Pulmonary alveolar microlithiasis: an interesting case report with systematic review of Indian literature

Abstract

Pulmonary alveolar microlithiasis is a rare disease characterized by intra-alveolar presence of microliths. This study reports an interesting case of pulmonary alveolar microlithiasis and provides a systematic review of cases reported from India. A 23-year-old female presented with a history of cough, wheeze, chest pain, and episodic wheeze for five months. Pulmonary function tests demonstrated an obstructive pattern, and chest X-ray showed fine micronodular opacities predominantly involving the middle and lower zones of both lungs. Transbronchial lung biopsy revealed the diagnosis. She responded well to inhaled steroid therapy. A systematic review of literature was performed and identified 73 cases of pulmonary alveolar microlithiasis reported from India. The mean (SD) age of the patients was 28.8 (14.9) years, with an almost equal male:female ratio. Many patients were asymptomatic at presentation. Breathlessness and cough were the most common symptoms, and the disease progressed into respiratory failure associated with cor pulmonale. About one-third of the cases were initially misdiagnosed and treated as pulmonary tuberculosis. Extra-pulmonary manifestations and co-morbidities were also evident in our series. This systematic review helps to determine epidemiological and clinical characteristics of pulmonary alveolar microlithiasis. Further research is needed to elucidate the etiopathogenesis, diagnosis, and therapeutic options, which are beneficial in developing and identifying cost-effective treatment for pulmonary alveolar microlithiasis.

Keywords

pulmonary alveolar microlithiasis / alveolar microlithiasis / microliths / rare diseases

References

[1] Huqun , Izumi S, Miyazawa H, Ishii K, Uchiyama B, Ishida T, Tanaka S, Tazawa R, Fukuyama S, Tanaka T, Nagai Y, Yokote A, Takahashi H, Fukushima T, Kobayashi K, Chiba H, Nagata M, Sakamoto S, Nakata K, Takebayashi Y, Shimizu Y, Kaneko K, Shimizu M, Kanazawa M, Abe S, Inoue Y, Takenoshita S, Yoshimura K, Kudo K, Tachibana T, Nukiwa T, Hagiwara K. Mutations in the SLC34A2 gene are associated with pulmonary alveolar microlithiasis. Am J Respir Crit Care Med2007; 175(3): 263−268

[2] Castellana G, Lamorgese V. Pulmonary alveolar microlithiasis. World cases and review of the literature. Respiration2003; 70(5): 549−555

[3] Viswanathan R. Pulmonary alveolar microlithiasis. Thorax1962; 17(3): 251−256

[4] Ucan ES, Keyf AI, Aydilek R, Yalcin Z, Sebit S, Kudu M, Ok U. Pulmonary alveolar microlithiasis: review of Turkish reports. Thorax1993; 48(2): 171−173

[5] Mariotta S, Guidi L, Papale M, Ricci A, Bisetti A. Pulmonary alveolar microlithiasis: review of Italian reports. Eur J Epidemiol1997; 13(5): 587−590

[6] Bhattacharjee BK, Barua R, Duta JK. Pulmonary alveolar microlithiasis. Indian J Chest Dis1965; 7: 51−53

[7] Banerjea JC, Mukerji A, Maiti CR. Chronic cor pulmonale due to pulmonary alveolar microlithiasis. Indian Heart J1967; 19(1): 47−56

[8] Rajagopalan CR, Krishna KVS, Rao M, Babu TN. Pulmonary alveolar microlithiasis. Report of a case. Indian J Pediatr1967; 34(236): 328−331

[9] Rao CS, Rao YM, Reddy DB, Sarada D. Pulmonary alveolar microlithiasis. J Indian Med Assoc1968; 50(9): 427−428

[10] Khattri HN, Sodhi JS, Gujeral JS, Dutta BN. Microlithiasis alveolaris pulmonale. J Assoc Physicians India1971; 19(4): 335−337

[11] Thiruvengadam KV, Arunachalam K, Murali MR, Sarma AV, Varadarajan MG, Shetty BS, Swamidasan G. Pulmonary alveolar microlithiasis in a family. J Assoc Physicians India1972; 20(9): 707−712

