Disease Information

Asthma is typically defined as acute reversible airways obstruction. Most typically, one thinks of the young patient with intermittent wheezing. However, asthma does present in the adult population as a new diagnosis. Some of these patients even present without shortness of breath or wheezing. Sometimes a patient’s chief problem is coughing. We call this “cough equivalent asthma”. Approximately 10% of asthmatics cough rather than wheeze.

Asthma is chiefly an inflammatory disease of the airways, and anti-inflammatory therapy with inhaled steroids is a cornerstone of asthma management. Often inhaled steroids are combined with other medicines in combination inhalers if the severity of the disease warrants.

The asthma patient should undergo a thorough evaluation for occult triggers such as sinus disease, gastroesophageal reflux, and allergies. These may all potentiate asthma and prevent successful treatment. Patients may undergo extensive testing and education to identify and help avoid triggers of their asthma.

Another key to control in asthma is patient education. We like all of our patients to have a thorough understanding of asthma, in particular asthma issues of monitoring and treatment. Peak flow meters can prove to be quite helpful as well as patient diaries. The Asthma Control Test can help to identify if a patient’s asthma is well controlled. Sometimes symptoms may be subtle, and patients often underestimate the severity of their disease. To take the Asthma Control test visit asthmacontrol.com.

Chronic cough is defined as a cough lasting three to eight weeks in duration or longer. The most common cause of cough in nonsmokers with a normal chest x-ray include postnasal drip syndrome (PNDS), asthma, and gastroesophageal reflux disease (GERD). Other less common causes of cough include angiotensin converting enzyme inhibitor (ACEI) induced cough, postinfectious syndrome, tympanic membrane irritation, habit cough, and psychogenic cough.

Our thoughts on this problem mirror that which was published recently by the consensus panel of the American College of Chest Physicians (Chest 1998; 114(2)S133-S181). We have found that the treatment of PNDS has been most effective with a combination of inhaled corticosteroids given nasally and older generation antihistamine/decongestants such as dexbrompheniramine maleate (eg Bromphen 12 mg qd, Dehist 6 mg bid) and azatadine maleate (eg Optimine 1 mg bid). We have found that the newer nonsedating antihistamines are not effective for the treatment of PNDS. Ipratropium bromide given intranasally is also effective in some patients. When chronic sinusitis is suspected as the etiology of PNDS, radiographs should be obtained. Chronic sinusitis requires prolonged antibiotic therapy. Many patients with PNDS induced cough do not have classic findings on history and physical examination and require empiric treatment.

GERD may cause cough in completely asymptomatic patients. We treat patients empirically and do not often obtain diagnostic tests unless patients have been unresponsive to treatment. H-2 blockers, proton pump inhibitors, and motility agents are employed in the treatment of GERD. It should be noted that chronic cough secondary to GERD can take months to abate. The mean time to recovery has been calculated in studies to be between 161 to 179 days.

Patients with asthma may present with cough and shortness of breath. This type of presentation is known as cough equivalent asthma. In patients with normal spirometry, we often obtain methacholine challenge testing (MCT). Standard treatment for asthma improves the patients symptoms and results in elimination of the cough.

When trying to get the specific cause of the patient’s cough under control, antitussive agents are often necessary to provide the patient with relief from a chronic nagging cough.

Chronic Obstructive Pulmonary Disease (COPD) is one of the most common diagnoses made in our office. COPD is a term used to reflect a disease characterized by both chronic coughing with sputum production and pulmonary emphysema.

Although many people with COPD have somewhat advanced airflow obstruction when diagnosed, this syndrome is not considered a death knell. There are many things we have to offer the patient with COPD.

Primary on the list is smoking cessation. It is absolutely necessary that patients with COPD stop smoking. If they have difficulty, we are frequently able to offer services through our office to assist with smoking cessation. As you know, many modalities are now available to assist patients with their efforts in smoking cessation.

Many different inhaled medications have been shown to be effective in treating the COPD patient. Oxygen therapy is needed for some, and all patients require education. COPD treatments are often divided into controller and rescue medicines. Rescue or reliever medicines are taken when patients are acutely short of breath. These medicines have a quick onset, but often do not last very long. They may have to be taken several times each day. Controller medicines do not relieve acute episodes and should not be taken in emergencies. They help to control the underlying disease and should be taken on a regular basis regardless of how a patient feels from day to day. These controller medicines have been shown to modify the disease over the long term, and, taken regularly, reduce the need for the reliever medicines. Some have even been shown to reduce the frequency of exacerbations of COPD which often require treatment with steroids and antibiotics. Supplemental oxygen has been shown to improve quality of life and even prolong survival in patients whose oxygen saturation is low enough (<88%) consistently.

