Diffuse systemic sclerosis with pulmonary involvement and scleroderma renal crisis: report of a rare clinical association

• Autor(a) principal: Matos-Costa, João
• Data de Publicação: 2009
• Outros Autores: Jorge, Sofia C., Barbas, Jorge, Prata, M. Martins, Rodrigues, Nelson
• Tipo de documento: Artigo
• Idioma: por
• Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
• Texto Completo: https://revista.spmi.pt/index.php/rpmi/article/view/1397
• topic: crise renal de esclerodermia; hipertensão pulmonar; hiper-reninemia; microangiopatia; scleroderma renal crisis; pulmonary hypertension,; hiperreninemia; microangiopathy

INTRODUCTION: Systemic sclerosis (SSc), an immunologic disorder of unknown aetiology, is characterized by progressive tissue fibrosis, localized or diffuse.The diffuse sub-type and prolonged course of the disease are associated with increased risk of visceral involvement, mainly of the lungs and kidneys. Pulmonary hypertension (PHT) and scleroderma renal crisis (SRC) are not mutually exclusive, but they are rarely associated; however, both are important causes of death in these patients. Mortality in SS is four times that of the general population; mainly in the first 3-5 years, the overall survival rate at 15 years is around 50%. We report a case of this rare association and present a brief review of pathophysiology and therapeutics. CASE REPORT: A 56 year-old female, caucasian, SSc diagnosed 3,5 years before, treated with vasodilators, oral anticoagulation (due to recurrent deep venous thrombosis) and corticosteroids (she had fibrosing alveolitis, and was previously treated with 13 pulses of cyclofosfamide with a favourable response).She was admitted with fever, arterial hypertension, periorbital oedema and worsening renal function. A bilateral pleural effusion was diagnosed, without alveolitis, it was a transudate. ANA e anti-Scl-70 were positive, ANCA and antiphospholipid being negative. The echocardiogram disclosed PHT and a pericardial effusion without haemodynamic compromise. Ultrasound of the kidneys was normal. Oedema and blood pressure improved on captopril; however renal function rapidly worsened with oliguria, so a renal biopsy (RB) was made and she began hemodialysis due to uremia and fluid overload. RB histology was typical of SRC. Renal function had no improvement under dialysis. In the 29th day of hospitalization she had typical signs of pulmonary embolism, and treatment began with IV heparin; latter a cardiorespiratory arrest with assystole ensued, that didn’t responded to cardiopulmonary ressuscitation. DISCUSSION: SRC is more frequently associated with diffuse SSc and anti-Scl-70. Its hallmark is an abrupt onset of severe arterial hypertension and rapidly progressive renal failure with hiperreninemia. It may coexist a microangiopathic hemolytic anemia similar to hemolyticuremic syndrome. Therapy with angiotensin converting enzyme inhibitors improved survival and prognosis, reducing the necessity of dialysis, and sometimes allowing suspension of renal replacement therapy. Overlap syndrome / renal vasculitis was considered a possibility due to poliserositis and recurrent deep venous thrombosis. Corticosteroid theraphy is likely associated with onset of SRC. There have been reports linking SRC with the onset of PHT in SSc. Some authors consider there is a common pathophysiology, namely the coexistence of renal and pulmonary thrombotic microangiopathy with myocardial dysfunction.