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Home , Acute chest syndrome, Asthma, Hemothorax, Pulmonary hypertension

Christopher Taggart, MD St. Mary’s Medical Center, Department of Family : Looking Medicine, Grand Junction, Colo beyond the usual suspects christopher.taggart@ sclhs.net COPD and come to mind when a patient The authors reported no potential conflict of interest is short of breath. But the detailed relevant to this article. here should lead you to suspect an uncommon cause.

CASE u Joan C is a 68-year-old woman who presents to the of- PRACTICE RECOMMENDATIONS fice complaining of an enlarging left chest wall mass that ap- peared within the past month. She was treated for small-cell ❯ Consider diagnoses other 11 years ago. She has a 45 pack-year smoking his- than , COPD, failure, and pneumonia in tory (she quit when she received the diagnosis) and has heart patients with persistent or failure, which is controlled. Your examination reveals a large progressive dyspnea. C (5 cm) firm mass on her left chest wall. There is no erythema or tenderness. She has no other complaints. You recommend surgi- ❯ Avoid steroids in patients with pericarditis cal biopsy and refer her to surgery. because research shows Ms. C returns to your office several days later complaining that they increase the of new and worsening shortness of breath with exertion that be- risk of recurrence. B gan the previous day. The presentation is similar to prior asthma exacerbation episodes. She denies any , fever, , ❯ Consider anticoagulation with warfarin in patients with symptoms at rest, or . On exam she appears comfort- pulmonary arterial hyperten- able and not in any acute distress. You refill her albuterol. sion and cor pulmonale. Evi- The next day you learn that she is being admitted to the dence shows that it improves hospital with respiratory distress. An x-ray of her chest shows a survival and quality of life. A concerning mass in her right upper lung.

Strength of recommendation (SOR) yspnea is an uncomfortable awareness of breath- A Good-quality patient-oriented evidence ing that occurs when complex neurochemical path- B Inconsistent or limited-quality ways used to maintain oxygenation and ventilation patient-oriented evidence D are disrupted. (See "The variable, and subjective, process of Consensus, usual practice,  C 1-5 opinion, disease-oriented dyspnea" on page 528). Sometimes described as air hunger, evidence, case series increased work of , chest tightness, or chest constric- tion, the symptom is usually disproportionate to the patient’s level of exertion. Most of the time dyspnea is due to either a primary lung or cardiovascular problem such as chronic obstructive pul- monary disease (COPD), asthma, pulmonary (PE), pneumonia, congestive (CHF), or myocardial in- farction. However, many other illnesses can also produce this symptom (TABLE 1). This article will review the uncommon eti- ologies of dyspnea that should be considered when the usual suspects have been eliminated.