Pulmonary rehabilitation has proved quite useful in this group of patients to improve well being, exercise tolerance, quality of life, and to help decrease hospitalizations. Patients tend to be more satisfied after successfully completing pulmonary rehabilitation due to their physical conditioning, but the educational component in pulmonary rehab is equally important.

Many patients will find themselves physically limited by COPD. Patients who understand that this is a chronic disease will be quicker to develop adaptive skills and techniques that are necessary for successfully living.

Dyspnea is a subjective sensation of difficult or uncomfortable breathing.  The underlying neural pathways responsible for dyspnea are not well understood.  Multiple interacting mechanisms are thought to be responsible for the sensation of dyspnea.

Chronic dyspnea is a challenging clinical entity and often frustrates both physicians and patients.  We have found it useful to think of causes of dyspnea as belonging to the following categories:

1. Pulmonary disease
2. Cardiovascular disease
3. Psychogenic disease
4. Neuromuscular disease
5. Miscellaneous

A study done by Dr. R.S. Irwin and colleagues (Archives of Internal Medicine 1989; 100:1293-1299) reviewed 100 consecutive patients presenting with chronic dyspnea in an outpatient setting. 75% of the patients were found to have pulmonary disorders, 10% had cardiac disorders and psychogenic disorders, and deconditioning and gastroesophageal reflux disease each accounted for 5%.  These authors found that specialized testing was often necessary, because the history and physical examination were often not sufficient to establish a diagnosis.

We have found the following approach to be most useful in assessing patients with unexplained dyspnea:

1. A thorough history and physical are carried out with focused questioning on a description of the dyspnea with associated symptoms and any triggers that might be present.
2. All patients should have a chest x-ray, pulmonary function tests, appropriate lab tests, and room air oxygen saturation.
3. In selected patients, further testing may be necessary.  We have found the most useful tests include methacholine challenge testing (MCT), cardiopulmonary exercise testing (CPET), ventilation perfusion scan, high resolution CT scan of the chest (HRCT chest), and echocardiography.

Interstitial lung disease (ISLD) is a multifaceted disorder. It is characterized by interstitial infiltrative and sometimes fibrotic lesions on plain chest x-ray. Patients often complain of shortness of breath, persistent dry cough or simple fatigue. Many patients are brought to the attention of physicians because of an unsuspected abnormality seen on their chest x-ray.

The differential diagnosis of interstitial disease includes many connective tissue diseases, granulomatous disorders, neoplasms, drug induced disorders, organic dust disease, inherited disorders, poison induced lung injury, infections, occupational induced lung disease, and idiopathic pulmonary fibrosis.

A fibrotic reaction may present after pneumonitis but will not demonstrate progression either by chest x-ray or pulmonary function testing criteria in 15-20% of patients evaluated for interstitial lung disease.

We believe that all patients with interstitial lung disease should receive a basic evaluation that includes the following:

1. All patients should have laboratories done for collagen vascular diseases
2. Careful history should be done for pneumoconiosis and drug induced disease
3. We try to establish disease progression:
   1. Obtain chest x-rays, two or more years apart to look for stability or advancing of the pulmonary markings
   2. Pulmonary function changes that are significant include a decrease in the vital capacity of 10% or more, decrease in the DLCO by 20% or more, or an elevation in the alveolar to arterial oxygen gradient by 5 mm of mercury or more
4. Appropriate patients should undergo fiberoptic bronchoscopy to rule out granulomatous disease, neoplasm, infection, pulmonary alveolar proteinosis, histiocytosis X. These disorders are treatable and patients may have an excellent prognosis.

In patients with irreversible forms of interstitial lung disease, the prognosis is quite grim. We have experienced similar results in survival that have been reported in the literature for patients with idiopathic pulmonary fibrosis. It has been largely reported that the average duration of survival in these patients is in the 4-5 year range after diagnosis. There are subsets of patients with idiopathic pulmonary fibrosis that have an excellent response to treatment and their survival is prolonged.

The treatment of idiopathic pulmonary fibrosis can be very frustrating. Of the options available, we favor corticosteroid therapy, sometimes combined with cyclophosphamide. 10-20% of patients will have a measurable response to corticosteroid therapy alone. We look at the pulmonary function changes as described above to determine if lung disease has stabilized or if the patient has actually regained function. Azathioprine has been used with some success in the literature but we have not found it to be that useful in clinical practice.

99% of all neoplasms occurring in the lung are cancer.  Benign tumors do occur.  However, of the cancers that occur in the lung and those considered primary to the lung, there are two broad categories, small cell and non-small cell carcinoma. 

