A 28-year-old man comes to the emergency department due to worsening dyspnea, cough, and blood-streaked sputum for the last 3 days. He has had no fatigue, fever, chills, upper respiratory symptoms, skin rash, eye symptoms, or joint pains. The patient has no history of lung disease but takes ibuprofen occasionally for knee pain. He does not use tobacco, alcohol, or illicit drug. His father had a myocardial infarction at age 65 and his mother has rheumatoid arthritis.
Blood pressure is 150/90 mm Hg, pulse is 100/min, and respirations are 24/min. Bilateral crackles are present in both lung bases and mid-lung fields. Extremity examination reveals 1+ pitting edema to the mid-shins bilaterally. The remainder of the examination is unremarkable.
Laboratory results are as follows:
Chest x-ray shows diffuse pulmonary opacities.
Which of the following is the most likely diagnosis in this patient?

Question

A 28-year-old man comes to the emergency department due to worsening dyspnea, cough, and blood-streaked sputum for the last 3 days.  He has had no fatigue, fever, chills, upper respiratory symptoms, skin rash, eye symptoms, or joint pains.  The patient has no history of lung disease but takes ibuprofen occasionally for knee pain.  He does not use tobacco, alcohol, or illicit drug.  His father had a myocardial infarction at age 65 and his mother has rheumatoid arthritis.
Blood pressure is 150/90 mm Hg, pulse is 100/min, and respirations are 24/min.  Bilateral crackles are present in both lung bases and mid-lung fields.  Extremity examination reveals 1+ pitting edema to the mid-shins bilaterally.  The remainder of the examination is unremarkable.
Laboratory results are as follows:
 Chest x-ray shows diffuse pulmonary opacities.
Which of the following is the most likely diagnosis in this patient?

Quizplus · Accepted Answer

11ec193a_98c9_4ee9_853d_295f7458193d_MD0002_00 This patient has evidence of pulmonary-renal syndrome (pulmonary hemorrhage and glomerulonephritis), which is commonly caused by anti-glomerular basement membrane (GBM) antibody disease (Goodpasture syndrome) or by granulomatosis with polyangiitis. His lack of constitutional vasculitic symptoms (eg, fever, weight loss, arthralgia, fatigue) makes anti-GBM antibody disease more likely. Patients with anti-GBM antibody disease typically have a progressive glomerulonephritis (eg, hematuria, red blood cell casts, proteinuria, hypertension) and acute renal failure. Pulmonary involvement occurs in up to 60% of patients and presents with dyspnea, cough, and hemoptysis with pulmonary infiltrates on chest x-ray. Diagnosis is suggested by positive serum anti-GBM antibody and is confirmed by renal biopsy showing linear immunofluorescence of IgG in glomerular capillaries. Plasmapheresis is the preferred treatment to remove circulating antibodies and is combined with corticosteroids and cyclophosphamide to prevent formation of new antibodies. Compared to anti-GBM antibody disease, granulomatosis with polyangiitis is an inflammatory vasculitis and therefore more likely to cause constitutional vasculitic symptoms; in addition, ear, nose, and throat and ocular manifestations are common (Choice D). (Choice B) Cryoglobulinemic vasculitis can involve the lung and kidney but more commonly presents with dyspnea, cough, and isolated proteinuria rather than hemoptysis and glomerulonephritis. In addition, systemic symptoms, elevated rheumatoid factor, and low complement levels would be expected in active disease. (Choice C) Churg-Strauss syndrome (allergic granulomatosis and angiitis) is a vasculitis that rarely causes hemoptysis and glomerulonephritis; it more commonly presents with severe asthma, rhinosinusitis, prominent eosinophilia, and systemic symptoms. (Choice E) Systemic lupus erythematosus can also cause nephritic syndrome and, rarely, hemoptysis. However, this patient's normal serum complement and negative double-stranded DNA make systemic lupus erythematosus unlikely. Educational objective: Anti-glomerular basement membrane antibody disease (Goodpasture syndrome) commonly presents with progressive glomerulonephritis, renal failure, and pulmonary hemorrhage. Systemic symptoms (eg, fever, weight loss, arthralgias, fatigue) are absent; their presence should raise suspicion for vasculitis. __________ References: Cutting edge issues in Goodpasture's disease. (http://www.ncbi.nlm.nih.gov/pubmed/21207195)

A 28-Year-Old Man Comes to the Emergency Department Due to Worsening