Rapidly Progressive Glomerulonephritis With Atypical Features

January 27, 2014

A case study this week in NEJM slowly came into focus as cryoglobulinemia. It shows how the resolution may evolve only slowly, not fitting the classical picture.

Case 3-2014 - A 61-Year-Old Woman with Gastrointestinal Symptoms, Anemia, and Acute Kidney InjuryNew England Journal of Medicine, Jan. 23, 2014

A Woman with Gastrointestinal Symptoms, Anemia, and Acute Kidney InjuryNow@NEJM, January 24th, 2014

The 61-year-old woman with epigastric pain, diarrhea, and vomiting in this complex case study had rapidly progressive glomerulonephritis, low complement levels, an abnormal serum free light-chain ratio, rheumatoid factor, and a clonal B-cell population.

Biopsy showed glomerular pseudothrombi, which are pathognomic for cryoglobulinemic glomerulonephritis. She was diagnosed with type II cryoglobulinemia with acute glomerulonephritis and renal vasculitis; monoclonal B-cell population of unknown significance.

Atypically, the M component was absent on serum immunoelectrophoresis, which could be explained by the specimen’s being kept at room temperature, and precipitation of the M component. Also atypical was the gastrointestinal presentation.

She was treated initially with high-dose glucocorticoids. After serum creatinine levels rose, she was treated with plasmapheresis, cyclophosphamide, and rituximab. Serum creatinine normalized to 1.5 mg/dL, then rose to 3.1 mg/dL. Oral cyclophosphamide was stopped after her cardiac ejection fraction dropped to 39%. After six cycles of bortezomib, she improved and was maintained first on rituximab and then on prednisone.

A teaching point of this case is that even though the presentation didn’t fit the classical picture of cryoglobulinemia, and was missing some key findings such as rash, those features may evolve over time.