It is important to establish a diagnosis when a suspected cancer is identified in the lung because the treatment may vary widely.

Small cell carcinomas are treated primarily with chemotherapy.  Once we at Pulmonary Specialists have identified this as the primary diagnosis, the usual response is for medical oncology referral for further evaluation and treatment.

Non-small cell carcinoma encompasses cell types such as squamous cell carcinoma, adenocarcinoma and large cell carcinoma.  These tumors are treated preferentially with surgery. If a patient is not considered a surgical candidate then other modalities are commonly used such as chemotherapy and radiation therapy. Additional therapies that are offered may include laser therapy, photodynamic therapy, or endobronchial brachytherapy. These latter three therapies are primarily considered palliative.  However, the use of these therapies may substantially improve a patients well being, particularly in the face of obstructing tumors.  Laser therapy is utilized for obstructing bronchial carcinoma and is administered through either rigid or fiberoptic bronchoscope for control of endobronchial tumor.  Similarly, photodynamic therapy is endobronchial application of laser for stimulation of light activated pharmaceutical agent.  Endobronchial brachytherapy is direct application of radiation within the bronchus that helps control tumor locally.

We consider our job not only to establish a diagnosis and to determine cell type, but also to determine the patient’s suitability for surgical therapy.  This is done through pulmonary function testing and occasionally other exams.

Although lung cancer is a horrible diagnosis with often poor outcomes particularly when discovered in late disease stages, there have been significant advances in treatment as well as palliative modalities to aide in the care of the lung cancer patient.   

Mesothelioma is a neoplastic growth of cells lining the chest cavity, ie the pleura. The most well known causative factor is asbestos exposure.

Patients with mesothelioma frequently present with recurrent bloody pleural effusions. Even with repeated thoracentesis, diagnosis can prove difficult. If thoracentesis does not provide a diagnosis even with multiple procedures, pleural biopsy is usually required. We find this most advantageous often times performed via a thoracoscopy, at which time not only can a diagnostic biopsy be performed but therapy in the form of pleural drainage and pleurodesis can be performed as well.

Other therapies such as radiation and chemotherapy have limited roles as well as surgical therapy of mesothelioma. These, however, do upon occasion have a place in the therapy of mesothelioma.

Nonspecific bronchial hyperresponsiveness (NBH) is an entity used to characterize the extreme sensitivity of airways to a variety of stimuli. Patients may respond to chemical, physical, or pharmacological stimuli with shortness of breath, coughing, or wheezing. NBH is not a diagnosis per se, but rather is an airway abnormality that can be measured in the pulmonary function laboratory. Perhaps the best method of quantifying NBH is with the use of methacholine bronchoprovocation testing. The methacholine challenge test (MCT) is performed by having patients inhale the bronchial irritant methacholine, which induces bronchoconstriction in sensitive patients. Subjects without bronchial hyperresponsiveness do not react to methacholine.

A number of diseases are characterized by NBH including asthma, reactive airways dysfunction syndrome (a syndrome caused from a brief massive exposure of an inhaled irritant, usually occurring in the workplace), chronic bronchitis, bronchiolitis, viral upper respiratory infection, foreign body aspiration, near drowning, smoke inhalation, sarcoidosis, chronic cough, and post-acute respiratory distress syndrome.

We have found it helpful to think of NBH as a physiologic abnormality rather than a disease. NBH can be a feature of the diseases listed above and it can be transient as well as a more lasting abnormality. We have found MCT to be exceedingly useful in our practice to help define the cause of wheezing in our patients in whom reversible airflow obstruction has not been documented.

Postpericardiotomy syndrome (PPS) is a disorder which occurs after cardiac surgery. Most patients present with a fever, pleurisy, pleural effusions, hypoxia, and may have pulmonary infiltrates. Those patients begin to develop symptoms within two days to four weeks after cardiac surgery. PPS occurs in approximately 18% of all patients who have undergone coronary artery bypass grafting (CABG).

We have found that a therapeutic and diagnostic thoracentesis greatly improves patients’ symptoms and sense of well-being. The pleural fluid is usually a bloody exudate.

PPS is responsive to nonsteroidal antiinflammatory drugs (NSAIDs). Indomethacin is used most commonly. We will often place patients on prednisone who are intolerant to NSAIDs.

Pulmonary hypertension is a condition in which pressures in the pulmonary artery are persistently elevated. It is therefore not a diagnosis, but instead a physiological condition that may accompany a number of diagnoses.

