Download LADY WINDERMERE, WHIRLPOOLS AND A GENTLEMAN WINDED

LADY WINDERMERE, WHIRLPOOLS AND A GENTLEMAN
WINDED
Dr Rajesh Thomas
Respiratory & Sleep Medicine
John Hunter Hospital, Newcastle
A GENTLEMAN WINDED
CLINICAL PRESENTATION
74 year old male
Excellent health till three weeks earlier
Exertional breathlessness, chest tightness, dry cough
Weight loss of 20 pounds
No fever, night sweats, hemoptysis or constitutional symptoms
Antibiotics with no improvement, worsened breathlessness
Retired aircraft maintenance engineer
Exposure to chemicals, fumes, machine oils - but never unwell at
work
Travel to USA, Europe & Asia. Last overseas visit 3 years back
Smoker - 1pack daily for 15 years, quit 40 years back
Nil medications
No recent contact with animals
Budgerigars 1979-81
No exposure to Tuberculosis, or risk factors for HIV infection
Chest Xray: ‘Pulmonary oedema’
Trial of Frusemide
Negative stress echocardiogram
PHYSICAL EXAMINATION
Afebrile
No cyanosis,
edema
lymphadenopathy,
clubbing
SaO2: 92% on room air
Clear lungs
No cardiac abnormalities or visceromegaly
or
peripheral
INVESTIGATIONS
Routine hematologic and biochemical blood tests - Normal
C-Reactive Protein
57.1mg/L
(< 3.0)
CTDs/ Vasculitis screening
ANA
Borderline
ANA pattern
Homogeneous
ANCA
Negative
ENA screen
Negative
1:80
Serum Bird Precipitins
Aspergillus
Not Detected
Budgie serum
Not Detected
Budgie dropping
Positive
Pigeon serum
Not Detected
Pigeon dropping
Not Detected
Fowl serum
Not Detected
Pulmonary Function test
Actual value
% Predicted
FEV1 (liters)
FVC (liters)
FEV1/FVC
1.99
2.65
0.75
71
72
Total lung capacity (liters)
Residual volume (liters)
RV/TLC
5.81
2.94
0.51
87
110
118
DLCO (ml/min/mm Hg)
DLCO/Va
14.56
4.30
54
64
SUMMARY
74-year-old retired aircraft engineer. Ex-smoker.
Loved swimming and “a bubbly in the bubbles” with wife
Progressive breathlessness, cough and weight loss
Previous budgies, occupational exposure and widely travelled
Diffuse interstitial lung disease on imaging & lung functions
Negative serology for CTDs, Positive for budgie dropping
DIFFERENTIAL DIAGNOSIS??
1) Pneumocystis jiroveci pneumonia
2) Tuberculosis
3) Sarcoidosis
4) Connective-tissue disease related ILD
5) Hypersensitivity pneumonitis
6) Lymphangitis carcinomatosis
WHAT NEXT ??
BRONCHOSCOPY
Normal bronchial tree and mucosa
BAL cytology:
macrophages.
Benign
bronchial
cells
and
pulmonary
BAL Leucocyte surface marker: Mostly lymphocytes (53%) with
a T helper cell subtype
BAL Culture
MCS: Scanty oro-pharyngeal flora
Fungal culture: No fungi isolated after 3 weeks incubation
AFB culture: No AFB seen in direct smear, but AFB isolated on
culture
Mycobacterium avium complex (MAC) identified by DNA
probe
gene
DISCUSSION
MAC – WHAT CAN IT DO?
Four distinct clinical and pathologic presentations
a) Fibrocavitary MAC
b) MAC in Association With Immunodeficiency
c) Reticulonodular MAC with cylindrical bronchiectasis
d) Hot Tub Lung
RETICULONODULAR MAC WITH CYLINDRICAL BRONCHIECTASIS
Elderly females without preexisting lung disease
Chronic, indolent course - months to years
Multiple lung nodules, ‘tree-in-bud’ appearance and cylindrical
bronchiectasis
Predilection for RML & lingula
Historically dubbed “Lady Windermere Syndrome”
Hypothesis - Fastidiousness in these women causes the
disease
Lady Windermere – Who is she?
