Cystic fibrosis differential diagnosis: Difference between revisions

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==Cough==
==Cough==
 
Cystic fibrosis must be differentiated from other diseases presenting with cough and wheeze.


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Revision as of 19:09, 7 March 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shaghayegh Habibi, M.D.[2]

Overview

Cystic fibrosis has to be differentiated from other conditions with similar presentation of cough and wheeze like asthma, bronchiolitis, COPD, bacterial pneumonia, emphysema, primary ciliary dyskinesia (Kartagener syndrome) and Alpha 1-antitrypsin deficiency.

Differentiating cystic fibrosis from other Diseases

Differential diagnosis of cough with wheezes is :

Diseases Symptoms Signs Diagosis
Fever Cough Chest pain Wheezes Crackles Tachypnea Lab tests Imaging
Asthma - Dry/Productive - + - +
  • CT scan shows:
    • Dilated bronchi.
    • Bronchial wall thickening.
    • Air trapping.
Bronchiolitis +/- Dry - + + +/-
COPD + Productive - + + +
Bacterial pneumonia + Productive + + + +/-
Cystic Fibrosis +/- Productive +/- - - + Cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction evidenced by : X-ray :

Hyperinflation presents as:

  • Anterior bowing of the infant sternum.
  • Increased retrosternal air space.
  • Generalized pulmonary overinflation.
  • Multiple nodular densities represent mucus plugging and may present in finger-in-glove shape or as a combination of V- or Y-shaped branching and bandlike shadows.

Abdominal findings include dilated multiple loops of the small bowel are seen in neonatal meconium ileus.

Emphysema +/- Productive - + +/- + Chest X-ray reveals signs of emphysema include:
  • Increased retrosternal air space (see on lateral chest films).
  • A long narrow heart shadow.
  • Tapering vascular shadows.
  • Hyperlucency of the lungs.
Primary Ciliary Dyskinesia (Kartagener Syndrome) +/- Productive - + + + Chest X-ray reveals :
Alpha 1-antitrypsin deficiency +/- Productive - + + + Chest X-ray Alpha1-antitrypsin deficiency (AATD) emphysema presents as:
  • a hyperlucent appearance because healthy tissue has been destroyed.
  • Affected regions also are described as oligemic because they lack the normal rich pattern of branching blood vessels.
  • An unusual characteristic in alpha1-antitrypsin deficiency is found in about 60% of PiZZ patients is a striking basilar distribution.
  • In contrast, cigarette smoking is associated with more severe apical disease.

Cough

Cystic fibrosis must be differentiated from other diseases presenting with cough and wheeze.

