nephropathy were the first clinical manifestations of systemic lupus erythematosus (SLE). Cardiovascular support and immunosuppressive treatment allowed a long lasting remission. Design and Method. A 45-year-old white man, with history of hypertension and smoking, was admitted to the hospital because of severe dyspnea and chest pain. He reported a three months history of progressive shortness of breath and fatigue. Clinical examination suggested an acute heart failure with elevated blood pressure (169/110 mmHg) and tachycardia (120 bpm). Laboratory findings showed increased troponin T (127.3 pg/mL), creatinine (2.5mg/dL), GOT (1355 UI/mL), GPT (1310 UI/mL), ferritin (4322 ng/mL) and CRP (2.58 mg/dL) levels. Urinalysis showed hemoglobinuria (3+) and proteinuria (0.3 g/L). Transthoracic Doppler echocardiography revealed a severe systolic dysfunction (LVEF: 15-20%) with normal valves and minimal pericardial effusion. Cardiac MRI detected two left ventricular areas of intramural myocardial delayed enhancement whereas coronary angiography was unremarkable. He was treated with intravenous inotropes, nitroglycerine and diuretics with improvement of the clinical status. Additional laboratory analyses showed anemia (Hb 10.8 g/dL), thrombocytopenia (73 x 103/ μL), positivity of antinuclear (homogeneous pattern - 1:2560 titer), anti-double stranded DNA (164 IU/ml) and anti-Sm/RNP antibodies, and low complement levels (C3 0.449 g/l, C4 0.036 g/L). During hospitalization, the patient presented fever without any evidence of infection. A CT scan revealed pleural-pericardial effusion, splenomegaly and diffuse lymphadenopathy. Results. According to clinical features and laboratory findings, a diagnosis of SLE was made and the patient was treated with methylprednisolone (1 g/day for 3 days) followed by oral prednisone (1 mg/kg/day) with remission of fever and improvement of hematological values. However, urinalysis confirmed hemoglobinuria (3+) and proteinuria (0.63 g/24h). These findings lead us to perform renal biopsy. Histology revealed moderate mesangial sclerosis with segmental proliferation and indirect immunofluorescence detected IgA (+++), C3 (+++) and IgG deposits (+). According to current criteria, histology was defined as IgA nephropathy (HAAS classification, class III). Prednisolone dose was tapered up to 25 mg/day in 4 months and hydroxychloroquine (200 mg twice a day) was added. After 6 months’ follow up we switched hydroxychloroquine to mycophenolate mofetil (1000 mg twice a day). Transthoracic Doppler echocardiography revealed improvement of left ventricular function (until 55% of ejection fraction) and urinalysis showed regression of proteinuria and hemoglobinuria. Nowadays, the patient is in good clinical conditions with a maintenance dose of prednisone (2.5 mg/day) and mycophenolate mofetil. Conclusions. This case reports and uncommon SLE onset with the association of acute cardiac failure and atypical renal involvement.

ACUTE CARDIAC FAILURE AND IGA NEPHROPATHY: AN UNCOMMON SLE ONSET

O. Magnani;M. Pellecchio;E. Penza;G. Murdaca;S. Negrini;F. Puppo
2016

Abstract

nephropathy were the first clinical manifestations of systemic lupus erythematosus (SLE). Cardiovascular support and immunosuppressive treatment allowed a long lasting remission. Design and Method. A 45-year-old white man, with history of hypertension and smoking, was admitted to the hospital because of severe dyspnea and chest pain. He reported a three months history of progressive shortness of breath and fatigue. Clinical examination suggested an acute heart failure with elevated blood pressure (169/110 mmHg) and tachycardia (120 bpm). Laboratory findings showed increased troponin T (127.3 pg/mL), creatinine (2.5mg/dL), GOT (1355 UI/mL), GPT (1310 UI/mL), ferritin (4322 ng/mL) and CRP (2.58 mg/dL) levels. Urinalysis showed hemoglobinuria (3+) and proteinuria (0.3 g/L). Transthoracic Doppler echocardiography revealed a severe systolic dysfunction (LVEF: 15-20%) with normal valves and minimal pericardial effusion. Cardiac MRI detected two left ventricular areas of intramural myocardial delayed enhancement whereas coronary angiography was unremarkable. He was treated with intravenous inotropes, nitroglycerine and diuretics with improvement of the clinical status. Additional laboratory analyses showed anemia (Hb 10.8 g/dL), thrombocytopenia (73 x 103/ μL), positivity of antinuclear (homogeneous pattern - 1:2560 titer), anti-double stranded DNA (164 IU/ml) and anti-Sm/RNP antibodies, and low complement levels (C3 0.449 g/l, C4 0.036 g/L). During hospitalization, the patient presented fever without any evidence of infection. A CT scan revealed pleural-pericardial effusion, splenomegaly and diffuse lymphadenopathy. Results. According to clinical features and laboratory findings, a diagnosis of SLE was made and the patient was treated with methylprednisolone (1 g/day for 3 days) followed by oral prednisone (1 mg/kg/day) with remission of fever and improvement of hematological values. However, urinalysis confirmed hemoglobinuria (3+) and proteinuria (0.63 g/24h). These findings lead us to perform renal biopsy. Histology revealed moderate mesangial sclerosis with segmental proliferation and indirect immunofluorescence detected IgA (+++), C3 (+++) and IgG deposits (+). According to current criteria, histology was defined as IgA nephropathy (HAAS classification, class III). Prednisolone dose was tapered up to 25 mg/day in 4 months and hydroxychloroquine (200 mg twice a day) was added. After 6 months’ follow up we switched hydroxychloroquine to mycophenolate mofetil (1000 mg twice a day). Transthoracic Doppler echocardiography revealed improvement of left ventricular function (until 55% of ejection fraction) and urinalysis showed regression of proteinuria and hemoglobinuria. Nowadays, the patient is in good clinical conditions with a maintenance dose of prednisone (2.5 mg/day) and mycophenolate mofetil. Conclusions. This case reports and uncommon SLE onset with the association of acute cardiac failure and atypical renal involvement.
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11567/889557
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