We have found it useful to think of the causes of pulmonary hypertension as belonging to one of the following three categories:

1. Increased vascular resistance
   1. Hypoxia
   2. Obstructive
2. Increased left atrial pressure
3. Increased pulmonary flow

Patients with pulmonary hypertension often complain of shortness of breath and weakness. Additionally, they may experience dizziness, palpitations and/or syncope.

We believe that patients with pulmonary hypertension should receive a basic evaluation that includes the following:

1. All patients should have an echocardiogram done to estimate pulmonary artery pressures and to rule out valvular heart disease, left atrial myxoma, atrial/ventricular septal defect, and patent ductus arteriosis.
2. A careful history should be done with an emphasis on discovering possible connective tissue disease, a history of venous thromboembolism, sleep apnea, or a family history of primary pulmonary hypertension.
3. Pulmonary function tests should be done that include lung volumes and a room air arterial blood gas.
4. In the appropriate patient, a V/Q scan should be done to rule out chronic pulmonary emboli.

The treatment of pulmonary hypertension should be directed toward the causative disorder if it can be discovered. In cases in which a secondary cause of pulmonary hypertension cannot be discovered, the treatment is much more challenging. It is our policy to treat these patients with oxygen, diuretics when necessary and calcium channel blockers. We are routinely referring patients to the university centers in which Prostacyclin therapy is delivered with the use of the long-term intravenous pump. We have been very encouraged by the literature and our own experience with the long-term infusion of Prostacyclin.

Radiation lung injury is a common complication of radiation therapy to the thorax. Approximately 43% of all patients undergoing thoracic radiation therapy will develop radiographic change consistent with radiation pneumonitis. However, only 8% of patients undergoing thoracic radiation develop symptoms along with a radiographic change.

There are two well-recognized syndromes of radiation induced lung injury. The first is radiation pneumonitis. There is a latent period of 1-3 months following radiation therapy. Symptoms of dyspnea, cough, fever, or weakness may develop prior to any chest x-ray changes. Chest x-ray findings that are typical include a diffuse haziness around the hila, indistinct pulmonary vessels, and an infiltrate that does not follow anatomic boundaries of the lung.

The second syndrome is that of radiation fibrosis. This is permanent damage that takes 6-24 months to evolve after radiation treatment. Patients may present with dyspnea, cor pulmonale, or chronic cough. Chest x-ray findings include honeycombing and volume loss.

Risk factors for radiation damage to the lung include a radiation dose above 3500 cGy, prior radiation therapy, chemotherapy, steroid treatment withdrawal and pre-existing lung disease. We have found that Prednisone therapy in a dose of 60-100 mg by mouth per day, tapered over several weeks provides significant relief of symptoms to most patients. Patients with concomitant exposure to Bleomycin or Adriamycin represent a subset of patients that in our experience does poorly.

Reactive airways dysfunction syndrome (RADS) is defined as the sudden onset of asthma like symptoms following a high level irritant gas, vapor, or fume exposure. It was originally described by Doctors Brooks, Weiss, and Bernstein in an article in the journal Chest in 1985 (Chest 1985; 88:376-84).

Patients with RADS present with symptoms of shortness of breath, wheeze and cough within 24 hours of an exposure to a high level of respirable irritant. The exposure is typically single, high level and brief. Patients present appearing extremely ill with signs of toxicity, including conjunctival and mucous membrane erythema and edema with labored breathing.

Patients respond to supportive therapy, including bronchodilators, intravenous steroids, and intravenous crystalloid for volume resuscitation where indicated. Some patients are left with permanent airway hyperresponsiveness. Some recover fully after a number of months.

A second type of irritant induced asthma-not so sudden induced asthma-has been described by a number of investigators but most notably by Dr. Brooks and colleagues in an article appearing in the journal Chest (Chest 1998; 111:42-49). These patients are exposed to a low level of respirable irritant for more prolonged periods of time. The exposure is generally of a more chronic nature. Symptoms of airway hyperresponsiveness take longer to develop in these patients. Their presentation is one of dyspnea, wheezing, and sometimes chronic cough. They do not appear as acutely ill as patients with sudden onset irritant induced asthma. Dr. Brooks and colleagues have found patients with not so sudden induced asthma have a high prevalence of atopy, with 52% of the patients in their study in the aforementioned journal having a history of atopy. We feel that patients in this group often have pre-existing host susceptibility. They may have undiagnosed asthma or atopy or other airway dysfunction. These patients also respond favorably to inhaled bronchodilators, inhaled steroids, and other supportive measures. Their symptoms tend to be more protracted and they may require lifelong treatment.

