik antifosfolipid sendromu vakalarının üçte ikisin de, en sık enfeksiyonların tetiklemesine sekonder olarak gelişmektedir. Katastrofik antifosfolipid sendromu’na otoimmün hastalıklar özellikle sistemik lupus eritematozus eşlik etmektedir. Olgumuz; Sjögren sendromunun katastrofik APS etyolojisinde düşünülmesini gerektiğini vurgulamak amacıyla sunulmaktadır.

Objective: The aim of this study was to examine the relationship between alexithymia and depression, anxiety, quality of life, disease duration, and inflammatory markers in patients with Sjogren's Syndrome (SS). Material-method: The study included 168 individuals with primary SS (pSS) (145 females, 23 males) with a mean age of 51.14 ± 9.42 years. The Turkish Version of the Toronto Alexithymia Scale was used to evaluate alexithymia, the Beck Anxiety and Depression Scale to evaluate anxiety and depression, and the World Health Organization Quality of Life Scale-Short Form to evaluate the quality of life. Results: The average alexithymia of individuals with pSS was found to be 53.83 ± 12.61 points. A low and moderately significant correlation was found between alexithymia with anxiety (r:0.514, p:0.00), depression (r:0.522, p:0.00), mental (r:0.474, p:0.0) and social (r:0.486,p: 0.04) sub-parameters of quality of life and Erythrocyte sedimentation rate (ESR) values (r:0.432, p:0.03). Results: According to the results of this study, as alexithymia increases in individuals with pSS, the levels of depression, anxiety and inflammatory markers increase and the quality of life decreases in terms of mental and social aspects. Our results are twofold. According to the results of our study, considering that alexithymia is mediated by depressive symptoms in pSS, it is recommended that patients should be evaluated in detail in terms of alexithymia, depression and anxiety, and therefore these results support the need for a multidimensional approach to treatment. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Bu vaka ile kuru gözde sistemik değerlendirmenin ve hastalığın ciddiyetine göre tedavi seçiminin önemi vurgulandı. Kırksekiz yaşında kadın hastanın yaklaşık yirmi yıldır var olan ve giderek kötüleşen yanma, kaşıntı ve görme azlığı şikayetleri vardı. Schirmer I test sonuçları iki gözde 0mm/5 dakika idi. Ön segment muayenesinde iki gözde filamentöz keratit saptandı. Sjögren sendromu tanısı için yapılan lakrimal bez biyopsisi ve serumda Anti-Ro/ SSA, anti-La/ SS-B antikorları pozitifti. Hastanın tedavisine geçici punktum tıkaçları ve oral hidroksiklorokin eklendi..10 gün sonra göz kuruluğu şikayetleri geriledi ve iki göz alt punktumuna kalıcı plaklar yerleştirildi. Tüm kuru göz hastaları sistemik olarak değerlendirmeli ve tedaviden önce hastalığın ciddiyeti belirlenmelidir. Sjögren sendromuna bağlı kuru göz tedavisinde yerine koyma tedavisine ek olarak punktum tıkacı uygulaması da uygun bir seçenektir.

The importance of systemic evaluation of dry eye patients and choosing appropriate treatment based on the severity of disease were emphasized with this case. 48 years old woman complained about decreased vision, burning, itching in both eyes which got worse over the years, for about 20 years. Schirmer I test value was 0 mm/5min in both eyes. Slit lamp examination revealed filamentary keratitis in both eyes. Anti-Ro/ SSA, anti-La/ SS-B antibodies and salivary gland biopsy for Sjogren syndrome were positive. Temporary punctal occlusion and oral hydroxychloroquine were added to her treatment. After 10 days, her overall dry eye condition improved and permanent punctual plugs were inserted in both lower puctums.Dry eye patients should be evaluated systemically and severity of disease should be considered before treatment is started. Addition to topical application of artificial tears, punctal occlusion may be a proper option in dry eye patients with Sjogren syndrome. [Cukurova Med J 2013; 38(4.000): 818-822]

Sjögren syndrome is chronic systemic autoimmune syndrome especially affecting the tear and salivary glands, characterized by lymphocytic infiltration all of the exocrine glands. Attention should be paid on multi-systemic effects, potential fluid-electrolyte balance disorders, immunodepression, autoimmune hepatitis-induced liver dysfunction, aspiration risk, delayed postoperative recovery and difficult intubation in anesthesia management. In this case report we aimed at discussing the anesthetic management during cesarean section operation in a thirty nine years old patient with Sjögren syndrome. [ABSTRACT FROM AUTHOR]

Erythema annulare centrifigum is a dermatose which is frequently seen in adults. It is characterized by erythematous lesions which spread asymmetrically to periphery and have a collarette desquamation. Although infection, tumor, food allergy, drug reaction can play a role in the aetiology, most of the cases are idiopathic. A forty-nine years old, female patient presented to our clinic with erythematous lesions on both of her lower extremities. Six weeks prior to her referral, she treated with quinine for Sjogren syndrome. She had a diagnosis of granuloma annulare in her personal history. There was no significance in her family history. In dermatologic examination; annular erythematous plaques and collarette desquamation were detected on lower extremities. Histopathologic examination of the lesional biopsy specimen revealed focal spongiosis in the epidermis, dermal oedema, vascular proliferation and perivascular infiltration of lymphocytes, eosinophils and histiocytes. In the laboratory examination; blood count, liver and kidney function tests, sedimentation, C-reactive protein was normal. Rheumatoid factor was 30. Antinuclear antibody was 1/640 granular pattern. A case of erythema annulare centrifigum with Sjögren Syndrome is discussed with the other skin findings of the disease. [ABSTRACT FROM AUTHOR]