1. Lead actress of the movie “Slumdog Millionaire“
2. Lead doll of “Pussycat Dolls“
3. Lady at the centre of “Four Corners”
4. Character in Oscar Wilde's play "Lady Windermere's Fan"
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.“
Librarian’s letter to the editor of Chest challenging use of Lady
Windermere as the eponymous ancestor of the syndrome
“… Lady Windermere is a vivacious young
woman,
married only 2 years, who never coughs or displays any
other signs of illness.
She not only appears to have excellent health, she does
not seem exceptionally prissy…”
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”
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”
HOT TUB LUNG – WHAT IS IT?
1990s - Cluster of patients with HP-like granulomatous disease
Associated with MAC and regular hot tub/spa exposure
“Hot Tub Lung”
Growth of MAC not inhibited by temperatures upto 42°C - above
usual hot tub temperature
1,000 times more resistant to chlorine than is E coli
Poor hot tub maintenance & poor personal hygiene causes
uninhibited growth of MAC
Jet aerosolization & inhalation of MAC – causes hot tub lung
Only 4 cases of HTL reported with outdoor hot tub/spa use
PATHOGENESIS
Pathogenesis of HTL poorly understood – hypersensitivity
pneumonitis vs infection
Satisfy the criteria for HP - presence of both BAL lymphocytosis
and typical HRCT findings
Direct infection of the lung by MAC also suggested
CLINICAL COURSE
Subacute presentation; variable latency, severity and course
Patients often do not associate symptoms with hot tub exposure
Imaging - Diffuse ground glass opacities and lung nodules
Sputum culture positive for MAC in 74% patients; TBB and BAL
cultures increase yield further
TREATMENT
No standard approach
Significant improvement with mere abstinence
Oral corticosteroids probably the next line of treatment
If steroids fail, antimycobacterial therapy or both may be used –
Duration of therapy not known
Expected course - Recovery without relapse
WHIRLPOOL – TO BATH OR NOT TO BATH?
“Spa" - Derived from the town of Spa (once part of the Roman
empire; now east of Belgium)
Evidence of organized use of spa 5000 years ago
Queen Elizabeth I (1589 A.D.) insisted on bathing at least once a
month, "whether she needed it or not“
1954 - Joseph Jacuzzi developed a portable whirlpool pump &
coupled it with an jet air-injection system to produce
bubbles
PRIMARY PREVENTION
Adequate ventilation of hot tub room
Frequent cleaning of hot tub/ change of hot tub water & filter
Use disinfectants as bromine and ultraviolet light
Superheating the tub water to 70°C for 1 hr before use
Showering before getting into the hot tub
SECONDARY PREVENTION
Avoidance of hot tub
Education of general public and clinicians to allow for early
diagnosis
WHAT HAPPENED TO THE PATIENT SUBSEQUENTLY??
Back to swimming 1500meters daily
Spa - Decomissioned
Lost $2000 and gained 20 pounds
LEARNING POINTS
Environmental mycobacteria can cause hypersensitivity
pneumonitis, in addition to usual infectious diseases
Maintain high index of suspicion for Hot Tub Lung
Ask about hot tub use in patients with unexplained respiratory
symptoms
Treatable and reversible - if detected early
Hot showers and hot tubs are fine, provided…
Tubs have a Capacity!
REFERENCES
1.
CHEST 2006:130;1234.The expanding spectrum of MACassociated pulmonary disease
2. CHEST 1992:101;1605. MAC presenting as an isolated lingular
or middle lobe pattern. The Lady Windermere syndrome
3. NEJM 2006: 343; 642. Weekly Clinicopathological Exercises
4. ENVIRONMENTAL HEALTH PERSPECTIVES 2007: 115; 262.
HP - like lung disease with non-tuberculous mycobacteria from
exposure to hot water aerosols
5. WIKIPEDIA
The Duck Stops Here!!!
The Duck Stops Here!!!