Mesothelioma• Unilateral haziness• Smaller left hemithorax• Chest wall swelling• Loss of diaphragmatic silhouette• Blunting of costophrenic angle• Pleural thickening / Mass Sunday, January 06, 2013
CXR-44Diagnosis is: Neurofibroma Arrrow points to neurofibromas.
• Round lesions projected over lung fields are in chest wall• Lesions in chest wall along both sides and on abdominal wall• Posterior mediastinal mass: Para vertebral line on right side Black arrow points to posterior mediastinal mass distorting paravertebral line. White arrows point to neurofibromas in chest wall Sunday, January 06, 2013
chest clinical cases Uncontrolled asthma, recurrent rhinosinusitis, and infertility in a young womanSubmitted byP. Scott Meehan MDFellowDivision of Pulmonary, Allergy, Critical Care and Sleep MedicineThe Ohio State University Medical CenterColumbus, OhioJennifer W. McCallister MDAssistant Professor of Internal MedicineDivision of Pulmonary, Allergy, Critical Care and Sleep MedicineThe Ohio State University Medical CenterColumbus, Ohio
History• A 27 year old Caucasian woman with a history of uncontrolled asthma, allergic rhinitis, gastroesophageal reflux disease and recurrent pneumonias was referred for evaluation in the pulmonary clinic.• She was diagnosed with asthma at age twelve.• In the last six years she had been treated as an outpatient for pneumonia, sinusitis, and bronchitis several times per year and had required systemic corticosteroids several times per year for presumed asthma exacerbations.• Since last year she described persistent dyspnea with exertion, daily cough, and thick, yellow sputum production which had worsened.• This had not improved despite multiple courses of antibiotics, systemic glucocorticoids, and escalation of her inhaled corticosteroids.• She also reported wheezing and recurrent episodes of left sided chest pain.• There was no nighttime cough or limitation in daily activities.• She had not had frequent ear infections, yeast infections, epistaxis or hearing loss.• Her medications included a combination high dose inhaled corticosteroid and long acting beta agonist, montelukast, a proton pump inhibitor, albuterol, a multi-vitamin and methylphenidate.• She had been using short acting beta agonists multiple times per day without relief.• She had a 5 pack-year smoking history and quit eight years prior.• She was married and had been trying to conceive for about 15 months without success.• She had no known occupational exposures and owned one dog.• There was no known family history of asthma or other respiratory diseases. Sunday, January 06, 2013
Physical Exam• On physical exam, she was a well developed, obese (Body Mass Index 32 kg/m2) woman in no acute distress.• Her resting oxygen saturation (SpO2) was 97% on room air.• Head and neck exam were normal.• Heart sounds were normal without murmur, rub, or gallop with sounds heard best at right sternal border.• Auscultation of the lungs revealed inspiratory squeaks over the left upper lung field, otherwise clear.• Percussion and diaphragmatic excursion were normal.• The abdomen was soft and non-tender with no hepatosplenomegaly appreciated.• There was no peripheral edema or clubbing. Sunday, January 06, 2013
Lab• White blood cells 6.2 K/Ul• Differential, normal• Hemoglobin12.4 g/dLHematocrit 39.4 %• Platelets285 K/uL• Alpha 1 Anti-trypsin117 mg/dL (80-240 mg/dL)• Immunoglobulin G (IgG) 1001 mg/dL (700-1600 mg/dL)• Immunoglobulin A312 mg/dL (70-400 mg/dL)• Immunoglobulin M 116 mg/dL (40-230 mg/dL)• Immunoglobulin E 18.4 IU/mL (7-135 IU/mL)• Total hemolytic complement titer (CH50)115 units (51-150 units)• Pneumococcal antibody titer normal• Tetanus IgG antibody titer normal• Diphtheria IgG antibody titer normal Sunday, January 06, 2013
Pulmonary Function Testing• Pulmonary Function Testing• (FVC) …..3.54 L (109% predicted)• (FEV1) 2.94 L (105% predicted)• FEV1/FVC 81.4%• (TLC) 4.5 L (104% predicted)• (DLCO) 19 mL/min/mm Hg (82% predicted)• Flow volume loop normal Sunday, January 06, 2013
Question 1• What is the most likely diagnosis?• A. Uncontrolled Asthma• B. Primary Ciliary Dyskinesia/Kartagener Syndrome• C. Cystic Fibrosis• D. Bronchiectasis• E. Chronic Sinusitis Sunday, January 06, 2013