Organ system Diseases Clinical manifestations Diagnosis Other features
Symptoms Physical exam
Onset Duration Productive cough Hemoptysis Weight lost Fever Dyspnea Ascultation Lab findings Imaging PFT Gold standard
Respiratory Upper airway diseases Common Cold[3] Acute
  • 3-10 days
- - -
  • Bacterial culture is not indicated
  • Normal function
  • Clinical diagnosis
Lower airway Asthma[4] Chronic
  • Years
✔ Clear mucoid or yellow sputum - - -
  • Family history
  • Seasonal variation
Acute Bronchitis[5] Acute
  • From 5 days to 1 or 3 weeks
- - -
  • FEV1 < 80%
  • Clinical diagnosis
Chronic Bronchitis[6][7] Chronic
  • Most of the days for three months in the las two years.
✔ Clear sputum - -
Non-asthmatic eosinophilic bronchitis[8][9] Chronic
  • More than 8 weeks
Eosinophilic sputum - -
  • Exposure to an occupational cause
Bronchiectasis[10] Chronic
  • Months to years
✔ Mucopurulent sputum -
  • CT of chest
Emphysema [11] Chronic
  • Months to years
✔ Mucoid or purulent sputum - -
  • Exposure of tobacco and air pollution
Foreing body aspiration[12][13][14] Acute
  • Variable
-
  • No specific
  • Not specific
  • In children <1 year and adults >75 years
  • Organic materials in children
  • Inorganic materials in adults
Bronchiolitis[15][16] Acute
  • 8-15 days
-
  • Clinical diagnosis
Parenchyma Pneumoconioses[17][18] Acute, Chronic
  • Years
- -
Interstitial lung disease[19][20] Chronic
  • Variable
- -
  • Lung biopsy when lab, imaging, and PFT has indeterminate result
Cystic fibrosis (CF)[21][22] Chronic
  • Variable
-
  • Evidence of CFTR dysfunction
Cardiac Cardiogenic pulmonary edema[23][24] Acute
  • Days to weeks
✔ Pink frothy, liquid - -
  • Not specific
  • Clinical diagnosis
  • Tests are supportive
Gastrointestinal Gastroesophageal reflux[25][26] Chronic
  • Variable
- -
  • Not specific
  • Normal function
  • PH testing
--
Autoinmune Wegener's disease (GPA) [27][28] Chronic
  • Months
Sarcoidosis[29][30] Chronic
  • Years
- -
Microscopic polyangitis (MPA)[31] Chronic
  • Variable
Churg-Strauss[32][33] Chronic
  • Variable
  • Infiltrates in chest X-Ray
  • Ground glass opacities, tree-in-bud sign and small nodules in chest CT
Medication ACE inhibitors[34][35] Acute (depend on the medication)
  • From 2 weeks to 6 months
- - - -
  • Not required
  • No required
  • Normal function
  • Clinical diagnosis
  • Resolves in four to five days of stopping the medication
  • Angioedema