Sarcoidosis is a multisystem granulomatous disorder of unknown etiology and most commonly affects young adults. Most studies have shown the highest of incidence in the third and fourth decades with a variable female predominance. It is most commonly seen in people of African or Scotch-Irish decent. Patients with sarcoidosis most often present with abnormal chest x-rays. Sarcoidosis can effect all vital organs including the eye, central nervous system, the bones and joints, the skin, the gastrointestinal tract, the heart, and the kidneys.

Thoracic sarcoidosis has been classified in four stages based on the appearance of the chest radiograph:

• Stage I – bilateral hilar lymphadenopathy
• Stage II – bilateral hilar adenopathy plus pulmonary opacities
• Stage III – pulmonary opacities only
• Stage IV – irreversible pulmonary fibrosis

Most patients with thoracic sarcoidosis present with Stage II or III disease. Sometimes the patient is asymptomatic, but more commonly patients present with shortness of breath, cough or chest tightness.

The five most commonly encountered sleep disorders we encounter in our practice are the sleep apnea hypopnea syndrome (SAHS), the upper airway resistance syndrome (UARS), the restless legs syndrome (RLS), the periodic limb movement disorder (PLMD), and narcolepsy. The key to diagnosis begins with clinical suspicion and involves a thorough history, a directed examination, and a diagnostic polysomnogram (PSG) when necessary.

Patients with SAHS may experience breathing disturbances, difficulty maintaining sleep, excessive daytime somnolence (EDS), fatigue, personality changes, and impaired general function. Risk factors for SAHS include male gender, obesity, ethanol use, use of sedatives, familial factors, craniofacial features, snoring, sleep deprivation, and tobacco use. We feel that the treatment of SAHS should be individualized, but for all patients it should include weight loss and improved sleep hygiene. Other treatment options include oral devices, NCPAP, BiPAP, and surgical options.

UARS was described in 1991 and is a syndrome characterized by hypersomnolence, polysomnogram revealing an AHI of less than 5, and abnormal sleep architecture. The EEG shows abnormal arousals that occur because of increased airway resistance. These patients are generally young (20-40 years of age) but can be of any age. They are generally not obese and snoring may or may not be a complaint. Rhinitis is common. UARS is treated with NCPAP, therapy for rhinitis, and improved sleep hygiene. Occasionally nasal surgery is necessary.

PLMD is very common and may occur in up to 29% of patients over the age of 50. PLMD is best diagnosed by a polysomnogram revealing a PLM index of greater than 5 plus evidence of arousals and sleep fragmentation. We have found that the treatment of PLMD is most effective when it involves anti-Parkinson medication (Sinemet) and benzodiazepines (clonazepam). Occasionally, we find it necessary to use opiates (oxycodone).

RLS is a syndrome characterized by vague and difficult to describe leg discomfort. It is most prominent during times of inactivity. It is relieved by movement. Patients often experience insomnia and arousals from sleep causing sleep fragmentation. RLS is very common and affects up to 5-10% of the population. RLS is made worse by antidepressants (TCAs and MOIs). The treatment of RLS is identical to the treatment of PLMD described above.

Narcolepsy is a disorder of unknown origin. It is characterized by abnormal sleep tendencies including EDS, disturbed nocturnal sleep, and pathological manifestations of REM sleep. Narcolepsy is usually seen in patients in their teens to 20s that complain of EDS. The diagnosis is made by finding a relatively normal polysomnogram and a diagnostic multiple sleep latency test (MSLT). We have found that the treatment of narcolepsy is most efficacious when it involves scheduled naps, stimulants, and agents to combat cataplexy.

A solitary pulmonary nodule (SPN) is spot on the lung four centimeters or less surrounded by normal lung tissue. Many are not malignant. Depending on the population studied, 50-85% of are benign.

Strong evidence of a benign SPN includes the following:

1. The presence of a dense central, laminated, or popcorn calcification on routine chest radiograph.
2. Old chest radiograph showing the lesion present but unchanged over the last two years.
3. Having a patient less than 30 years of age who is a nonsmoker.

In evaluating a patient with an SPN, it is our practice to find any old chest x-rays that could have been done previously. It is important for us to look at the actual film and not just rely on a radiology report. In our patients who are smokers, we are always worried about malignancy, and bronchoscopy is often undertaken to rule out tumors in the bronchial tubes that sometimes cannot be seen on chest x-rays. Often biopsies are required. These can be achieved at the time of bronchoscopy, or are often done by radiologists in a CT scanner with direct computer guidance. Sometimes, biopsies are not needed, and patients can undergo serial examinations with chest x-rays or CT scans to watch for growth in the lung nodule. Lesions followed over a two year period that do not change on chest imaging are benign lesions approximately 99% of the time. This often provides great reassurance to both physician and patient.