A 53-year-old man presented to the emergency department with shortness of breath, anasarca, and hypertension. He had a history of non-controlled hypertension, alcohol abuse (120 g per day), and previous hepatitis A. He had no history of chronic kidney disease and was not taking any medication.
On admission, his blood pressure was 163/103 mmHg, and pulse oximetry 96% at rest without an oxygen supply. He had reduced breath sounds on both sides and bilateral tender pitting edema up to the hip.
Blood tests revealed anemia (hemoglobin 8,2 g/dL), kidney dysfunction (creatinine 4,28 mg/dL, with no known previous creatinine), low serum albumin (2,3 g/L), low bicarbonate (11,7 mmol/L), hyperkalemia (6,25 mmo/L) and increased N-terminal prohormone of brain natriuretic peptide (> 35.000 ng/L).
The initial urinary protein-creatinine ratio (UPCR) was 2740 mg/g. Urinary sediment was remarkable for extensive dysmorphic hematuria (with > 5% acanthocytes and erythrocytic casts) (Fig. 1). The kidney ultrasound excluded relevant alterations.
A thoracic CT scan showed pericardial and pulmonary effusions.
Concerning the etiology of kidney involvement, immunoglobulin-A levels were increased (667 mg/dL), whereas the other relevant serological tests (related infectious, auto-immune, and neoplastic causes) were negative.
Kidney biopsy showed 6 glomeruli, including 1 globally sclerotic. The others revealed endocapillary hypercellularity, double-contours on glomerular basement membrane (GBM) and podocyte hypertrophy. Subendothelial deposits were found in 2 glomeruli. The interstitium had 60% of fibrosis and lipidic protein reabsorption was found in the proximal tubules. Immunofluorescence revealed on 4 glomeruli for immunoglobulin A (IgA), κ and λ light chain, and C3 deposition in the glomerular mesangium (Fig. 2). These findings endorsed the IgA Nephropathy diagnosis.
Initial medical management included endovenous diuretics with good clinical response but worsening pericardial effusion. Intercurrent urinary sepsis motivated the worsening of kidney function with the need for dialysis.
Despite the intensification of ultrafiltration and dialysis, pericardiocentesis was necessary to avoid cardiac tamponade. The cytological analysis of the pericardial effusion excluded the neoplastic and infectious causes. Pleural effusion was solved by this time.
One week after the procedure, the pericardial effusion relapsed. At the same time, the patient presented acute, pitting edema and redness of both wrists and left elbow. The joint ultrasound confirmed synovitis. No other etiology had been identified, including infectious, malignant, and autoimmune causes.
Prednisolone was started at 20 mg (0,3 mg/kg) for 5 days with remission of arthralgias. Relapse occurred at 48h after discontinuation, so it was decided to reintroduce prednisolone, considering the hypothesis of R3SPE syndrome. After starting GC therapy, there was sustained improvement in pericardial effusion and joint complaints.
The patient was discharged asymptomatic with corticosteroid weaning (20 mg). About 6 months later, GC therapy was stopped with no recurrence of the previous complaints after 2 years.
Although some findings could favor a better kidney prognosis (normal clinical ultrasound, interstitial fibrosis < 80%), the patient remained dialysis dependent because of volume overload refractory to diuretics and water intake restriction.