CASE 1

History:

This 43-year-old woman noticed the insidious onset of a red rash that involved the cheeks of her face and bridge of her nose. Otherwise, she was in good health. You perform a physical examination and find that the examination is entirely normal, except for the rash. You refer the patient to a dermatologist who performs a biopsy of the affected skin. Half the biopsy is sent for routine light microscopy and half for immunofluorescence microscopy.

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Questions:

1. What is the diagnosis.
2. Should an antinuclear antibody test be performed on this patient?
3. How likely is it that this patient will have systemic symptoms from her disease?
4. What is your interpretation of the light and immunofluorescent microscopic findings (what is her disease process)? How specific are these findings?

CASE 2

History:

This patient is a 16-year-old girl who tells you that she was feeling well until about six months ago. At that time she noticed increasing fatigue and her ankles periodically swelled up. Her local physician did a routine physical examination and noted 2+ pitting edema of her ankles, but no other findings. A routine urinalysis showed 4+ proteinuria and red blood cells present, but no RBC casts. Further laboratory workup revealed that she had an elevated erythrocyte sedimentation rate (ESR), slight anemia with Hgb 11.2, slight leukopenia with total WBC count 3500, and normal serum chemistries except for a slightly decreased serum albumin (2.9 gm/dl). Her antinuclear antibody (ANA) was positive at 1:256 (normal <1:20). Her serum C3 complement level was decreased. You order a nephrology consult, and the nephrologist performs a renal biopsy.

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Questions:

1. What is the diagnosis?
2. Why is a renal biopsy being done? What difference would the results of this biopsy make?
3. What would a test for extractable nuclear antigens (ENA)--double stranded DNA--show? Should you also do an LE cell test?
4. What other organ system involvement would you expect?
5. What immunologic mechanisms are operative in this disease?

CASE 3

History:

This 40-year-old woman has a long-standing history of diabetes mellitus, type I, which resulted in renal failure three years ago. Because of her renal failure, she was placed on dialysis awaiting renal transplantation. She received a cadaveric kidney transplant that was well-matched on HLA typing. She had done well for two and a half weeks, but then her creatinine began rising and her urine output decreased. A renal biopsy was performed.

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Further History:

She was treated for the above condition and did well for another two years, at which time renal function began to gradually decrease, with rising BUN and creatinine over the next 4 months. Another renal biopsy was performed.

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Questions:

1. What is the significance of the pathologic findings? What do you suspect is happening?
2. What immunologic mechanisms are operative here?
3. What other problems can develop in this setting to produce a similar clincal picture?
4. What would be the mode of therapy and what would you predict as a response?
5. What is the significance of the pathologic findings seen in the second biopsy? What do you suspect is happening?
6. What immunologic mechanisms are operative in the first and second renal biopsies?
7. What is the prognosis?

CASE 4

History:

A 60-year-old man presented to his physician because he was having increasing difficulty doing any kind of exercise. He also had swelling of his ankles. He said his heart beats seemed to be irregular at times. He was referred to a cardiologist. An echocardiogram revealed thickening of the left ventricle and septum. An EKG revealed arrhythmias. Cardiac catheterization revealed decreased cardiac output.

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Further History:

He has back pain. A radiograph reveals lytic bone lesions in the vertebrae. A bone marrow biopsy is performed. His serum calcium is 12.2 mg/dL (range 8.8 - 11.0). A urinalysis shows proteinuria, all Bence Jones protein. He notes numbness and tingling involving the thumb and first two fingers of his hands. He develops a productive cough with yellowish sputum. A sputum culture is positive for Streptococcus pneumoniae and a chest radiograph reveals bilateral patchy infiltrates.

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Questions:

1. What is the diagnosis?
2. How did this disease cause the cardiac findings?
3. Explain the findings in the vertebral bone.
4. Explain the pulmonary findings.
5. Explain the numbness and tingling in his hands.

CASE 5

History:

A 55-year-old woman went to her local physician because she had difficulty swallowing. She had also noticed increasing difficulty in grasping objects along with stiffness of her fingers. Her blood pressure was markedly elevated. Laboratory testing revealed an antinuclear antibody (ANA) postive at 1:128 with a nucleolar pattern, normal serum complement levels, and a slightly elevated serum creatinine. She was referred to a nephrologist who performed a renal biopsy. A skin biopsy was also obtained.

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Questions:

1. What is the diagnosis? Are the pathologic findings specific? How do you arrive at a diagnosis in this case?
2. Does the immunofluorescence pattern help in making a diagnosis in this case?
3. What other symptoms might this patient have?
4. What pathologic finding(s) might you expect in the gastrointestinal tract, particularly the esophagus?

CASE 6

History:

A 27-year-old man was brought to the emergency room following an episode of minor trauma in which he sustained minor bruises to his left upper arm and chest in a fall off a ladder. Approximately one hour after admission, he became suddenly short of breath, gasping for air. Despite resuscitation, he died minutes later.

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Questions:

1. What do you think happened?
2. What immunologic mechanism is operative here?

CASE 7

History:

A 40-year-old man was hiking through a dense rainforest. Twelve hours later, he noted a rash on the left upper back, which intensified over the next couple of days.

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Questions:

1. What is the diagnosis?
2. What immunologic mechanism is operative here?
3. What findings would you expect to see in the skin?

CASE 8

History:

A 15-year-old adolescent underwent bone marrow transplantation for acute lymphocytic leukemia. About a month after transplantation, his pancytopenia was resolving as the marrow showed engraftment, and there was no evidence for recurrent leukemia. However, he developed a fine scaling skin rash, increasing jaundice, and diarrhea.

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Questions:

1. What is the name of this complication following transplantation?
2. How does this occur?
3. In what types of transplants does this occur?