526 THE JOURNAL OF FAMILY PRACTICE | AUGUST 2016 | VOL 65, NO 8 Cardiovascular culprits trapezius that is relieved by leaning Dyspnea is a common symptom with car- forward diovascular diseases because cardiac output • pericardial friction rub relates directly to tissue oxygenation. Any pa- • electrocardiographic changes show- thology that decreases the ability of the heart ing ST segment elevation in all leads and blood vessels to transport will like- but aVR and V1 and diffuse PR interval ly trigger discord between the central, periph- eral, and neurochemical respiratory centers. • pericardial effusion on Two uncommon cardiovascular etiologies of . dyspnea are pericarditis and . ❚ Treatment. Treat non-severe and non- Pericarditis life threatening pericarditis with nonsteroi- Pericarditis is generally a self-limited con- dal anti-inflammatory drugs (NSAIDs). Avoid dition that responds promptly to initial steroids because research has shown that treatment, although it can cause significant they increase the risk for developing recur- morbidity and mortality. One study showed rent pericarditis.8 Hospitalize patients with that acute pericarditis accounted for 5% of large pericardial effusions and consider them patients presenting to the emergency depart- for pericardiocentesis. Treat cardiac tam- ment with non-ischemic chest pain.6 Another ponade with urgent pericardiocentesis and study found that the in-hospital mortality hospitalization. Suspect rate for acute pericarditis was 1.1%.7 pulmonary Pericarditis causes dyspnea by restrict- Myocarditis arterial ing the heart’s ability to relax, thus decreasing Myocarditis can have a variety of etiologies in and cardiac output. This occurs with (TABLE 29,10). Myocarditis causes dyspnea ei- younger patients large effusions (>20 mm in width on echo- ther by causing pericardial effusion or heart with exertional cardiography) and can lead to cardiac tam- failure. dyspnea, ponade—a medical emergency that should ❚ Diagnosis. Myocarditis can be difficult , chest be suspected in patients with muffled heart to diagnose. Suspect it in any patient with pains, or sounds, , and increased jugular cardiogenic shock, acute or subacute left ven- who venous distention (Beck’s triad). tricular dysfunction, or myocardial damage don't have other Pericarditis etiologies include: from a non-coronary disease source. heart or lung • infectious causes (viral and bacterial Echocardiography and cardiac serum bio- disease signs entities, myocarditis), markers can help diagnose myocarditis, but or symptoms. • rheumatologic causes (gout, systemic the diagnostic gold standard remains myo- erythematosus, tumor necrosis cardial biopsy. factor receptor-associated periodic ❚ Treatment. Treatment is focused on syndrome [TRAPS], familial Mediter- 2 goals: treating the specific etiology sus- ranean fever), pected and stabilizing any hemodynamic • post-cardiac syndromes (either instability. Patients with mild cases can be of the acute [2-4 days post injury] or treated and monitored in the outpatient late [Dressler syndrome] variety), setting. • metabolic disorders (hypothyroid dis- Immunosuppressive therapy with im- ease, dialysis-related conditions), and munoglobulin or steroids is not routinely rec- • malignancy. ommended, but a trial may be considered in children, patients with severe hemodynamic More than 80% of pericarditis cases in compromise, or patients with giant cell arteri- developed countries are idiopathic and are tis, another autoimmune condition, sarcoid- assumed to have a viral source.8 osis, or eosinophilic or non-viral myocarditis. ❚ Diagnosis. Acute pericarditis is diag- ❚ Because of the risk of sudden death nosed when 2 or more of the following symp- from ventricular , any patient toms are present: with cardiac symptoms such as chest pain, • pleuritic chest pain radiating to the dyspnea, or palpitations should be admitted