[12] Prabhakar BR, Sabharwal BD, Prabhakar H, Sethl RS. Pulmonary alveolar microlithiasis. A case report with review of literature. Indian J Pathol Bacteriol1974; 17(3): 193−196

[13] Bhatia JL, Thind GS. Pulmonary alveolar microlithiasis. Ind J Tub1976; 23(3): 110−115

[14] Mathur AS, Gupta DK, Garg RK. Pulmonary alveolar microlithiasis. Indian JRadiol1977; 31(3): 207−208

[15] Thind GS, Bhatia JL. Pulmonary alveolar microlithiasis. Br J Dis Chest1978; 72(2): 151−154

[16] Taneja VP, Sen D. Pulmonary alveolar microlithiasis (case report). J Assoc Physicians India1981; 29(5): 411−414

[17] Gupta SK, Maini VK, Bedi RS. Pulmonary alveolar microlithiasis. Indian J Chest Dis Allied Sci1982; 24(1): 29−32

[18] Saigal RK, Gupta K, Sandhu SK, Sidhu PK. Pulmonary alveolar microlithiasis. (A case report.)J Postgrad Med1982; 28(4): 235−237

[19] Chetty A, Paul VK, Kumar A, Mitra DK, Chopra P. Alveolar microlithiasis—a case report with review of literature. Indian J Pediatr1983; 50(405): 453−455

[20] Challa S, Dusaj IS, Kumar V, Mehrotra R. Pulmonary alveolar microlithiasis. Indian J Chest Dis Allied Sci1984; 26(4): 263−266

[21] NatrajaIyer K. Sasikumar S, Devi SS. Pulmonary alveolar microlithiasis- a case report. Indian J Radiol1986; 40(1): 63−65

[22] Bhadur P, Seetharaman ML, Krishnamurthy H. Pulmonary alveolar microlithiasis — a case report. Lung India1987; 5(4): 187−188

[23] Pant K, Chawla R, Chhabra SK, Jain SK, Malik GB, Gaur SN. Pulmonary alveolar microlithiasis—a case report and review of literature. Indian J Chest Dis Allied Sci1988; 30(2): 141−147

[24] Swarnkar JS, Wakodkar TD, Kirolikar SK, Diwakar RK. Pulmonary alveolar microlithiasis— a report of two cases. Lung India1989; 7(3): 140−142

[25] Venkateswarlu J, Waghray P, Subbarao K, Kishore LT, Subbalakshmi M. pleural calcification in pulmonary alveolar microlithiasis − a case report. Lung India1989; 7(3): 143−145

[26] Pant K, Shah A, Mathur RK, Chhabra SK, Jain SK. Pulmonary alveolar microlithiasis with pleural calcification and nephrolithiasis. Chest1990; 98(1): 245−246

[27] Rao SD, Rekha S, Chandrasekhara MK, Shetty N, Srikrishna. Pulmonary alveolar microlithiasis in siblings. Indian Pediatr1991; 28(10): 1206−1210

[28] Sharma SK, Sharma S, Mukhopadhyaya S, Padhy AK, Vijayraghavan M. Pulmonary alveolar microlithiasis–report of three cases with pulmonary function and exercise studies. Indian J Chest Dis Allied Sci1992; 34(4): 205−215

[29] Babu MK, Kumar KR, Thomas JA. Pulmonary alveolar microlithiasis. Indian J Pathol Microbiol1992; 35(2): 149−152

[30] Malhotra B, Kailan BM. Pulmonary alveolarmicrolithiasis–a case report. Ind J Tub1993; 40(1): 31−33

[31] Malur PR, Ajay K, Jayaram N. Alveolar microlithiasis–report of two cases. Indian J Pathol Microbiol1993; 36(2): 163−167

[32] Boghani A, Kumaresan K, Sundaram C, Bhaskar G. Pulmonary alveolar microlithiasis revisited with CT scan, bone scan and broncho-alveolar lavage. Lung India1993; 11(2): 37−39

[33] Gaude GS, Pinto MJW, Bagga AS. Pneumothorax in pulmonary alveolar microlithiasis: a case report. Antiseptic1994; 91(4): 135−136