References

  1. Ghanei M, Tazelaar HD, Chilosi M, Harandi AA, Peyman M, Akbari HM; et al. (2008). "An international collaborative pathologic study of surgical lung biopsies from mustard gas-exposed patients". Respir Med. 102 (6): 825–30. doi:10.1016/j.rmed.2008.01.016. PMID 18339530.
  2. Lazović B, Svenda MZ, Mazić S, Stajić Z, Delić M (2013). "Analysis of electrocardiogram in chronic obstructive pulmonary disease patients". Med Pregl. 66 (3–4): 126–9. PMID 23653989.
  3. Eccles R (2005). "Understanding the symptoms of the common cold and influenza". Lancet Infect Dis. 5 (11): 718–25. doi:10.1016/S1473-3099(05)70270-X. PMID 16253889.
  4. Ukena D, Fishman L, Niebling WB (2008). "Bronchial asthma: diagnosis and long-term treatment in adults". Dtsch Arztebl Int. 105 (21): 385–94. doi:10.3238/arztebl.2008.0385. PMC 2696883. PMID 19626179.
  5. Wenzel RP, Fowler AA (2006). "Clinical practice. Acute bronchitis". N. Engl. J. Med. 355 (20): 2125–30. doi:10.1056/NEJMcp061493. PMID 17108344.
  6. Brusasco V, Martinez F (2014). "Chronic obstructive pulmonary disease". Compr Physiol. 4 (1): 1–31. doi:10.1002/cphy.c110037. PMID 24692133.
  7. Qaseem A, Snow V, Shekelle P, Sherif K, Wilt TJ, Weinberger S, Owens DK (2007). "Diagnosis and management of stable chronic obstructive pulmonary disease: a clinical practice guideline from the American College of Physicians". Ann. Intern. Med. 147 (9): 633–8. PMID 17975186.
  8. Brightling CE (2006). "Chronic cough due to nonasthmatic eosinophilic bronchitis: ACCP evidence-based clinical practice guidelines". Chest. 129 (1 Suppl): 116S–121S. doi:10.1378/chest.129.1_suppl.116S. PMID 16428700.
  9. Cho J, Choi SM, Lee J, Park YS, Lee SM, Yoo CG; et al. (2018). "Clinical Outcome of Eosinophilic Airway Inflammation in Chronic Airway Diseases Including Nonasthmatic Eosinophilic Bronchitis". Sci Rep. 8 (1): 146. doi:10.1038/s41598-017-18265-2. PMC 5760521. PMID 29317659.
  10. King PT, Holdsworth SR, Freezer NJ, Villanueva E, Holmes PW (2006). "Characterisation of the onset and presenting clinical features of adult bronchiectasis". Respir Med. 100 (12): 2183–9. doi:10.1016/j.rmed.2006.03.012. PMID 16650970.
  11. Rossi A, Butorac-Petanjek B, Chilosi M, Cosío BG, Flezar M, Koulouris N; et al. (2017). "Chronic obstructive pulmonary disease with mild airflow limitation: current knowledge and proposal for future research - a consensus document from six scientific societies". Int J Chron Obstruct Pulmon Dis. 12: 2593–2610. doi:10.2147/COPD.S132236. PMC 5587130. PMID 28919728.
  12. Hewlett JC, Rickman OB, Lentz RJ, Prakash UB, Maldonado F (2017). "Foreign body aspiration in adult airways: therapeutic approach". J Thorac Dis. 9 (9): 3398–3409. doi:10.21037/jtd.2017.06.137. PMC 5708401. PMID 29221325.
  13. Rafanan AL, Mehta AC (2001). "Adult airway foreign body removal. What's new?". Clin. Chest Med. 22 (2): 319–30. PMID 11444115.
  14. Haddadi S, Marzban S, Nemati S, Ranjbar Kiakelayeh S, Parvizi A, Heidarzadeh A (2015). "Tracheobronchial Foreign-Bodies in Children; A 7 Year Retrospective Study". Iran J Otorhinolaryngol. 27 (82): 377–85. PMC 4639691. PMID 26568942.
  15. Bordley WC, Viswanathan M, King VJ, Sutton SF, Jackman AM, Sterling L, Lohr KN (2004). "Diagnosis and testing in bronchiolitis: a systematic review". Arch Pediatr Adolesc Med. 158 (2): 119–26. doi:10.1001/archpedi.158.2.119. PMID 14757603.
  16. "www.nice.org.uk".
  17. Jp NA, Imanaka M, Suganuma N (2017). "Japanese workplace health management in pneumoconiosis prevention". J Occup Health. 59 (2): 91–103. doi:10.1539/joh.16-0031-RA. PMC 5478517. PMID 27980247.
  18. Weiland DA, Lynch DA, Jensen SP, Newell JD, Miller DE, Crausman RS, Kuhn C, Kern DG (2003). "Thin-section CT findings in flock worker's lung, a work-related interstitial lung disease". Radiology. 227 (1): 222–31. doi:10.1148/radiol.2271011063. PMID 12668748.
  19. Lama VN, Martinez FJ (2004). "Resting and exercise physiology in interstitial lung diseases". Clin. Chest Med. 25 (3): 435–53, v. doi:10.1016/j.ccm.2004.05.005. PMID 15331185.
