sandstorm in her chest ? manmadha rao talluri nizam’s institute of medical sciences, hyderabad,...
TRANSCRIPT
Sandstorm in her Chest ?
Manmadha Rao Talluri
Nizam’s Institute of Medical Sciences, Hyderabad, India
History
• 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
• 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
2. Bronchoscopy with bronchoalveolar lavage
3. Bronchoscopy with transbronchial biopsies
4. Video-assisted thoracoscopic (VATS) biopsy
5. 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
• 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 thin-section 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