Could sclerodermal renal crisis be the first finding of systemic sclerosis?

Yıl 2025, Cilt: 18 Sayı: 2, 263 - 268, 15.08.2025

Damla Melemezoğlu , Kenan Turgutalp , Eylem Sercan Özgür , Yasemin Yuyucu Karabulut

https://doi.org/10.26559/mersinsbd.1486470

Öz

Systemic sclerosis is a multisystem connective tissue disease characterized by immune system activation, microvascular damage, and widespread fibrosis in the skin and internal organs. It primarily affects the skin, heart, lungs, kidneys, and gastrointestinal tract and can exists in both diffuse and limited forms. Sclerodermal renal crisis is a life-threatening complication of systemic sclerosis, characterized by new-onset malignant hypertension and progressive acute renal failure, often with associated microangiopathic haemolytic anaemia and thrombocytopenia. Thrombotic microangiopathy findings evaluated in the kidney needle biopsy material of oure case followed up with suspicion of of systemic sclerosis were found to support renal involvement. Our case shows clinicians that sclerodermal renal crisis can develop even in patients with limited or no skin involvement, and that kidney biopsy can be extremely important in the diagnosis of the disease in patients in whom lung biopsy cannot be performed. In patients with new-onset refractory hypertension, renal failure, and microangiopathic haemolytic anaemia, a comprehensive physical examination is important in this group of patients, as findings such as early sclerodactyly may provide clues that scleroderma is the underlying etiology.

Anahtar Kelimeler

Scleroderma , renal crisis , thrombotic microangiopathy

Sklerodermal renal kriz sistemik sklerozun ilk bulgusu olabilir mi?

Öz

Sistemik skleroz, immün sistem aktivasyonu, mikrovasküler hasar, deri ve iç organlarda yaygın fibrozis ile karakterize multisistemik bir bağ dokusu hastalığıdır. Öncelikle cildi, kalbi, akciğerleri, böbrekleri ve gastrointestinal sistemi etkiler ve hem yaygın hem de sınırlı formlarda bulunabilir. Sklerodermal renal kriz, sıklıkla ilişkili mikroanjiopatik hemolitik anemi ve trombositopeni ile birlikte yeni başlayan malign hipertansiyon ve ilerleyici akut böbrek yetmezliği ile karakterize, sistemik sklerozun hayatı tehdit eden bir komplikasyonudur. Sistemik skleroz şüphesi ile takipli olgumuza ait böbrek iğne biyopsi materyalinde değerlendirilen trombotik mikroanjiopati bulguları renal tutulumu destekler nitelikte bulunmuştur. Olgumuz klinisyenlere sklerodermal renal krizin cilt tutulumu sınırlı olan veya hiç olmayan hastalarda da gelişebileceğini ve akciğer biyopsisinin yapılamadığı hastalarda böbrek biyopsisinin hastalığın tanısında son derece önemli olabileceğini göstermektedir. Yeni başlayan refrakter hipertansiyonu, böbrek yetmezliği ve mikroanjiopatik hemolitik anemisi olan hastalarda, erken sklerodaktili vb. bulgular, sklerodermanın altta yatan etiyoloji olduğuna dair ipuçları sağlayabileceğinden, bu hasta grubunda kapsamlı fizik muayene önemlidir.

Anahtar Kelimeler

Skleroderma , renal kriz , trombotik mikroanjiopati

Kaynakça

• Lazaros IS. New Developments İn The Pathogenesis of Systemic Sclerosis. Autoimmunity. 2005; 38: 113-6.
• Basyal B, Finnigan NA. Scleroderma and Renal Crisis. StatPearls. 2023 Jul 4.
• Cole A, Ong VH, Denton CP. Renal Disease and Systemic Sclerosis: an Update on Scleroderma Renal Crisis. Clin Rev Allergy Immunol. 2023 Jun; 64(3): 378-391.
• Batal I, Domsıc RT, Shafer A et al. Renal Biopsy Findings Predicting Outcome İn Scleroderma Renal Crisis. Human Pathology. 2009; 40: 332-40.
• Trostle DC, Bedettı CD, Steen VD, Alsabbagh MR, Zee B, Medsger TA. Renal Vascular Histology and Morphometry İn Systemic Sclerosis: A Case-Control Autopsy Study. Arthritis Rheum. 1988; 31: 393-400.
• Denton CP, Lapadula G, Mouthon L, Muller-Ladner U. Renal Complications and Scleroderma Renal Crisis. Rheumatology. 2009; 48: iii32-iii35.
• Shapıro CB, Lerner NE, Ackad AS, Abramson RG, Steın RM. Malignant Hypertension and Uremia İn Scleroderma: Efficacy Of Nephrectomy And Hemodialysis. Clin Nephrol. 1977; 8: 321-3.
• Mouton L, Mehrenberger M, Teıxeıra L Et Al. Endothelin-1 Expression in Scleroderma Renal Crisis. Hum Pathol. 2011; 42: 95-102.
• Teıxeıra L, Mouthon L, Mahr A Et Al. Mortality and Risk Factors Of Scleroderma Renal Crisis: A French Retrospective Study Of 50 Patients. Ann Rheum Dis. 2008; 67: 110-6.
• Iudıcı M, Van Der Goes MC, Valentını G, Bıjlsma JWJ. Glucocorticoids in Systemic Sclerosis: Weighing Up The Benefits and Risks – A Systematic Review. Clin Exp Rheumatol. 2013; 31 (Suppl. 76): 157-165.
• Penn H, Denton CP: Diagnosis, Management and Prevention Of Scleroderma Renal Disease. Curr Opin Rheumatol. 2008; 20: 692-6
• Arad U, Balbır-Gurman A, Doenyasbarak K, Amıt-Vazına M, Caspı D, Elkayam O. Anti-Neutrophil Antibody Associated Vasculitis in Systemic Sclerosis. Semin Arthritis Rheum 2011; 41: 223-9.