CPC # 2: Tuesday, October 23, 2001
Hurd Hall, The Johns Hopkins Hospital
12:00 Noon

A 37-year-old female with Wegener’s granulomatosis and progressive shortness of breath.

CC: The patient is a 37 year old white female with a history of Wegener’s granulomatosis admitted to Johns Hopkins Hospital on 1/22/01 for evaluation of shortness of breath

HPI: The patient was previously healthy until 1998 when she developed recurrent episodes of sinusitis that improved with oral antibiotics but recurred 3-4 times year.  In 3/00 she had a turbinectomy to relieve obstruction.  In 10/00 a chest X-ray and CT are reported to show a large cavitating infiltrate in the upper lobe of the left lung.  In 12/00 she developed epistaxis, ankle swelling, the lung lesion was still present, and her creatinine (which had previously been normal) was elevated to 3.0 mg/dl.  A renal biopsy demonstrated pauci-immune necrotizing vasculitis and cresenteric glomerulonephritis.  C-ANCA was positive and p-ANCA was negative.  This constellation of findings was consistent with Wegener’s granulomatosis, and she was started on Prednisone 80 mg/d and cyclophosphamide 125 mg/d.   

Beginning approximately 1/1/01, the patient noted increasing dyspnea on exertion and malaise.  She had a cough which intermittently produced yellow sputum and specks of blood.  She reported intermittent chills and sweats.  There was no headache, visual changes, chest pain, abdominal pain, diarrhea, dysuria, joint pain, rash, or bruises.  Her dyspnea limits her daily activities.  She was admitted to Hopkins for further evaluation.

Past Medical History:  Prior to the diagnosis of Wegener’s granulomatosis, patient was otherwise completely healthy. 

Past Surgical History:  Ceasarian Section (2 children)

Social History:  She grew up and currently lives in Kansas with her husband and 2 healthy children.  She does not smoke cigarettes

Family History:  No history of autoimmune disease

Allergies: NKDA

Medications:  Cyclophosphamide 125/d, Prednisone 80/d, TMP/SMX 1/d, Vitamin C and D

ROS: Only positive for difficulty rising out of chair and climbing steps due to weakness since beginning Prednisone

Physical Examination: 

• The patient is a thin, lethargic female who is slightly short of breath. 
• Vitals: 37²-38¹ C; 100/68, 84, 20, 95% saturation (room air)
• Oral: no mucositis or ulceration. 
• Pulmonary:  left upper crackles, decreased sounds at bilateral bases, no squeaks or wheezes
• Cardiac:  without significant rubs, murmurs or gallops.
• Abdominal:  demonstrates no mass, tenderness, organomegaly. A 14 cm. suprapubic well-healed scar is noted.  There is no cervical, supraclavicular, axillary or inguinal lymphadenopathy. 
• Skin: normal
• Neurologic: lethargic, fully oriented, good memory, normal and symmetric except for mild muscle weakness in the quadriceps and psoas.

Laboratory:  Na 136, K 4.9, Cl 99, HCO3 24, BUN 39, Cr 1.4, Glu 228, TP 5.5, Alb 2.6, ALT 146, AST 110, Alk Phos 123.  WBC 5.4 (88% P, 5% B, 6% L, 1% M),  Hct 27%, Plt 290, Urine 2+ protein, ESR 130 mm/hr.

Chest CT:  “ numerous thick walled cavitary lesions throughout both lungs (largest in left upper lobe=  8 cm) compatible with known Wegener’s granulomatosis.  No previous films available.  An area of air-trapping in the LLL and ill-defined infiltrate in the left mid-lung” (See image below). 

Hospital Course: 

On 1/23/01 Bronchoscopy with bronchoalveolar lavage was performed.  The airways appeared normal.  Stains for fungus, mycobacteria, pneumocystis were negative.  Cytopathology showed “predominantly alveolar macrophages, many of them are hemosiderin-laden.  A few small groups of atypical epithelial cells, consistent with reactive changes.”

On 1/25/01 she developed progressive somnolence and lethargy without focal neurologic findings.  A head CT showed “focal areas of decreased density throughout the cerebral hemispheres bilaterally affecting the basal ganglia and parietal lobes”.  A lumbar puncture revealed clear fluid with a normal opening pressure, 0 WBC, 1 RBC, protein 15 mg/dl, glucose 150 mg/dl.  WBC 1.3, Hct= 20%, Plt 230.  The patient was transferred to the Neurosciences Critical Care Unit for closer monitoring. 

What is/are the likely cause(s) of this patient’s clinical deterioration?  How was this diagnosis determined?

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