Mycobacterium avium Complex Pulmonary Disease Presenting as an Isolated Lingular or Middle Lobe Pattern: The Lady Windermere Syndrome

doi:10.1378/chest.101.6.1605

Chest

Volume 101, Issue 6, June 1992, Pages 1605-1609

https://doi.org/10.1378/chest.101.6.1605 Get rights and content

Background

Pulmonary disease due to Mycobacterium avium complex (MAC-PD) radiographically resembles that due to tuberculosis; it preferentially affects elderly white men with predisposing pulmonary disorders (PDPD).

Methods

Twenty-nine patients with MAC-PD were identified from a community-based population, and the medical records and chest roentgenograms (CRs) of six with a previously undescribed pattern of MAC-PD were reviewed. The distinctive clinical and demographic features of these six patients were identified and summarized.

Results

All were women who tended to be elderly. None had clinically evident PDPD. The dependent portion of the lingula or its counterpart, the middle lobe, was initially affected. Hilar adenopathy, volume loss, and cavitary disease were uniformly absent.

Conclusions

To account for the distinctive features of this syndrome, we offer the hypothesis that habitual voluntary suppression of cough may have led to the development of nonspecific inflammatory processes in these poorly draining lung regions, upon which MAC-PD engrafted. We offer the term, Lady Windermere's syndrome, to describe this pattern among elderly women and to suggest that their fastidiousness may be its root cause.

Section snippets

Materials and Methods

In a prior report, we described the research setting and the methodology employed to identify and evaluate cases of MAC-PD over a period of 12 yr in a nonreferral population.¹⁰ Briefly, all isolates of MAC from pleuropulmonary sources resulted in a medical record review. When indicated, chest roentgenograms (CRs) were reviewed by the senior author. Patients whose CRs were compatible with a diagnosis of mycobacterial disease and from whom MAC was repeatedly recovered in profusion or in whom

Case 1

A 45-yr-old nonsmoking white female office worker developed a nonproductive cough and left submammary pleuritic pain in May 1976. A 3-cm infiltrate involving only the most dependent portion of the lingula was observed (Fig. 1). A 1965 CR was normal. The results of fiberoptic bronchoscopy (FB) were normal except for a return of turbid material on washing the lingular bronchus. Fluorescent stain for mycobacteria showed rare organisms. Culture of the washings grew 4 + MAC. Treatment was initiated

Discussion

The clinical and radiographic similarities of these six patients appear to comprise a distinct clinical syndrome, the cardinal features of which are (1) initial involvement of the periphery of the lingula or of its counterpart, the middle lobe; (2) absence of clinically evident PDPD; and (3) exclusivity of the features to female patients. These features, which implied a common pathogenetic mechanism peculiar to this group and distinct from that operating in the usual case of MAC-PD, gave rise

Conclusions

A syndrome of MAC-PD which represents a clear departure in both clinical and radiographic features from the anticipated pattern has two important consequences.

1.
The correct diagnosis may be delayed or missed in this group of patients unless this unusual pattern is recognized and appropriate diagnostic studies employed. If MAC is isolated in profusion, even in atypical circumstances, it should not be discounted as a commensal.
2.
The syndrome implies a different pathogenesis from the usual case. To

ACKNOWLEDGMENT

We thank Miles Edwards, M.D., and Alan Barker, M.D., for reviewing the manuscript.

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: Manuscript received May 31; revision accepted August 21.

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Chest

Clinical InvestigationsMycobacterium avium Complex Pulmonary Disease Presenting as an Isolated Lingular or Middle Lobe Pattern: The Lady Windermere Syndrome

Background

Methods

Results

Conclusions

Section snippets

Materials and Methods

Case 1

Discussion

Conclusions

ACKNOWLEDGMENT

Chest

Clin Chest Med

Clin Chest Med

Chest

Disease due to anonymous mycobacteria

Am J Roentgenol

Mycobacterial diseases

Am J Roentgenol

Roentgenographic features of lung disease due to Mycobacterium intracellulare (primary and secondary infection)

Kekkaku

Roentgen aspects of chronic pulmonary mycobacteriosis

Radiology

Clinical Investigations
Mycobacterium avium Complex Pulmonary Disease Presenting as an Isolated Lingular or Middle Lobe Pattern: The Lady Windermere Syndrome