[34] Singh NK, Gupta A. Pulmonary alveolar microlithiasis. J Assoc Physicians India1995; 43(9): 647−648

[35] Kashyap S, Kumar M, Pal LS. Pulmonary alveolar microlithiasis in a Himachali woman. J Assoc Physicians India1996; 44(1): 56−57

[36] Chatterji R, Gaude GS, Patil PV. Pulmonary alveolar microlithiasis: diagnosed by sputum examination and transbronchial biopsy. Indian J Chest Dis Allied Sci1997; 39(4): 263−267

[37] Vidyasagar B, Ranjana SD, Jayan R, NagarajaSetty KL, Shivalingappa GS. Pulmonary alveolar microlithiasis. Lung India1998; 16(2): 80−82

[38] Dwivedi MK, Pal RK, Borkar PB. Pulmonary alveolar microlithiasis. Indian J Radiol Imaging1999; 9(4): 195−196

[39] Hira HS, Singh T, Chowdhary V. Pulmonary alveolar microlithiasis: role of transbronchial lung biopsy. J Assoc Physicians India2000; 48(8): 832−833

[40] Gupta D, Aggarwal AN, Jindal SK. Lung static pressure- volume analysis can demonstrate alveolar involvement in pulmonary alveolar microlithiasis. Lung India2000; 18(2): 64−65

[41] Shah MS, Nanavati KJ, Airon A, Shah RR, Joshi BD. Case report— pulmonary alveolar microlithiasis. Indian J Radiol Imaging2003; 13(3): 277−279

[42] Bhalotra B, Gogia A, Gupta P, Jain N. A pulmonary snowstorm. Med J Aust2004; 180(9): 454

[43] Patro SS, Kar CR. Pulmonary alveolar microlithiasis. Indian J Radiol Imaging2006; 16(4): 879−881

[44] Unnithan J. Nandagopal. A rare case of pulmonary alveolar microlithiasis. Pulmon2006; 8(1): 27−29

[45] Mitra S, Ghoshal AG. A family of pulmonary alveolar microlithiasis— a case report. Lung India2000; 18(3): 97−100

[46] Hephzibah J, Oommen R, Mugesh B, Pradhip J. Pulmonary alveolar microlithiasis scinitigraphic features. Internet J Pulmonol Med2006; 9(1)：12504

[47] Thapa R, Ganguly D, Ghosh A. Pulmonary alveolar microlithiasis in siblings. Indian Pediatr2008; 45(2): 154−156

[48] Chandra S, Mohan A, Guleria R, Das P, Sarkar C.Pulmonary alveolar microlithiasis. BMJ Case Rep2009; 2009: bcr09.2008.1012

[49] Malhotra B, Sabharwal R, Singh M, Singh A. Pulmonary alveolar microlithiasis with calcified pleural plaques. Lung India2010; 27(4): 250−252

[50] Gayathri Devi HJ, Mohan Rao KN, Prathima KM, Das JK. Pulmonary alveolar microlithiasis. Lung India2011; 28(2): 139−141

[51] Shah M, Joshi JM. Bone scintigraphy in pulmonary alveolar microlithiasis. Indian J Chest Dis Allied Sci2011; 53(4): 221−223

[52] Gayathri B, Keerthivasan S. Pulmonary alveolar microlithiasis. J Med2011; 12(1): 55−57

[53] Sonwalkar P, Karthik M, Vishal NS, Praveen M, Patil MM. Pulmonary alveolar microlithiasis. JCDR2012; 6(7): 1296−1297

[54] Galhotra R, Gupta K. Images–pulmonary alveolar microlithiasis (PAM). J Pulmon Resp Med2012; 2: 124

[55] Flynn A, Agastyaraju AD. Pulmonary alveolar microlithiasis. Int J Case Rep Images2013; 4(2): 108−110

[56] Sahoo MK, Karunanithi S, Bal CS. Pulmonary alveolar microlithiasis: imaging characteristics of planar and SPECT/CT bone scan versus 18F-FDG and 18F-sodium fluoride PET/CT scanning. Jpn J Radiol2013; 31(11): 766−769

