Lady Windermere syndrome

Infobox_Disease
Name = Lady Windermere syndrome


Caption = CT scan of patient with right middle lobe aspiration and Mycobacterium avium infection consistent with Lady Windermere syndrome
DiseasesDB = 29182
ICD10 = ICD10|A|31|0|a|30
ICD9 = ICD9|031.0
ICDO =
OMIM =
MedlinePlus =
eMedicineSubj = med
eMedicineTopic = 1532
MeshID = D015270

Lady Windermere syndrome describes infection in the lungs due to "Mycobacterium avium" complex.cite journal |author=Reich JM, Johnson RE |title=Mycobacterium avium complex pulmonary disease presenting as an isolated lingular or middle lobe pattern. The Lady Windermere syndrome |journal=Chest |volume=101 |issue=6 |pages=1605–9 |year=1992 |month=Jun |pmid=1600780 |doi= |url=http://www.chestjournal.org/cgi/reprint/101/6/1605 ] It is named after a character in Oscar Wilde's play "Lady Windermere's Fan".cite book | last =Wilde | first =Oscar | authorlink =Oscar Wilde | coauthors = | title =The Importance of Being Earnest and Other Plays | publisher =Penguin | year =1940 | location = | pages = | url = | doi = | id = | isbn =0-14-048209-1 ]

Classification

Lady Windermere syndrome is a type of mycobacterial lung infection.cite journal |author= |title=Management of opportunist mycobacterial infections: Joint Tuberculosis Committee Guidelines 1999. Subcommittee of the Joint Tuberculosis Committee of the British Thoracic Society |journal=Thorax |volume=55 |issue=3 |pages=210–8 |year=2000 |month=Mar |pmid=10679540 |pmc=1745689 |doi= |url=http://thorax.bmj.com/cgi/pmidlookup?view=long&pmid=10679540]

Signs and symptoms

Patients with Lady Windermere syndrome experience chronic cough, shortness of breath, fatigue and other less specific symptoms.

Pathophysiology

Mycobacterium avium complex is the most commonly found form of non-tuberculous mycobacteria. [cite journal |author=Wickremasinghe M, Ozerovitch LJ, Davies G, "et al" |title=Non-tuberculous mycobacteria in patients with bronchiectasis |journal=Thorax |volume=60 |issue=12 |pages=1045–51 |year=2005 |month=Dec |pmid=16227333 |doi=10.1136/thx.2005.046631 |url=]

Immunodeficiency is not a requirement for Mycobacterium avium infection.cite journal |author=Martins AB, Matos ED, Lemos AC |title=Infection with the Mycobacterium avium complex in patients without predisposing conditions: a case report and literature review |journal=Braz J Infect Dis |volume=9 |issue=2 |pages=173–9 |year=2005 |month=April |pmid=16127595 |doi=/S1413-86702005000200009 |url=http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1413-86702005000200009&lng=en&nrm=iso&tlng=en]

"Mycobacterium avium" Complex (MAC) usually affects patients with abnormal lungs or bronchi. However, Jerome Reich and Richard Johnson describe a series of six patients with MAC infection of the right middle lobe or left lingula who did not have any predisposing lung disorders.

The right middle lobe and left lingula of the lungs are served by bronchi that are oriented downward when a person is in the upright position. As a result, these areas of the lung may be relatively more dependent upon vigorous voluntary expectoration (cough) for clearance of bacteria and secretions.

Since the six patients in their retrospective case series were older females, Reich and Johnson propose that patients without a vigorous cough may develop right middle lobe or left lingular infection with MAC. They propose that this syndrome be named Lady Windermere syndrome, after the character Lady Windermere in Oscar Wilde's play Lady Windermere's Fan.

Diagnosis

The diagnosis requires consistent symptoms with two additional signs:

*Chest x-ray or chest CT scan showing evidence of right middle lobe (or left lingular lobe) lung infection.
*Sputum culture or bronchoalveolar lavage culture demonstrating that the infection is caused by Mycobacterium avium complex (MAC).

Treatment

Lady Windermere syndrome is usually treated with a three-drug regimen of either clarithromycin or azithromycin, plus rifampicin and ethambutol. Treatment typically lasts at least 12 months.

