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

Abstract

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.

Keywords

• Scleroderma
• renal crisis
• thrombotic microangiopathy

Sklerodermal renal kriz sistemik sklerozun ilk bulgusu olabilir mi?

Abstract

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.

Keywords

• Skleroderma
• renal kriz
• trombotik mikroanjiopati

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