JFPONLINE.COM VOL 65, NO 8 | AUGUST 2016 | THE JOURNAL OF FAMILY PRACTICE 527 Pulmonary causes Shortness of breath is common with most The variable, and subjective, pulmonary diseases, although it may not process of dyspnea be an initial symptom and may have an in- sidious onset. It occurs once oxygenation of The of shortness of breath is a complex and blood becomes inadequate, resulting in pe- incompletely understood one that involves the central and pe- ripheral nervous system activation and neu- ripheral nervous systems and neurochemical modulators. In the rochemical dissociation. Most patients with central nervous system, the medullary respiratory center likely relays increased oxygen demand to the anterior insula. The anterior insula, a pulmonary , asthma exacerbation, which is where dyspnea is perceived as unpleasant, then simultane- or COPD will have dyspnea. Once infection, ously disseminates this information to the cerebral cortex and the asthma, and COPD have been ruled out, respiratory muscles to increase respiration and oxygen.1-3 other pathologic processes that interrupt oxy- genation should be considered. Unlike COPD The peripheral nervous system measures current oxygen flux and lung mechanics through pulmonary stretch mechanoreceptors, and , patients with may pulmonary irritant receptors, and alveolar C fibers. Input from all not have dyspnea until the end stages of their 11 of these receptors ascends the respiratory pathway and affects how disease. The following entities should be dyspnea is perceived. For example, a patient may complain of short- considered in patients with dyspnea when ness of breath because the medullary respiratory center interprets more common causes have been eliminated. input from activated pulmonary muscular stretch receptors in the setting of discordant oxygen (measured via peripheral chemorecep- Restrictive lung diseases tors) and carbon dioxide levels (measured by medullary chemorecep- occurs when func- 2,4,5 tors) as an increased work of breathing. tional lung volume is decreased, either by Neurochemical dissociation, which is the difference between the an intrinsic or extrinsic source. As a result, brain’s desired oxygen level and the amount it gets, is one potential these lung diseases cover a wide variety of hypothesis to explain why dyspnea is subjective and variable.2,5 One pathologies and disease processes including patient may complain of moderate or severe shortness of breath be- interstitial lung diseases (which we’ll discuss cause he or she has a large dissociation between desired and actual here), environmental exposures, neuromus- oxygenation despite having only mild to moderate disease severity. cular diseases, and other forms of chest wall However, another patient may report mild dyspnea despite having severe disease because his or her dissociation is small. dysfunction. ❚ Interstitial lung disease occurs in the Take, for example, a patient who has had an acute myocardial presence of lung parenchymal scarring or infarction. Such patients often complain of significant difficulty thickening, which can have many causes in- breathing, likely because of the acute and sudden neurochemical cluding pulmonary , connective tissue dissociation that occurs with the infarction. On the other hand, a patient with gradually worsening moderate heart failure may diseases (eg, or rheumatoid ar- complain of only mild dyspnea because the change in the patient’s thritis), and inflammatory processes (eg, hy- perception of the ability to breathe is slow and small. persensitivity and worker's ). Dyspnea results because parenchymal thickening decreases oxygen diffusion between the alveolar and for cardiopulmonary monitoring. Patients endothelium. Additionally, the lung’s ability with heart failure secondary to myocarditis to exchange air is restricted by parenchymal should be treated according to the American stiffness and decreased total lung and func- Heart Association treatment guidelines for tional lung capacity. Treatment is disease heart failure (available at: http://circ.aha- specific. journals.org/content/128/16/e240.extract). Idiopathic is the Some patients may benefit from surgical in- most common interstitial pneumonia with a terventions such as percutaneous cardiopul- prevalence of 13 to 20 per 100,000 people.12 It monary support, extracorporeal membrane commonly affects men between the ages of oxygenation, mechanical circulatory support, 50 and 75 years. Risk factors include and left ventricular assistive devices. Ventric- smoking, exposure (to metals, woods, ular arrhythmias may require implantable vegetables), and exposure to livestock or defibrillators or pacemakers.10 other animals.12 Suspect it when you have a

528 THE JOURNAL OF FAMILY PRACTICE | AUGUST 2016 | VOL 65, NO 8 SHORTNESS OF BREATH

middle-aged farmer or mill worker who com- TABLE 1 plains of shortness of breath. Differential diagnosis for shortness of breath ❚ Treatment recommendations have changed recently and now consist of using Cardiac Other only nintedanib (a tyrosine-kinase inhibi- • Arrhythmias (, • Allergic reaction/ paroxysmal ventricular tor), antacid , and pirfenidone. • Deconditioning Anticoagulation (with warfarin), steroids, ) • Drug overdose other immunologic agents including aza- • Cardiomyopathy • Iatrogenic/environmental causes • CHF (systolic, diastolic) thioprine, receptor antagonists, (drug reaction, Farmer’s lung, and -5 inhibitors are not • Coronary artery disease/MI chemical exposure)* 13 recommended. • Myocarditis* • Otorhinolaryngeal disorders (vocal cord spasm, throat cancer) • Other Pulmonary arterial hypertension (atrial/ventricular septal defect, • Uncontrolled pain hypertrophic cardiomyopathy) and cor pulmonale Psychiatric Pulmonary arterial hypertension (PAH) • Pericarditis* • /panic disorder is defined as a mean resting precapillary • Valvular dysfunction pressure >25 mm Hg or • Conversion disorder Hematologic, oncologic, and • Malingering >30 mm Hg with activity. It can be idiopathic metabolic Pulmonary or caused by a variety of agents, diseases, and • /thalassemia/sickle cell 14 conditions (TABLE 3 ). PAH is rare (15 in one disease* • Asthma TK million adults) and underdiagnosed, and • Cancer (lung, leukemia,* • COPD more often occurs in 20- to 30-year-old black lymphoma, oral, throat, other) 14 • women. • Metabolic /alkalosis Suspect PAH in younger, otherwise • Interstitial lung disease/ Infectious pulmonary diffusing problem* healthy patients who complain of exertional • • Lung cancer dyspnea, fatigue, chest pain, or palpitations who do not have any other heart or lung dis- • Coccidioidomycosis • Obstructive sleep ease signs or symptoms. A diagnosis of PAH • • // pulmonary effusion/trauma is often delayed because patients are worked • Pneumonia • Pulmonary emboli up for other etiologies such as CHF, coronary • Severe inflammatory response artery disease, PE, and COPD. syndrome/ • / cor pulmonale* ❚ Diagnosis. When PAH is suspected, the • Tuberculosis initial work-up should include: Neuromuscular • Restrictive lung disease* • an echocardiogram with a possible • • /marijuana/e-cigarette bubble study, • Muscular dystrophy • arterial blood gas measurements, • complete blood count, • * • complete metabolic panel, • Parkinson’s disease • human immunodeficiency (HIV) • Transverse myelitis