[57] Caffrey PR, Altman RS. Pulmonary alveolar microlithiasis occurring in premature twins. J Pediatr1965; 66(4): 758−763

[58] Mariotta S, Ricci A, Papale M, De Clementi F, Sposato B, Guidi L, Mannino F. Pulmonary alveolar microlithiasis: report on 576 cases published in the literature. Sarcoidosis Vasc Diffuse Lung Dis2004; 21(3): 173−181

[59] Castellana G, Gentile M, Castellana R, Fiorente P, Lamorgese V. Pulmonary alveolar microlithiasis: clinical features, evolution of the phenotype, and review of the literature. Am J Med Genet2002; 111(2): 220−224

[60] Corut A, Senyigit A, Ugur SA, Altin S, Ozcelik U, Calisir H, Yildirim Z, Gocmen A, Tolun A. Mutations in SLC34A2 cause pulmonary alveolar microlithiasis and are possibly associated with testicular microlithiasis. Am J Hum Genet2006; 79(4): 650−656

[61] Dogan OT, Ozsahin SL, Gul E, Arslan S, Koksal B, Berk S, Ozdemir O, Akkurt I. A frame-shift mutation in the SLC34A2 gene in three patients with pulmonary alveolar microlithiasis in an inbred family. Intern Med2010; 49(1): 45−49

[62] Terada T. Pulmonary alveolar microlithiasis with cor pulmonale: an autopsy case demonstrating a marked decrease in pulmonary vascular beds. Respir Med2009; 103(11): 1768−1771

[63] Chalmers AG, Wyatt J, Robinson PJ. Computed tomographic and pathological findings in pulmonary alveolar microlithiasis. Br J Radiol1986; 59(700): 408−411

[64] Lauta VM. Pulmonary alveolar microlithiasis: an overview of clinical and pathological features together with possible therapies. Respir Med2003; 97(10): 1081−1085

[65] Sears MR, Chang AR, Taylor AJ. Pulmonary alveolar microlithiasis. Thorax1971; 26(6): 704−711

[66] Moran CA, Hochholzer L, Hasleton PS, Johnson FB, Koss MN. Pulmonary alveolar microlithiasis. A clinicopathologic and chemical analysis of seven cases. Arch Pathol Lab Med1997; 121(6): 607−611

[67] Mascie-Taylor BH, Wardman AG, Madden CA, Page RL. A case of alveolar microlithiasis: observation over 22 years and recovery of material by lavage. Thorax1985; 40(12): 952−953

[68] Kanat F, Teke T, Imecik O. Pulmonary alveolar microlithiasis with epididymal and periurethral calcifications causing obstructive azospermia. Int J Tuberc Lung Dis2004; 8(10): 1275

[69] Arslan A, Yalin T, Akan H, Belet U. Pulmonary alveolar microlithiasis associated with calcifications in the seminal vesicles. J Belge Radiol1996; 79(3): 118−119

[70] Coetzee T. Pulmonary alveolar microlithiasis with involvement of the sympathetic nervous system and gonads. Thorax1970; 25(5): 637−642

[71] Yesner R, Skorneck AB. Pulmonary alveolar microlithiasis in mitral stenosis. Am J Pathol1956; 32(3): 611

[72] Schmidt H, Lörcher U, Kitz R, Zielen S, Ahrens P, König R. Pulmonary alveolar microlithiasis in children. Pediatr Radiol1996; 26(1): 33−36

[73] Ratjen FA, Schoenfeld B, Wiesemann HG. Pulmonary alveolar microlithiasis and lymphocytic interstitial pneumonitis in a ten year old girl. Eur Respir J1992; 5(10): 1283−1285

[74] Portnoy LM, Amadeo B, Hennigar GR. Pulmonary alveolar microlithiasis: an unusual case (associated with milk alkali syndrome). Am J Clin Pathol1964; 41(2): 194−201

[75] Ritchie DA, O’Connor SA, McGivern D. An unusual presentation of pulmonary alveolar microlithiasis and diaphyseal aclasia. Br J Radiol1992; 65(770): 178−181

