Case 28: 45 year old man with progressive fatigue, decreased exercise tolerance, and worsening edema
To preserve patient privacy and for didactic purposes, case descriptions and pathology reports have been anonymized and partially fictionalized. The pathology images are representative images from a mixture of similar cases.
Malvinder ParmarCase Presenter | Northern Ontario School of Medicine, Ontario, Canada | No conflict of interest reported
Helmut RennkeNephropathology Discussant | Brigham Women's Hospital, Boston, MA, USA | No conflict of interest reported
Adam SegalNephrology Discussant | Atrius, Boston, MA, USA | No conflict of interest reported
Patient is a 45-year-old man who has been feeling tired with decreased energy level for almost 1 year.
Elevated TSH was noted 3-months ago, and patient started on thyroid replacement, and statin for hyperlipidemia.
Patient continued to feel tired, experiencing decreased exercise tolerance and noted some ankle swelling for past few months. Rural clinic working diagnosis – “CHF”. At the time of consult noted 1-2+ lower extremity edema. On further questioning admits to ‘foamy urine’ for 5-6 months. No history of joint pains, stiffness, skin rashes, weight loss, fever, chills or night sweats. No history of gross hematuria or NSAID use
Medical history: Gastro-esophageal reflux disease History of squamous cell cancer Bilateral carpal tunnel release Current smoker
Medications: Pantoprazole L-thyroxine Rosvustatin
Laboratory and other data: Normal white and red blood cell count Serum creatinine 70 umol/L (eGFR 75), Serum albumin 33 g/L. HbA1C, ANA, C3, IgG, anti-HCV, HBsAg requested. Uric acid Normal at 229 (149-369 umol/L), Serum calcium 2.30 (2.10-2.55 mmol/L) ANA – Negative, C3 1.41, APLA- Negative Anti-HCV – Negative, HBsAg – Negative Serum immuno-electrophoresis: monoclonal IgG lambda band Low Total IgG 1.4 (6.00- 16.00 g/L) ESR 40 cm CRP 0.7 Urinalysis showed 3+ protein, trace of blood, 24-hr Urine protein 12.4 g/day Echocardiogram: Normal myocardial function Stress test: negative for ischemia
Pathology images pending
15% of glomeruli globally sclerosed. No increase in mesangial matrix or cellularity, no segmental scars, patent glomerular capillaries, normal walls, few glomeruli show prominent podocytes with foamy cytoplasm.
On IF: Linear staining for lambda in capillary loops. On EM: The mesangium and glomerular BM contain non-branching, randomly arranged fibrils with diameter from 9 to 12 nm, c/w amyloid.
Questions posed & summary of key discussion points
1. Any additional work up at this point? 2. What are the therapeutic options considering the potential diagnosis?
Author(s) of case summary:
Case summary pending
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