testing, CHF, congestive heart failure; COPD, chronic obstructive pulmonary disease; MI, myocardial • thyroid-stimulating hormone levels, infarction. • chest x-ray (which is abnormal in 90% *Discussed in article. of patients and shows right ventricu- lar enlargement, a prominent cen- If clinically suggested, tests for anticen- tral pulmonary artery, or peripheral tromere , antinuclear , hypovascularity),14 anti-Scl-70 antibodies, and ribonucleopro- • electrocardiogram (to rule out other tein antibodies should be ordered, as well acute cardiac etiologies, but not to di- as sickle cell screening, cardiac magnetic agnosis PAH because of poor sensitiv- resonance imaging, and chest computed to- ity and specificity), mography. A right heart catheterization is • liver , and required to confirm PAH and determine dis- • pulmonary function tests. ease severity. CONTINUED

JFPONLINE.COM VOL 65, NO 8 | AUGUST 2016 | THE JOURNAL OF FAMILY PRACTICE 529 TABLE 2 simple test you can perform in the office by Myocarditis: Possible causes9,10 measuring how far your patient can walk in 6 minutes. Miyamoto et al showed the test to Cardiotoxins be predictive of survival in idiopathic PAH.15 A is never indicated or needed Carbon monoxide for diagnosis, disease severity classification, or prognosis. ❚ Treatment. Collaboration between Heavy metals (copper, mercury, lead) primary and subspecialty physicians is usu- Drug reactions ally recommended because PAH treatment requires advanced testing such as right heart catheterization or vasoreactivity testing. Re- Lithium search has shown anticoagulation with war- Penicillin farin prolongs survival and improves quality 16 Sulfonamide of life. Oxygen may improve symptomatic control and should be started for anyone with Vaccinations saturation less than 90%. Infectious Newer that target vari- Adenovirus ous pathways resulting in in- The 6-minute Babesia clude analogues (epoprostenol, walk test is Campylobacter , ), endothelin receptor the best way Chagas disease antagonists (, ), and to estimate phosphodiesterase type 5 inhibitors (silde- Chlamydia prognosis and nafil, ).14 disease severity Cholera in patients with Cytomegalovirus pulmonary Enterovirus Hematologic diseases arterial Epstein-Barr virus Hematologic diseases, including sickle cell hypertension. Fungi disease, gammopathies, and malignancies, Human immunodeficiency virus can cause dyspnea primarily by decreasing the body’s ability to transport oxygen. This Influenza usually is due to anemia, but it also can be Malaria caused by increased viscosity or sickling. Sus- pect a hematologic cause of dyspnea when a Salmonella patient repeatedly returns to your office com- Staphylococcus plaining of progressive dyspnea on exertion Tuberculosis and possible Raynaud’s-like symptoms. Varicella Yellow fever Sickle cell disease is a heterogeneous genetic disease with varied physical manifestations. Rheumatologic The sickling phenomenon occurs in patients Kawasaki disease who inherit the homozygous hemoglobin S trait or heterozygous hemoglobin S and C (hemo- Sarcoidosis globin SC) disease. Sickle cell patients develop dyspnea due to comorbid anemia, infectious processes, or cardiopulmonary disease. Vasoreactivity testing helps guide treat- Cardiac disease is common and an often ment because it identifies which patients unrecognized comorbidity. It is the leading will benefit from calcium channel blockers. cause of mortality in adults with sickle cell The 6-minute walk test is the best way to es- disease, resulting in 26% of deaths (usually timate prognosis and disease severity. It is a from pulseless electrical activity, pulmonary