[76] Giallauria F, Giallauria G. Pulmonary alveolar microlithiasis. Thorax2011; 66(9): 840

[77] Gasparetto EL, Tazoniero P, Escuissato DL, Marchiori E, Frare E Silva RL, Sakamoto D. Pulmonary alveolar microlithiasis presenting with crazy-paving pattern on high resolution CT. Br J Radiol2004; 77(923): 974−976

[78] Marchiori E, Gonçalves CM, Escuissato DL, Teixeira KI, Rodrigues R, Barreto MM, Esteves M. Pulmonary alveolar microlithiasis: high-resolution computed tomography findings in 10 patients. J Bras Pneumol2007; 33(5): 552−557

[79] Siddiqui NA, Fuhrman CR. Best cases from the AFIP: pulmonary alveolar microlithiasis. Radiographics2011; 31(2): 585−590

[80] Sumikawa H, Johkoh T, Tomiyama N, Hamada S, Koyama M, Tsubamoto M, Murai S, Inoue A, Nakamura H, Tachibana T, Müller NL. Pulmonary alveolar microlithiasis: CT and pathologic findings in 10 patients. Monaldi Arch Chest Dis2005; 63(1): 59−64

[81] Brown J, Leon W, Felton C. Hemodynamic and pulmonary studies in pulmonary alveolar microlithiasis. Am J Med1984; 77(1): 176−178

[82] Dahabreh M, Najada A. Pulmonary alveolar microlithiasis in an 8-month-old infant. Ann Trop Paediatr2009; 29(1): 55−59

[83] Yilmaz MI, Koc B, Kantarcioglu M, Akinci SB, Ayta H, Bulucu F, Bal S. Pulmonary alveolar microlithiasis after Varicella zoster infection in a patient presenting with antiphospholipid syndrome and discoid lupus. Rheumatol Int2002; 22(5): 213−215

[84] Ozcelik U, Yalcin E, Arıyurek M, Ersoz DD, Cinel G, Gulhan B, Kiper N. Long-term results of disodium etidronate treatment in pulmonary alveolar microlithiasis. Pediatr Pulmonol2010; 45(5): 514−517

[85] Jankovic S, Pavlov N, Ivkosic A, Erceg I, Glavina-Durdov M, Tocilj J, Dragisic-Ivulic S, Primorac D. Pulmonary alveolar microlithiasis in childhood: clinical and radiological follow-up. Pediatr Pulmonol2002; 34(5): 384−387

[86] Shigemura N, Bermudez C, Hattler BG, Johnson B, Crespo M, Pilewski J, Toyoda Y. Lung transplantation for pulmonary alveolar microlithiasis. J Thorac Cardiovasc Surg2010; 139(3): e50−e52

[87] Borrelli R, Fossi A, Volterrani L, Voltolini L. Right single-lung transplantation for pulmonary alveolar microlithiasis. Eur J Cardiothorac Surg2014; 45(2): e40

[88] Edelman JD, Bavaria J, Kaiser LR, Litzky LA, Palevsky HI, Kotloff RM. Bilateral sequential lung transplantation for pulmonary alveolar microlithiasis. Chest1997; 112(4): 1140−1144

[89] Samano MN, Waisberg DR, Canzian M, Campos SV, Pêgo-Fernandes PM, Jatene FB. Lung transplantation for pulmonary alveolar microlithiasis: a case report. Clinics (Sao Paulo)2010; 65(2): 233−236

[90] Yin X, Wang H, Wu D, Zhao G, Shao J, Dai Y. SLC34A2 Gene mutation of pulmonary alveolar microlithiasis: report of four cases and review of literatures. Respir Med2013; 107(2): 217−222

[91] Olauson H, Brandenburg V, Larsson TE. Mutation analysis and serum FGF23 level in a patient with pulmonary alveolar microlithiasis. Endocrine2010; 37(2): 244−248

[92] Takahashi H, Chiba H, Shiratori M, Tachibana T, Abe S. Elevated serum surfactant protein A and D in pulmonary alveolar microlithiasis. Respirology2006; 11(3): 330−333

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