I've had the symptoms I am about to describe for a year and a half. (I've never smoked) I have excessive mucous in my lungs which causes a chronic cough. I am unable to get much up and when I start coughing, it often ends up in a rattling vibration that causes me to gag and sometimes even vomit. When my cough does produce the mucous, it is VERY thick and white. I have rheumatoid arthritis and atrial fibrulation, so am on meds for those. I've also been diagnosed with Barretts syndrome and am on meds for that. I have been through a battery of tests - 3 lung CAT scans, CAT scans of sinuses by ENT, allergy tests, X-rays, blood work, and 2 bronchoscopies. All tests have come back inconclusive. The doctor who did the last bronchoscopy said he had to go in and out 5 times because the mucous was so thick it clogged the scope. No relief from OTC meds. Some quieting of the couch after heavy dose of cough medicine with codine. I've had several rounds of antibiotics and prednysone with no improvements. I've seen general physician, 2 pulmonary specialists, ENT, allergist, cardiologist, gastro doctor and rheumatologist. Doctors are stumped, they recommend I go to National Jewish or one of the Mao clinics. What could be going on?
There is a good possibility that your condition is a disease called, Pulmonary Alveolar Proteinosis. The following is from Chapter 59 Pulmonary Alveolar Proteinosis (below) in: Mason: Murray & Nadel's Textbook of Respiratory Medicine, 4th ed. 2005
This is the most important statement from that chapter.: “There are many reports in the literature of alveolar proteinosis-like lung disease in the setting of other systemic disorders.”
It suggests that physicians with a special interest & knowledge regarding this condition, Pulmonary Alveolar Proteinosis, would also be aware of the varieties of the disorder and other diagnoses that can mimic it.
I suggest that you first discuss the contents of this E-mail with your main pulmonary physician. Depending on the outcome of that discussion, you might then wish to consider requesting a second opinion at the Cleveland Clinic, the Beth Israel Deaconess Medical Center in Boston or National Jewish Health in Denver, Colorado.
Chapter 59 – Pulmonary Alveolar Proteinosis
Mani S. Kavuru, M.D.
Director, Pulmonary Function Laboratory, Department of Pulmonary and Critical Care Medicine, Cleveland Clinic Foundation, Cleveland, Ohio
Tracey L. Bonfield, Ph.D.
Department of Pulmonary Medicine, Cleveland Clinic, Cleveland, Ohio
Mary Jane Thomassen, Ph.D.
Director, Cytokine Biology Laboratory, Division of Pulmonary and Critical Care Medicine, Cleveland Clinic Foundation, Cleveland, Ohio
Historically, there has been no effective pharmacotherapy for PAP, and sequential whole-lung lavage (WLL) under general anesthesia has become the mainstay of treatment. In fact, WLL is the only therapy that has emerged to improve symptoms and oxygenation in patients with PAP. See the attachment above. From the Department of Interventional Pulmonology,
Beth Israel Deaconess Medical Center, Boston, MA.
Correspondence to: Armin Ernst, MD, Chief, Interventional
Pulmonology, Beth Israel Deaconess Medical Center, Harvard
Medical School, Deaconess Building 201, Boston, MA 02215;
The cardinal feature of PAP is the filling of lung alveoli with periodic acid-Schiff-positive, lipid-rich material with preservation of normal lung architecture and paucity of inflammation or fibrosis. PAP may be viewed as a syndrome consisting of a heterogeneous group of diseases resulting from failure to clear surfactant. Excess accumulation of alveolar surfactant can occur by various mechanisms ( Fig. 59.1): relative GM-CSF deficiency, abnormal or dysfunctional surfactant, or overwhelming of clearance mechanisms. There are several potential ways to classify this syndrome clinically:
PAP occurring in the presence of circulating anti-GM-CSF
Neonatal or congenital presentation of PAP
PAP in the setting of a systemic inflammatory disease or malignancy (most often hematologic)
PAP in association with specific exogenous or occupational exposures
ADULT IDIOPATHIC PULMONARY ALVEOLAR PROTEINOSIS
Most of the published reports of the PAP syndrome involve its occurrence in adults, usually as an isolated idiopathic lung disease without an underlying malignancy, immunologic abnormality, exogenous occupational exposure, or infection. This sporadic rare disorder may be referred to as "primary" or "idiopathic." Since the initial report of neutralizing anti-GM-CSF antibodies in the circulation of PAP patients by Kitamura and colleagues, all of the published cases of idiopathic PAP in adults have been associated with the presence of this antibody.
SECONDARY PULMONARY ALVEOLAR PROTEINOSIS
There are many reports in the literature of alveolar proteinosis-like lung disease in the setting of other systemic disorders ( Table 59.1 ). When PAP develops in association with another medical condition, it is often referred to as "secondary PAP." Chronic myelogenous leukemia and myelodysplastic syndrome are the most frequently noted underlying marrow disorders, but several others have also been reported. Cordonnier and colleagues recently reported that PAP accompanied 5.3% of all hematologic malignancies at their institution, which rose to 8.8% among neutropenic patients. Among their 10 patients with PAP, 5 had acute myeloid leukemia, 4 had chronic myelogenous leukemia, and 1 had acute lymphocytic leukemia. Three patients had undergone bone marrow transplantation. Nine of their 10 patients were neutropenic when they developed PAP, and most patients recovered spontaneously at the time of bone marrow restoration. A recent report of acute lymphoid leukemia with secondary PAP during the neutropenic phase resolved with granulocyte-colony stimulating factor. Several possible mechanisms have been advanced for this form of secondary PAP, including the malignant clone affecting the monocyte-alveolar macrophage lineage (i.e., leukemic cells lacking expression of the βc chain of the GM-CSF receptor), the effects of chemotherapy and radiation on macrophage number and function, and possibly the use of corticosteroid in this setting causing increased production of phospholipids. Isolated case reports of systemic disease with effects on cell-mediated immunity have also been described in association with secondary PAP, including thymic alymphoplasia, immunoglobulin A deficiency, solid organ transplantation, and acquired immunodeficiency syndrome.
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