530 THE JOURNAL OF FAMILY PRACTICE | AUGUST 2016 | VOL 65, NO 8 SHORTNESS OF BREATH

emboli, multiorgan failure, or stroke).17 Non- TABLE 3 fatal cardiac complications may also develop, Possible causes of pulmonary including chronic heart disease from pro- 14 longed increased cardiac output (leading to arterial hypertension ventricular ), heart failure, or ar- Congenital heart disease 18 rhythmias; non-atherosclerotic MI; and he- Connective tissue diseases mosiderosis-induced cardiomyopathy from Mixed connective tissue disease repeat blood transfusions. Pulmonary-related complications may Rheumatoid arthritis be chronic or acute and may include re- strictive lung disease, chronic , Sjögren’s syndrome pulmonary hypertension, and interstitial fi- Systemic lupus erythematosus brosis. and cor pulmo- Drugs nale cause sudden pulmonary disease. Acute Aminorex chest syndrome is often caused by pneumo- nia, in situ infarction of the lung, or embolic infarction from or bone mar- row. It is a medical emergency that should Chemotherapy be considered in any patient with pulmonary symptoms, fever, chest pain, or cough and an Dexfenfluramine Suspect a infiltrate on chest x-ray. hematologic Treatment for acute chest syndrome L-tryptophan cause of consists of oxygen, aggressive analgesia, an- dyspnea when tibiotics (if infection is suspected), and trans- a patient fusions. Research has shown that steroids Genetic mutations repeatedly provide improvement, but result in more Activin receptor-like kinase 1 returns to your hospital readmissions.19 Bone morphogenetic protein receptor type II office Caveolin-1 complaining Multiple myeloma and other Decapentaplegic 9 of progressive hematologic malignancies Endoglin dyspnea Multiple myeloma and Waldenstrom macro- on exertion globulinemia (discussed here), as well as leu- Potassium channel super family K member-9 and possible kemia, and other hematologic malignancies, HIV infection Raynaud's-like can cause dyspnea or dyspnea on exertion Persistent pulmonary hypertension of the symptoms. through anemia, increasing blood viscosity, newborn or direct lung involvement. ❚ Multiple myeloma, a plasma cell neo- plasm, is associated with anemia in 73% of Sickle cell anemia 20 patients at time of diagnosis. This is because Toxins of bone marrow destruction. Anemia preva- Rapeseed oil lence increases in patients treated with che- motherapy because of the agent's adverse effects. The decision to treat with irradiated, leukoreduced red cell transfusion is based on other , and/or direct lung involve- anemia severity, the presence of symptoms, ment including , pulmonary and whether the patient is currently undergo- infiltrates, or a mass. ing chemotherapy. Hyperviscosity syndrome usually re- ❚ Waldenstrom macroglobulinemia is sults in neurologic symptoms such as vision an IgM-specific monoclonal gammopathy changes, headaches, vertigo, dizziness, demen- associated with a lymphoplasmacytic lym- tia, or other changes in consciousness. Heart phoma in the bone marrow. Dyspnea results failure, which is often associated with comor- from hyperviscosity syndrome, hemolytic or bid anemia, can develop in severe cases. CONTINUED

