A 27-year-old female presents to your office complaining
of dyspnea and cough productive of blood.
She has had fever, arthralgias, and weight loss as
well. She is a smoker. Chest radiograph shows patchy
infiltrates. You consider SARS but find that she
hasn™t traveled. You consider Goodpasture™s syndrome
and take an occupational history. She is an
office worker without any exposure to hydrocarbons.
Your interpretation of this information is:
A) Smoking is a common precipitant of Goodpasture™s
syndrome.
B) Goodpasture™s syndrome is unlikely because she is
not exposed to hydrocarbons.
C) This presentation is more consistent with SARS
and health authorities should be contacted despite
the lack of travel.
D) œNil disease with pulmonary involvement is the
more likely explanation for this patient™s symptoms.
E) None of the above is true.
Since this is the nephrology chapter, you of course want to order a urinalysis. For some unknown reason, your laboratory staff has forgotten to stock urine dipsticks and all of the microscopes are broken (seems like a common occurrence in our hospital!!). However, you are able to get a CBC, which shows a normal white count with a normal differential. Given her history and laboratory findings, diagnostic considerations in this patient include all of the following EXCEPT:
A) Wegner™s granulomatosis.
B) Neoplasm.
C) Lupus erythematosus.
D) Churg-Strauss disease.
Normal CBC ruling out d----> (d)
churgstrauss has eosinophilia.
The next day the laboratory staff finds the dipsticks and the patient is noted to have an active sediment (protein, white cells, red cell casts, etc.) along with an elevated creatinine of 2.5 mg/dl. You decide to check an anti-glomerular basement membrane titer (anti-GBM). This returns positive and you make the presumptive diagnosis of Goodpasture™s syndrome. The next step in the treatment of this patient is to:
A) Initiate steroids.
B) Initiate dialysis to preserve renal function.
C) Initiate plasmapheresis to remove anti-GBM from
the serum.
D) Initiate plasmapheresis, steroids, and cyclophosphamide.
E) Begin watchful waiting; most cases are self-limited.
(d) Initiate plasmapheresis, steroids, and cyclophosphamide
OOOPS sorry D makes more sense
1-Discussion
The correct answer is A. While the classic description of anti-glomerular basement antibody disease (Goodpasture™s syndrome) involves exposure to hydrocarbons, this is not the most common precipitator of anti-GBM disease. Anti-GBM disease can occur as a result of any pulmonary injury, and smoking is the most common precipitant of the disease. For this reason,
B is incorrect. Answer C is possibly correct given the fever and dyspnea. However, SARS is not associated with hemoptysis. œNil, or minimal change disease, is not associated with pulmonary findings. Thus, D is effectively ruled out.
2-Discussion
The correct answer is D. While all of the answers can cause hemoptysis with fever, Churg-Strauss disease is effectively ruled out with a normal CBC since the diagnosis requires at least 10% eosinophils in the peripheral smear. Churg-Strauss syndrome is also known as allergic granulomatosis and angiitis. It is associated with the use of steroids plus leukotriene inhibitors in patients with asthma as well as with other allergic causes of angiitis, including freebase
cocaine. All of the others are diagnostic possibilities. Note that all of these can cause a nephritic urine sediment.
3-Discussion
The correct answer is D. One must start immunosuppressants as well as plasmapheresis. The other answer choices are incorrect. Answer E is of particular note. This is not a patient to watch: she already has evidence of renal failure.