A 25-year-old man presented with diarrhea for 6 months, unintentional 20-pound weight loss, and upper-torso flushing when taking showers. Evaluation of diarrhea was nondiagnostic, including bacterial and parasitic stool cultures, celiac sprue serology, GI endoscopies with biopsies, and abdominal CT scan. Incidentally, CT of the chest revealed diffuse bilateral nodular opacities without adenopathy or pleural abnormalities (Fig 1). On further questioning,
the patient denied cough, dyspnea, hemoptysis,
fever, or night sweats.
The patient was born in India but had lived in
Chicago for the past 7 years. He was treated for
malaria 10 years ago and was not taking medications.
He smoked infrequently, denied illicit drug
use or recent or recent travel, and had no pets or occupational
exposures.
Physical Examination
BP, heart rate, temperature, and oxygen saturation
were normal. He appeared thin with temporal wasting.
His pulmonary examination was unremarkable,
without crackles or wheezes. A grade II/VI systolic
murmur was auscultated at the right sternal border.
His abdomen was soft without organomegaly. He
had no edema or clubbing.
Laboratory and Diagnostic Testing
CBC with differential, comprehensive metabolic
panel, liver and thyroid studies, urinalysis, HIV
antibody, erythrocyte sedimentation rate, and antinuclear
antibody were all normal. Pulmonary function
testing showed severe restriction; total lung
capacity was 48% of predicted. The patient was
unable to perform a breath hold to allow evaluation
of diffusing capacity for carbon monoxide.
Flexible fiberoptic bronchoscopy revealed normal
airways, and BAL samples showed no bacterial, viral,
fungal, or mycobacterial pathogens. Microscopic examination
of transbronchial biopsies showed acellular
waxy eosinophilic deposits. When stained with
Congo red and viewed under polarized light, the
biopsy sample was diagnostic of pulmonary amyloidosis. Evaluation for associated systemic
amyloid included a normal serum and urine immunoelectrophoresis,
normal bone marrow biopsy,
echocardiogram with normal systolic and diastolic
function, and no amyloid on prior GI biopsies.
the patient denied cough, dyspnea, hemoptysis,
fever, or night sweats.
The patient was born in India but had lived in
Chicago for the past 7 years. He was treated for
malaria 10 years ago and was not taking medications.
He smoked infrequently, denied illicit drug
use or recent or recent travel, and had no pets or occupational
exposures.
Physical Examination
BP, heart rate, temperature, and oxygen saturation
were normal. He appeared thin with temporal wasting.
His pulmonary examination was unremarkable,
without crackles or wheezes. A grade II/VI systolic
murmur was auscultated at the right sternal border.
His abdomen was soft without organomegaly. He
had no edema or clubbing.
Laboratory and Diagnostic Testing
CBC with differential, comprehensive metabolic
panel, liver and thyroid studies, urinalysis, HIV
antibody, erythrocyte sedimentation rate, and antinuclear
antibody were all normal. Pulmonary function
testing showed severe restriction; total lung
capacity was 48% of predicted. The patient was
unable to perform a breath hold to allow evaluation
of diffusing capacity for carbon monoxide.
Flexible fiberoptic bronchoscopy revealed normal
airways, and BAL samples showed no bacterial, viral,
fungal, or mycobacterial pathogens. Microscopic examination
of transbronchial biopsies showed acellular
waxy eosinophilic deposits. When stained with
Congo red and viewed under polarized light, the
biopsy sample was diagnostic of pulmonary amyloidosis. Evaluation for associated systemic
amyloid included a normal serum and urine immunoelectrophoresis,
normal bone marrow biopsy,
echocardiogram with normal systolic and diastolic
function, and no amyloid on prior GI biopsies.