Unusual presentation of end-stage IgA nephropathy: about a clinical case of thrombotic microangiopathy

• Published in: Acta clinica belgica (English ed. Online) 2016-01, Vol.71 (S1), p.34
• Main Authors: David, Cristina, Goubella, Ahmed, Gastaldello, Karine, Bienfait, Lucie, Massart, Annick, Nortier, Joelle
• Format: Article
• Language: English
• Subjects: Hypertension
• ISSN: 1784-3286; 2295-3337

IgA nephropathy (IgAN) is a frequent cause of end-stage kidney disease of glomerular origin in adults. Its diagnosis is histologically confirmed by the presence of diffuse mesangial deposition of Ig A in the glomeruli. It is occasionally associated with biological signs of thrombotic microangiopathy (TMA). We report here such an unusual presentation of end-stage IgAN. A 34 yrs old man was admitted in our emergency dept with asthenia, dyspnea, vomiting and diffuse myalgia for 1 week. No recent infection was reported. One month before, a severe hypertension was detected by his general practitioner but the patient refused to take any prescribed medication. He just reported the regular intake of NSAID for recurrent headache. He also acknowledged to be a cannabis and energy drinks consumer. Three years ago, a routine dipstick performed at the occupational medicine Dept was positive for red blood cells (+) and proteins (+). Upon admission, malignant hypertension was present (225/110 mm Hg). Blood tests showed severe renal failure (urea 200 and creatinine 16 mg/dl, respectively), hemolytic anemia (hemoglobin 6.5 g/dl, LDH 412 U/L and undetectable haptoglobin), thrombocytopenia (87,000/mm3) and circulating schistocytes (1/1,000), highly suggesting of TMA. Hemodialysis sessions were immediately initiated and a renal biopsy was planned. Histologically, the pathologist described the presence of 13 glomeruli/18 completely destroyed, severe interstitial fibrosis with tubular atrophy and positive IgA immunofluorescence, associated with mucoid endarteritis, confirming the diagnosis of end-stage IgAN with TMA. Primary IgAN has diverse clinical presentations with still uncertain prognosis. Its association with TMA as a renovascular complication in advanced disease has been recently reported. In this context, malignant hypertension may worsen the progression of renal failure. In 2012, El Karoui et al. examined 128 renal biopsies from IgAN patients. Among those, 53% exhibited TMA lesions in relation with uncontrolled hypertension (71%) and with malignant hypertension (26%). These last cases had a greater percentage of interstitial fibrosis, sclerotic glomeruli, glomerular extracapillary proliferation and more TMA related endothelial lesions (J Am Soc Nephrol 2012; 23: 137). In the present case, histopathological findings related to TMA might be likely due to malignant hypertension, as no abnormality of the alternative complement pathway has been detected (in particular, C3 a