Literary reference

The original "Chest" article proposing the existence and pathophysiology of the Lady Windermere syndrome suggests that the character Lady Windermere in Oscar Wilde's Victorian-era play Lady Windermere's Fan is a good example of the fastidious behavior believed to cause the syndrome. The article states:: We offer the term, Lady Windermere's Syndrome, from the Victorian-era play, Lady Windermere's Fan, to convey the fastidious behavior hypothesized: "How do you do, Lord Darlington. No, I can't shake hands with you. My hands are all wet with the roses."

Victorian women presumably believed that "Ladies don't spit," and consequently might have been predisposed to develop lung infection.

Shortly after the Lady Windermere syndrome was proposed, a librarian wrote a letter to the editor of "Chest"cite web |url=http://www.chestjournal.org/cgi/eletters/101/6/1605#85 |title=Chest -- eLetters for Reich and Johnson, 101 (6) 1605-1609 |format= |work= |accessdate=] challenging the use of Lady Windermere as the eponymous ancestor of the proposed syndrome. In Lady Windermere's Fan, Lady Windermere is a vivacious young woman, married only 2 years, who never coughs or displays any other signs of illness. While her avoidance of shaking hands might be interpreted as "fastidiousness," two alternative explanations may be just as probable: :1) Lady Windermere actually is in the midst of arranging flowers and consequently cannot properly greet her guest:

:: ["LADY WINDERMERE is at table R., arranging roses in a blue bowl".] cite web |url=http://wilde.thefreelibrary.com/Lady-Windermeres-Fan/1-1 |title=Oscar Wilde: Lady Windermere's Fan: ACT I. Morning-room in Lord Windermere's house. - Free Online Library |format= |work= |accessdate=]

:2) Lady Windermere wishes to discourage the flirtatious advances of her would-be suitor Lord Darlington and cites her wet hands as an excuse to keep him from touching her:

::"LADY WINDERMERE. Lord Darlington, you annoyed me last night at the Foreign Office. I am afraid you are going to annoy me again. . . .

::"LORD DARLINGTON. [Takes chair and goes across L.C.] I am quite miserable, Lady Windermere. You must tell me what I did. [Sits down at table L.]

::"LADY WINDERMERE. Well, you kept paying me elaborate compliments the whole evening.] "cite web |url=http://wilde.thefreelibrary.com/Lady-Windermeres-Fan/1-1 |title=Oscar Wilde: Lady Windermere's Fan: ACT I. Morning-room in Lord Windermere's house. - Free Online Library |format= |work= |accessdate=]

The OScholars highlight the literary malapropism, [cite web |url=http://ics.leeds.ac.uk/papers/ids/exhibits/250/Vol_III_No_9.doc |title=oscholars |format= |work= |accessdate=] but some in the medical community have adopted the term regardless, and peer-reviewed medical journals still sometimes mention the Lady Windermere syndrome.cite journal |author=Sexton P, Harrison AC |title=Susceptibility to nontuberculous mycobacterial lung disease |journal=Eur. Respir. J. |volume=31 |issue=6 |pages=1322–33 |year=2008 |month=June |pmid=18515557 |doi=10.1183/09031936.00140007 |url=http://erj.ersjournals.com/cgi/pmidlookup?view=long&pmid=18515557]

In recent years, some have described the eponym as inappropriate,cite journal |author=Kasthoori JJ, Liam CK, Wastie ML |title=Lady Windermere syndrome: an inappropriate eponym for an increasingly important condition |journal=Singapore Med J |volume=49 |issue=2 |pages=e47–9 |year=2008 |month=February |pmid=18301826 |doi= |url=http://smj.sma.org.sg/4902/4902cr6.pdf] and some have noted that it would have been unlikely that Lady Windermere had the condition to which her name was assigned.cite journal |author=Rubin BK |title=Did Lady Windermere have cystic fibrosis? |journal=Chest |volume=130 |issue=4 |pages=937–8 |year=2006 |month=October |pmid=17035420 |doi=10.1378/chest.130.4.937 |url=http://www.chestjournal.org/cgi/pmidlookup?view=long&pmid=17035420]

ee also

* paratuberculosis

References