JFPONLINE.COM VOL 65, NO 8 | AUGUST 2016 | THE JOURNAL OF FAMILY PRACTICE 531 Patients are generally asymptomatic if The most reliable test is the anti-AChR an- serum viscosity is <3 centipoises (cP). Symp- tibody assay, which is positive in 50% to 90% toms increase in frequency and severity with of patients with the disease.22 Less reliable increasing serum viscosity so that about two- is the anti-MuSK antibody assay, which can thirds (67%) of patients have symptoms when be positive in 40% to 60% of patients who are viscosity is >4 cP and 75% have symptoms AChR-seronegative.23 An anti-striated mus- when viscosity is >5 cP.21 cle antibody assay is only helpful in patients with or onset of disease after age 40 years.24 Neuromuscular diseases Consider electrophysiologic tests, in- Dyspnea occurs when respiratory muscles are cluding repetitive nerve stimulation stud- weakened by neuromuscular diseases such ies and single-fiber electromyography, if the as myasthenia gravis (discussed here), mul- above laboratory tests are inconclusive.25 tiple sclerosis, or muscular dystrophy. Such ❚ Treatment depends on symptom se- diseases can cause respiratory insufficiency, verity and frequency. It can range from ob- increased rates of infection, or complete re- servation for mild occasional symptoms to spiratory failure. Respiratory involvement is chronic steroids and immunosuppressant usually a manifestation of advanced disease. medications in severe cases. Suspect neuromuscular causes of dyspnea Suspect when you are seeing a patient admitted to the CASE uYou see Ms. C in the intensive care unit neuromuscular nursing home for long-term care because of the next day. She is intubated and has been causes of profound weakness affecting their ability to responding poorly to the diuresis and breath- dyspnea when do activities of daily living. ing treatments used overnight. Her biopsy pa- a patient is thology results return and show recurrence of admitted to a Myasthenia gravis her small-cell lung cancer. She begins chemo- nursing home Myasthenia gravis, an autoimmune-mediated therapy immediately and is extubated a few because destruction of the postsynaptic acetylcholine days later. She is discharged from the hospital of profound receptors of the neuromuscular junction, is a week later. Her shortness of breath is mild at weakness the most common disorder of neuromuscu- this time, although she does require 2 liters of affecting his or lar transmission. It often affects the ocular continuous oxygen. JFP her ability to (>50%; ptosis, diplopia), bulbar (15%; dys- perform activities arthria, dysphagia, fatigable chewing), limb CORRESPONDENCE Christopher Taggart, MD, St. Mary’s Medical Center, Depart- of daily living. (<5%; usually proximal weakness), and respi- ment of Family Medicine, 2698 Patterson Rd, Grand Junction, ratory muscles. Weakness typically fluctuates CO 81506; [email protected]. and worsens with muscle fatigue. Myasthenic crisis, an acute that occurs in 15% to 20% of patients, is often precipitated References by an event such as surgery, an infection, or a 1. von Leupoldt A, Sommer T, Kegat S, et al. The unpleasantness of 22 perceived dyspnea is processed in the anterior insula and amyg- medication change. dala. Am J Resp Crit Care Med. 2008;177:1026-1032. ❚ Diagnosis. Myasthenia gravis is diag- 2. Thoma J, Gunten CV. Dyspnea. In: Bruera E, Higginson IJ, Ri- pamonti C, et al, eds. Textbook of Palliative Medicine. London: nosed by a clinical history and exam sug- Hodder Arnold; 2009. gestive of the disease. Suspect it if signs and 3. Manning H, Schwartzstein R. Pathophysiology of dyspnea. N Engl symptoms include weakness worse with fa- J Med. 1995;333;1547-1553. 4. O’Donnell DE, Bain DJ, Webb KA. Factors contributing to relief tigue especially of the ocular muscles (ptosis of exertional breathlessness during hyperoxia in chronic airflow or diplopia), dysphagia, dysphonia, chewing limitation. Am J Respir Crit Care Med. 1997;155:530-535. 5. O’Donnell DE, Webb KA. Exertional breathlessness in patients difficulty, or limb weakness. Consider labo- with chronic airflow limitation. The role of lung hyperinflation. ratory testing with an anti-acetylcholine re- Am Rev Respir Dis. 1993;148:1351-1357. 6. Seferovic PM, Ristic AD, Maksimovic R, et al. Pericardial syn- ceptor (AChR) antibody assay, an assay for dromes: an update after the ESC guidelines 2004. Heart Fail Rev. 2013;18:255-266. muscle-specific kinase (MuSK) antibody, or 7. Kytö V, Sipilä J, Rautava P. Clinical profile and influences on out- an anti-striated muscle (anti-SM) antibody comes in patients hospitalized for acute pericarditis. Circulation. 2014;130:1601-1606. assay if the history and exam are suggestive 8. LeWinter MM. Acute pericarditis. N Engl J Med. 2014;371:2410- of the disorder. 2416.

