Pulmonary alveolar microlithiasis: lung function in five cases

Abstract

Pulmonary alveolar microlithiasis is a relatively rare disease of unknown aetiology, characterized by the presence of multiple microscopic stones within the pulmonary alveoli. The striking radiological picture often contrasts with mild or even absent symptoms except late in the course of the disease.The radiological and clinical features of the disease have been well described (Sosman, Dodd, Jones, and Pillmore, 1957;Viswanathan, 1962;Yang and Lin, 1963). Detailed pulmonary function studies have received somewhat less attention.The purpose of this communication is to present respiratory function data in five cases of pulmonary alveolar microlithiasis, and to review some results of pulmonary function tests reported in the literature.

Pulmonary Alveolar Microlithiasis

“…The most common findings are decreased vital and total lung capacity, normal residual volume/total lung capacity ratio and decreased diffusing capacity. 19 The standard chest radiograph and CT, with results for PAM, are enough to diagnose the disease, even if microscopic evidence of the microliths in the alveoli was obtained in most cases. 20,21 Microliths in the sputum and bronchoalveolar lavage are not diagnostic because patients with chronic obstructive pulmonary disease and tuberculosis expectorate microliths as well .…”

Pulmonary Alveolar Microlithiasisc

“…Death may occur from respiratory failure, cor pulmonale or severe respiratory infection [1,2,11,37,42], However respiratory function tests are often normal or near normal even with extensive radiological findings as in cases 2 and 3. Restrictive ventilatory defect as in cases 1 and 4 [14,25], diffusion defect and ventilation perfusion imbalance (V/Q) have been recorded [4,25], The treatment for PAM is only supportive, as there is still no specific or satisfactory treatment for this disorder. Many therapies such as corticosteroids, che lating agents as well as bronchial lavage have not been beneficial.…”

“…Until 1987, 172 cases of PAM were reported [1]. Its clinical presentation, radiologic appearance, pulmonary function abnor malities [2][3][4], radionuclide imaging [3], con ventional computed tomography (CT scan) [6,7], high resolution computed tomography (HR-CT) [8] and pathology [9,10] have all been documented. Although the aetiology is unknown, many environmental causes as well as a hereditary predisposition have been sug gested [2,11], The progression of the disease is very slow.…”

Pulmonale alveoläre Mikrolithiasis - eine seltene Ursache bilateraler ausgedehnter pulmonaler Infiltrate

“…Lungenfunktionell ist der Befund initial normal. 30% der Patienten entwickeln schließlich eine pathologische Lungenfunktion mit restriktiver Ventilationsstörung und reduzierter CO-Diffusionskapazität [2].…”

“…D ie pulmonale alveoläre Mikrolithiasis (PAM) ist eine seltene Erkrankung, die durch die pulmonale Ablagerung von Calciumphosphatkonkrementen in den Alveolen charakterisiert ist [1][2][3][6][7][8][9]. Diagnostisch richtungsweisend ist das Röntgenbild mit einer typischen "Sandsturm"-artigen Verschattung des Lungengewebes [10].…”