discussion• Primary Ciliary Dyskinesia (PCD) is a rare, typically autosomal recessive disease characterized by dysfunctional ciliary motility and impaired mucociliary clearance leading to multiple clinical manifestations including sino-pulmonary disease, infertility, and anatomic defects.• In about half of the cases it is associated with situs inversus, also known as Kartagener syndrome.• The link between Kartagener syndrome and immotile cilia was first described in 1976 .• The prevalence of situs inversus appears to be random and thought to be associated with loss of ciliary nodal function in embryogenesis .• The incidence of PCD is estimated at 1/16,000 births based on the presence of bronchiectasis and situs inversus which is likely underestimating the burden of disease given the heterogeneous nature and difficulty in diagnosing the disease .• Given this heterogeneity, diagnosis is often delayed until late childhood or early adulthood.• This diagnosis should be considered in those patients with the following: – Those with situs inversus/heterotaxia – Those with unexplained neonatal respiratory distress or hydrocephaly – Those with a chronic productive cough, bronchiectasis of unknown cause, or other severe upper airway disease – Males with immotile sperm – Females with recurrent ectopic pregnancy and/or infertility with other features of PCD Sunday, January 06, 2013
Question 2• What is the best therapeutic management of this patient?• A. Bronchodilators• B. Hypertonic saline• C. Oral corticosteroids• D. Lung transplantation• E. Aggressive antibiotic therapy and airway clearance maneuvers Sunday, January 06, 2013
discussion• There are no current medications specifically approved for use in PCD.• Additionally there are no randomized control trials in the treatment of PCD.• Treatment strategies are largely extrapolated from the cystic fibrosis literature and based on expert opinion.• Goals of treatment are to detect and treat complications with hopes of restoring and maintaining normal lung function.• Generally, aggressive treatments of pulmonary infection as manifested by worsening cough and sputum production, regular airway clearance maneuvers, and prevention of infection through proper immunization are the primary management modalities.• Sputum cultures should be obtained at baseline and with exacerbations to tailor antibiotic therapy.• Haemophilus influenzae, Staphylococcus aureus, and Streptococcus pneumoniae are the most common bacteria isolated from sputum.• Psuedomonas aeruginosa is seen more often in adults and isolated in 15% of patients.• Inhaled antibiotics in the presence of pseudomonas have been recommended based on cystic fibrosis evidence that there is better penetration with deceased systemic effects .• There is no evidence to recommend prophylactic antibiotics, however, there are some reports of azithromycin causing slight improvements in pulmonary function and rates of exacerbations in non-cystic fibrosis bronchiectasis.• Inhaled hypertonic saline has been demonstrated to improve lung function and reduce frequency of exacerbations in cystic fibrosis .• No studies have evaluated the use of this medication in PCD.• Additionally, rhDNase has anecdotally been shown to improve symptoms but no randomized studies are available .• If there is evidence of reversible airflow obstruction, true asthma, or wheezing, then it is recommended that the patient with PCD be treated according to current asthma guidelines . Sunday, January 06, 2013
Question3• Currently, what is the "gold standard" for diagnosis of primary ciliary dyskinesia?• A. Exhaled nitric oxide measurement• B. Genetic testing• C. Respiratory epithelial biopsy with electron microscopy• D. Sweat chloride testing• E. Pulmonary function testing Sunday, January 06, 2013
Question 4• What is the expected course of lung function change in patients diagnosed with PCD?• A. Stabilization of lung function• B. Deterioration of lung function• C. Improvement of lung function• D. Initial improvement, then stabilization of lung function• E. Variable course of lung function Sunday, January 06, 2013
