Medical Grand Rounds Clinical Vignette

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Medical Grand RoundsClinical Vignette

• Aditya Mattoo ,MD
• February 27, 2008

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Chief Complaint

• 57 year old white man presents with complaints of
  malaise, intermittent low grade fevers, nasal
  congestion, and cough for 6 months.

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History of Present Illness

• The patient was in his usual state of health when
  he first began to experience sinus congestion and
  pain 6 months prior to admission associated with
  a low grade fever of 100.0, remitting
  intermittently until the date of presentation.
  He also noticed worsening generalized malaise and
  weakness over this period.
• Two months prior to admission he noted a dry
  cough that progressed to a non-bloody cough
  productive of whitish sputum.
• His primary care physician performed blood work
  which demonstrated a creatinine of 9.0 from a
  creatinine of 1.2 approximately one year prior.
  He was instructed by his PCP to be admitted to
  NYUMC for further workup.

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History

• Past Medical History Hypertension, dyslipidemia.
• Past Surgical History Appendectomy
• Social History No tobacco, EtOH or illicit drug
  use.
• Family History Father, deceased, secondary to MI
  at age 62.
• Allergies None
• Medications HCTZ, lisinopril and simvastatin.
• ROS no dysuria, hematuria, or hemoptysis.

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Physical Exam

• GENERAL Well-appearing white male in no acute
  distress.
• VS Temp 99.6. HR 77 bpm. BP 144/81 mmHg. RR
  17. O2 saturation of 97 on room air.
• HEENT Maxillary sinus tenderness to percussion.
  Nasal mucosal erythema with whitish discharge.
• PULMONARY Fine crackles at base of right lung.
• The remainder of the exam was otherwise normal.

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Laboratory Values

• CBC
• WBC 4.6.
• Hb/Hct 8.9/26.8.
• MCV 86.
• Plts 456.
• BMP
• BUN/Cr 96/9.3
• Ca 7.4 Phos 5.5
• LFTs normal.
• Complements
• C3 was 63 (nl 60-175 mg/dL), C4 was 7 (nl 16-37
  mg/dL)
• Urinalysis large blood, RBC casts etc.

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Radiography

• CXR Cavitary lesion seen in right lower lobe
  with small right sided pleural effusion.
• CT Paranasal Sinus Mucosal thickening of
  maxillary, sphenoid and ethmoid sinuses greater
  on the right side with air fluid levels
  suggestive of acute sinusitis.

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CT Chest
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Preliminary Diagnosis

• Systemic vasculitis with Rapidly Progressing
  Glomerulonephritis.

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Renal Biopsy

• A renal biopsy was performed on hospital day 2 of
  the right kidney.

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Renal Pathology
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Hospital Course

• The patient was initiated on pulse dose steroids
  (methylprednisolone 500mg daily) and then given
  oral predisone on hospital day 4.
• His creatinine continued to rise to 11.3, so he
  was started on dialysis on hospital day 4.
• The patients blood work demonstrated, cANCA and
  anti-proteinase 3 (anti-PR3).
• Oral cyclophosphamide therapy was initiated.

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Final Diagnosis

• Wegeners Granulomatosis with Rapidly Progressive
  Glomerulonephritis

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Follow-up

• The patient is now two months into treatment with
  symptomatic improvement.
• He is still currently on hemodialysis, although
  his renal function is improving.
• There are plans to wean the patient off of
  dialysis in the upcoming months.

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