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Sandstorm in her Chest ?
Manmadha Rao Talluri
Nizam’s Institute of Medical Sciences,
Hyderabad, India
History
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32-year-old woman, housewife
Dry cough, exertional dyspnea – 1 year
No wheezing/ chest pain/ hemoptysis
No malar rash/ photosensitivity
No upper respiratory symptoms
Past History
No history of:
• Bronchial asthma
• Varicella in childhood
• Recurrent respiratory tract infections
• Rheumatic heart disease
• Tuberculosis
• Occupational dust exposure
• Similar complaints in family history
Examination
• P – 100, BP – 130/80, respiratory rate – 18,
afebrile
• Facial puffiness with acne
• Upper respiratory tract – normal
• Lungs – bilateral basal fine end-inspiratory
crepitations, no rhonchi
• Abdomen, CVS, CNS – normal
• No clubbing
Dr. Newell
Other Tests
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Hb –15.2, TLC – 10,200, N 82, L 13, E 4, M 1
ESR – 35mm
Urine routine – normal
Renal function test – normal
Rheumatoid factor – positive
ANA – positive
Ds DNA, Anti SS-a, SS-b, U1 RNP – negative
PFT – severe restrictive defect
2D ECHO – normal
Question
What is the most likely diagnosis?
1. Varicella zoster (chicken pox)
2. Pulmonary talcosis
3. Histoplasmosis
4. Pulmonary alveolar microlithiasis
5. Sarcoidosis
Question
What would you do next?
1. Nothing, the diagnosis is obvious
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Bronchoscopy with bronchoalveolar lavage
Bronchoscopy with transbronchial biopsies
Video-assisted thoracoscopic (VATS) biopsy
Open-lung biopsy
Dr. Heffner
Dr. Leslie
Transbronchial Biopsy
Transbronchial Biopsy
Final Diagnosis
PULMONARY ALVEOLAR MICROLITHIASIS
Clinical Course
• Given corticosteroids and theophylline for
dyspnea
• Advised lung transplantation
– Not done due to financial constraints
• Patient gradually worsened in course of 2
years and succumbed to respiratory failure
Pulmonary Alveolar Microlithiasis
• Rare disease of unknown pathogenesis
• Usually sporadic; autosomal recessive
form described (Mediterranean countries)
• Paucity of symptoms despite widespread
involvement
• Cough & dyspnea in 3rd & 4th decade
• Death usually in mid-life due to respiratory
failure and cor pulmonale
Pulmonary Alveolar Microlithiasis
• Widespread laminated calcispherites in
alveolar spaces
• Absence of any known disorder of calcium
metabolism
• Unknown stimulus
• Changes in the alveolar lining membrane or
secretions result in greater alkalinity,
promoting intra-alveolar precipitation of
calcium phosphates and carbonates
• Serum surfactant protein – A & D are markedly
elevated
– Increase as disease progresses
– Function as serum markers to monitor
disease activity and progression
• Mutations in SLC34A2 gene expressed in type
II pneumocytes which encode type IIb sodium
phosphate co-transporter
• No known therapy
• Corticosteroids, chelating agents and BAL
have demonstrated no benefit
• Role of bisphosphonates remains to be
proven
• Bilateral lung transplantation for advanced
cases
Chest X-ray/ CT
“Sand storm” appearance
Black Pleura sign
Crazy paving pattern
Take Home Message
• In no other condition is the lack of
association between roentgenologic
and clinical findings so striking as in
PAM
• PAM should always be considered in
the differential diagnosis of calcific
micronodular pulmonary lesions
References
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Barbolini G, Rossi G, Bisetti A. Pulmonary alveolar microlithiasis. N Engl
J Med 2002; 347:69–70.
K. Gowrinath and Arun R. Warrier Pulmonary alveolar microlithiasis,
Lung India 2006; 23:42-44.
Gasparetto EL, Tazoniero P, Escuissato DL, et al. Pulmonary alveolar
microlithiasis presenting with crazy-paving pattern on high resolution
CT. Br. J. Radiol.2004; 77: 974-976
Chan Ed, Morales DV, Welsh CH, et al. Calcium deposition with or
without bone formation in the lung. Am J Respir Crit Care Med.
2002;165(12):1654-1669
Korn MA, Schurawitzki H, Klepetko W, et al. Pulmonary alveolar
microlithiasis: findings on high-resolution CT. AJR Am J Roentgenol.
1992 ; 158(5):981-982.
Johkoh T, Itoh H, Müller NL, et al. Crazy paving appearance at thinsection CT. Spectrum of disease and pathologic findings. Radiology
1999; 211:155–160
Takahashi H, Chiba H, Shiratori M, et al. Elevated serum surfactant
protein A and D in pulmonary alveolar microlithiasis. Respirology. 2006;
11(3): 330-333