Monday, March 12, 2012

Cases from the Brigham and Women's Hospital: Is it the lupus?

Mr. HV is a 67 year old man
  • Presents with fatigue, general weakness, productive cough and a gradually rising serum creatinine.
  • Past history significant for lupus nephritis diagnosed in 1996 initially controlled on azathioprine and prednisone and off all immunosuppression roughly one year ago
  • Lupus flare in 1 year ago (elevated creatinine to 2.5 from baseline of 1.6 mg/dL and Uprot 2.4 g/24 hours) treated with MMF (switched to Myfortic because of GI intolerance with CellCept) and steroids (not biopsy-confirmed because of thrombocytopenia and uncontrolled HTN) 
  • Scr gradual increases to 3.1 mg/dL, complements have been persistently low, anti-dsDNA antibody level is 142 IU/L.
  • Worsening fatigue and generalized weakness since July.  He also reports 1 month of cough productive of yellow sputum. 
  • Has stopped Myfortic completely because of diarrhea, which is now resolved.
Past Medical History:
1. CKD secondary to immune complex GN likely due to Class IV/V lupus nephritis and possibly TMA
2. SLE/scleroderma overlap syndrome‎ - stiff hands, skin tightening, esophageal dysmotility, and Raynaud’s.
3. Hypertension
4. Mitral regurgitation: 1998 transthoracic echo (TTE) 55% ejection fraction, cardiac catheterization with no evidence of coronary artery disease – episodic congestive heart failure (CHF).
5. Granulomatous disease noted on chest CT

What's the differential diagnosis?