  20. Chetta A, Marangio E, Olivieri D (2004). "Pulmonary function testing in interstitial lung diseases". Respiration. 71 (3): 209–13. doi:10.1159/000077416. PMID 15133338.
  21. Farrell PM, Rosenstein BJ, White TB, Accurso FJ, Castellani C, Cutting GR, Durie PR, Legrys VA, Massie J, Parad RB, Rock MJ, Campbell PW (2008). "Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report". J. Pediatr. 153 (2): S4–S14. doi:10.1016/j.jpeds.2008.05.005. PMC 2810958. PMID 18639722.
  22. Kerem E, Reisman J, Corey M, Canny GJ, Levison H (1992). "Prediction of mortality in patients with cystic fibrosis". N. Engl. J. Med. 326 (18): 1187–91. doi:10.1056/NEJM199204303261804. PMID 1285737.
  23. Gheorghiade M, Zannad F, Sopko G, Klein L, Piña IL, Konstam MA, Massie BM, Roland E, Targum S, Collins SP, Filippatos G, Tavazzi L (2005). "Acute heart failure syndromes: current state and framework for future research". Circulation. 112 (25): 3958–68. doi:10.1161/CIRCULATIONAHA.105.590091. PMID 16365214.
  24. Yancy CW, Jessup M, Bozkurt B, Butler J, Casey DE, Drazner MH, Fonarow GC, Geraci SA, Horwich T, Januzzi JL, Johnson MR, Kasper EK, Levy WC, Masoudi FA, McBride PE, McMurray JJ, Mitchell JE, Peterson PN, Riegel B, Sam F, Stevenson LW, Tang WH, Tsai EJ, Wilkoff BL (2013). "2013 ACCF/AHA guideline for the management of heart failure: a report of the American College of Cardiology Foundation/American Heart Association Task Force on practice guidelines". Circulation. 128 (16): e240–327. doi:10.1161/CIR.0b013e31829e8776. PMID 23741058.
  25. Kahrilas PJ, Hughes N, Howden CW (2011). "Response of unexplained chest pain to proton pump inhibitor treatment in patients with and without objective evidence of gastro-oesophageal reflux disease". Gut. 60 (11): 1473–8. doi:10.1136/gut.2011.241307. PMID 21508423.
  26. Badillo R, Francis D (2014). "Diagnosis and treatment of gastroesophageal reflux disease". World J Gastrointest Pharmacol Ther. 5 (3): 105–12. doi:10.4292/wjgpt.v5.i3.105. PMC 4133436. PMID 25133039.
  27. Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, Rottem M, Fauci AS (1992). "Wegener granulomatosis: an analysis of 158 patients". Ann. Intern. Med. 116 (6): 488–98. PMID 1739240.
  28. Falk RJ, Gross WL, Guillevin L, Hoffman GS, Jayne DR, Jennette JC, Kallenberg CG, Luqmani R, Mahr AD, Matteson EL, Merkel PA, Specks U, Watts RA (2011). "Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis". Arthritis Rheum. 63 (4): 863–4. doi:10.1002/art.30286. PMID 21374588.
  29. Carmona EM, Kalra S, Ryu JH (2016). "Pulmonary Sarcoidosis: Diagnosis and Treatment". Mayo Clin. Proc. 91 (7): 946–54. doi:10.1016/j.mayocp.2016.03.004. PMID 27378039.
  30. Yanardağ H, Pamuk GE, Karayel T, Demirci S (2002). "Bone marrow involvement in sarcoidosis: an analysis of 50 bone marrow samples". Haematologia (Budap). 32 (4): 419–25. PMID 12803116.
  31. Jennette, J. Charles; Falk, Ronald J. (1997). "Small-Vessel Vasculitis". New England Journal of Medicine. 337 (21): 1512–1523. doi:10.1056/NEJM199711203372106. ISSN 0028-4793.
  32. Vaglio A, Buzio C, Zwerina J (2013). "Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): state of the art". Allergy. 68 (3): 261–73. doi:10.1111/all.12088. PMID 23330816.
  33. Lanham JG, Elkon KB, Pusey CD, Hughes GR (1984). "Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome". Medicine (Baltimore). 63 (2): 65–81. PMID 6366453.
  34. Israili ZH, Hall WD (1992). "Cough and angioneurotic edema associated with angiotensin-converting enzyme inhibitor therapy. A review of the literature and pathophysiology". Ann. Intern. Med. 117 (3): 234–42. PMID 1616218.
  35. Wood R (1995). "Bronchospasm and cough as adverse reactions to the ACE inhibitors captopril, enalapril and lisinopril. A controlled retrospective cohort study". Br J Clin Pharmacol. 39 (3): 265–70. PMC 1365002. PMID 7619667.

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