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Wegener’s Granulomatosis with Rapidly Progressive Glomerulonephritis

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					Medical Grand Rounds
    Clinical Vignette



   Aditya Mattoo ,MD
      February 27, 2008
             Chief Complaint

   57 year old white man presents with
    complaints of malaise, intermittent low
    grade fevers, nasal congestion, and cough
    for 6 months.
         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.
                        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.
                    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.
                   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.
                  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.
CT Chest
         Preliminary Diagnosis

   Systemic vasculitis with Rapidly
    Progressing Glomerulonephritis.
                Renal Biopsy

   A renal biopsy was performed on hospital
    day 2 of the right kidney.
Renal Pathology
               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 patient’s blood work demonstrated, cANCA+
    and anti-proteinase 3 + (anti-PR3+).
   Oral cyclophosphamide therapy was initiated.
             Final Diagnosis

   Wegener’s Granulomatosis with Rapidly
    Progressive Glomerulonephritis
                 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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