discussion• Observational studies have suggested that the earlier a patient is diagnosed, the more favorable the outcome.• Lung function is believed to stabilize when the patient is diagnosed and managed at a multidisciplinary center .• These observations have been based on small numbers of patients.• More recently a Danish group studied seventy-four children and adults with PCD and measured 2,937 lung function measurements over 30 years.• This is the largest longitudinal study reported to date.• They demonstrated that there was a variable course of lung function (measured FEV1) with 34% showing decline, 57% showing stability, and 10% showing improvement.• Age of diagnosis and initial FEV1 were not related to course of disease . Sunday, January 06, 2013
Question 5• For which non-respiratory complication is this patient at highest risk?• A. Chronic suppurative otitis media• B. Ectopic pregnancy• C. Speech delay• D. Congenital heart disease• E. Liver disease Sunday, January 06, 2013
discussion• Several non-respiratory complications can be seen in patients with PCD.• The most common are related to chronic sinusitis and chronic middle ear effusions and are most often observed in children.• Evaluation by an otolaryngologist is essential.• Chronic middle ear effusions in young children have been thought to contribute to hearing loss with speech delay.• The management of this is controversial.• Myringototomy tubes sometimes lead to chronic otorrhea, but may improve hearing and speech .• Fertility issues in men and woman are a concern and frequent complication in PCD.• Although it is thought that males are universally infertile secondary to dysfunctional sperm, the heterogeneity of the disease suggests that up to 50% of males may conceive .• Women are at a particular risk for ectopic pregnancies and may have reduced fertility although not as prevalent as in males .• Situs inversus totalis itself does not cause medical complications in those with PCD, however, those with laterality defects may be at increased risk for congenital heart disease and should be screened accordingly . Sunday, January 06, 2013
HRCT-1• Find and outline a group of coarse centrilobular nodules in the right lung.• Find a single coarse centrilobular nodule in the left lung.• Find a conglomerate mass that causes deviation of the major fissure in the right lung.
• Find a group of fine centrilobular nodules (rosette) in the left lung.• Find a fine tree-in-bud pattern in the left lung and a coarse one in the right lung.• Find a conglomerate mass in the right lung.• Find a conglomerate mass containing a cavity .
• This slice of lung has a mass of conglomerate nodules in the posterior segment of the upper lobe. The opaque white regions in the mass represent necrosis, and the pinkish areas, viable inflammatory infiltrates. The irregularly-shaped slits represent cavitation.• Find two slits.• Note the satellite centrilobular nodules, indicating in this case an endobronchial spread of disease.• Find 2 centrilobular nodules in a lobule outlined by edematous, grey, interlobular septa. Note also the centrilobular nodules in another lobule just below this one.
• Outline and describe (name) the type of tissue at the lower right.• How would you describe the inflammatory response in the rest of the picture?• Find and name 4 of the large, multinucleated cells in the inflammatory infiltrate.• What types of cells compose the rest of the infiltrate?
Differential diagnosis of centrilobular nodules and cavitating masses on HRCT:• Tuberculosis• fungal,• Non-tuberculous mycobacterial.• chronic bacterial.• Centrilobular nodules and cavitating masses can also be seen with: • Langerhans cell histiocytosis. • Sarcoidosis. • Silicosis. • coal workers pneumoconiosis. • Wegeners granulomatosis. • rheumatoid arthritis.
Summary• Summary of diagnostic features of endobronchial tuberculosis on HRCT: • Tree-in-bud pattern • Clustered centrilobular nodules • Mass-like areas of consolidation • Cavitation in larger nodules or masses