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9. Pursnani A, Yee H, Slater W, et al. myocardi- tis associated with azithromycin exposure. Ann Intern Med. 2009;150:225-226. 10. Sagar S, Liu PP, Cooper LT Jr. Myocarditis. Lancet. 2012;379:738- 747. 11. Gore JM, Brophy CJ, Greenstone MA. How well do we care for patients with end stage chronic obstructive pulmonary disease (COPD)? A comparison of palliative care and quality of life in USPSTF's colorectal cancer COPD and lung cancer. . 2000;55:1000-1006. 12. King TE Jr, Pardo A, Selman M. Idiopathic pulmonary fibrosis. screening recs: How the final Lancet. 2011;378:1949-1961. 13. Raghu G, Rochwerg B, Zhang Y, et al. An official ATS/ERS/JRS/ differs from the draft ALAT clinical practice guideline: treatment of idiopathic pulmo- nary fibrosis. An update of the 2011 clinical practice guideline.  Doug Campos-Outcalt, MD, MPA Am J Respir Crit Care Med. 2015;192:e3-e19. 14. Stringham R, Shah NR. Pulmonary arterial hypertension: an up- date on diagnosis and treatment. Am Fam Physician. 2010;82:370- 377. 15. Miyamoto S, Nagaya N, Satoh T, et al. Clinical correlate and prognostic significance of six-minute walk test in patients with primary pulmonary hypertension. Am J Respir Crit Care Med. 2000;161:487-492. 16. Frank H, Mlczoch J, Huber K, et al. The effect of therapy in primary and drug-induced pulmonary hy- pertension. Chest. 1997;112:714-721. 17. Fitzhugh CD, Lauder N, Jonassaint JC, et al. Cardiopulmonary complications leading to premature deaths in adult patient with sickle cell disease. Am J Hematol. 2010;85:36-40. 18. Martin CR, Johnson CS, Cobb C, et al. D. in sickle cell disease. J Natl Med Assoc. 1996;88:428-432. INSTANT POLL 19. Paul RN, Castro OL, Aggarwal A, et al. Acute chest syndrome: sickle cell disease. Eur J Haematol. 2011;87:191-207. Do you perform ultrasound exams in your office? 20. Palumbo A, Anderson K. Multiple myeloma. N Engl J Med. 2011;364:1046-1060. 21. Crawford J, Cox EB, Cohen HJ. Evaluation of hyperviscosity in monoclonal gammopathies. Am J Med. 1985;79:13-22. 22. Silvestri NJ, Wolfe GI. Myasthenia gravis. Semin Neurol. ONLINE EXCLUSIVES 2012;32;215-226. 23. Guptill JT, Sanders DB. Update on muscle-specific tyrosine ki- • CASE REPORT nase antibody positive myasthenia gravis. Curr Opin Neurol. 2010;23:530-535. Severe pruritus • crusted lesions affecting face, 24. Skeie GO, Mygland A, Aarli JA, et al. Titin antibodies in patients extremities, and trunk • hepatitis C virus with late onset myasthenia gravis: clinical correlations. Autoim- carrier • Dx? munity. 1995;20:99-104. 25. Benatar M. A systematic review of diagnostic studies in myasthe- nia gravis. Neuromuscul Disord. 2006;16:459-467. • RESIDENTS RAPID REVIEW A 5-question monthly quiz to help you prepare for the family medicine certification exam. This month: USPSTF recommendations WE WANT TO HEAR FROM YOU! PHOTO ROUNDS FRIDAY  Test your diagnostic skills at www.jfponline.com/ Have a comment on an article, edi- articles/photo-rounds-friday.html torial, or department? You can send it by: PLUS  Today’s headlines in family medicine 1. E-MAIL: [email protected] 2. FAX: 973-206-9251 or 3. MAIL: The Journal of Family Practice, GET UPDATES FROM US ON 7 Century Drive, Suite 302, Parsippany, FACEBOOK TWITTER GOOGLE+

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