腎臓学と治療学ジャーナル

Scleroderma Renal Crisis without Cutaneous Involvement: One Report Case in a Black African

Abstract

Eliane Mikkelsen Ranivoharisoa*,Benja Ramilitiana,Olivah Henintsoa Rakotonirainy,Harinjara Razanakoto,Fahafahantsoa Rapelanoro Rabenja,Willy Franck Harilalaina Randriamarotia

Objective: To report an atypical case of renal scleroderma crisis without cutaneous involvement and no serological manifestation. Observation: A black African, 33 years old was hospitalized for a malignant hypertension. He reached rapidly the end-stage of chronic kidney disease due to a scleroderma renal crisis. Presumption diagnostic was established by clinical signs, biological and imagery investigations. Results: The patient didn’t have personal and familial medical backgrounds a part losing 20 kilos two months before hospitalisation. In admission, he has malignant hypertension 220/140 mmHg and there was no cutaneous involvement of scleroderma. Blood tests concluded a chronic kidney disease with an estimated Glomerular Filtration Rate less than 2 ml/mn/1.73 m2. The C- reactive protein was negative and the sedimentation rate in first hour was accelerated to 119 mm. The proteinuria was 0.8 g/24 hours. The immune balance of the ribonucleic acid polymerase, the anticytoplasmic antibodies, the antinuclear antibodies were repeated more times but were negative. Imagery investigations found systemic manifestation such as lung fibrotic with atelectasia. Heart doppler found hypertension in pulmonary artery and minim pericarditis. Renal Doppler confirmed hypotrophic sizes of both kidneys with high index resistances in small vessels. Renal biopsy was not done. Six months later appeared limit digitals ulceration on both fingers. The patient was treated by angiotensin-converting-enzyme inhibitor, calcic inhibitor and underwent to a chronic hemodialysis with significant improvement mainly about the blood pressure (120/70 mmHg). Conclusion: Any malignant hypertension in scleroderma in the absence of other causes with a rapid deterioration of renal function should think first of all a scleroderma renal crisis, cutaneous involvement and serological manifestation are not always obligatory.