Pulmonary Alveolar Microlithiasis: CT and pathologic findings in 10 patients

Background and Aim. To evaluate CT findings of pulmonary alveolar microlithiasis and correlate the CT with the pathologic findings. Methods. The study included 10 patients with pathologically proven microlithiasis. Two independent observers evaluated the presence, extent and distribution of the CT findings. CT findings were compared with those at autopsy in two patients and with transbronchial biopsy in eight patients. Results. All patients had a myriad of calcified nodules measuring approximately 1 mm in diameter. Close apposition of the nodules resulted in areas of ground-glass attenuation and consolidation, which were the predominant abnormality on CT in all 10 patients, involving 41% ± 16.3 (mean ± SD) and 30% ± 4.8 of the lung parenchyma, respectively. Calcifications were also seen along interlobular septa, bronchovascular bundles and pleura. Other findings included interlobular septal thickening, thickening of bronchovascular bundles, nodules, and subpleural cysts. There was a solid agreement between the observers for the presence (kappa value; 0.77) and extent (Spearman rank correlation; r= 0.81 to 1.0 p<0.01) of abnormalities. Autopsy specimens demonstrated microliths in alveolar airspaces and along interlobular septa, bronchovascular bundles and pleura. Subpleural small cysts were shown to represent dilated alveolar ducts. Conclusion. Pulmonary microlithiasis is characterised by the presence of numerous small, calcified nodules, calcifications along interlobular septa, bronchovascular bundles and pleura, ground-glass opacities, consolidation, and subpleural cysts. The cysts represent dilated alveolar ducts.

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Can chest high-resolution computed tomography findings diagnose pulmonary alveolar microlithiasis?

Radiologia Brasileira ◽

10.1590/0100-3984.2014.0123 ◽

2015 ◽

Vol 48 (4) ◽

pp. 205-210 ◽

Cited By ~ 19

Author(s):

Flávia Angélica Ferreira Francisco ◽

Rosana Souza Rodrigues ◽

Miriam Menna Barreto ◽

Dante Luiz Escuissato ◽

Cesar Augusto Araujo Neto ◽

...

Keyword(s):

Computed Tomography ◽

High Resolution ◽

Lung Biopsy ◽

Lung Parenchyma ◽

Ground Glass ◽

High Resolution Computed Tomography ◽

Pulmonary Alveolar Microlithiasis ◽

Alveolar Microlithiasis

Abstract Objective: The present study was aimed at retrospectively reviewing high-resolution computed tomography (HRCT) findings in patients with pulmonary alveolar microlithiasis in order to evaluate the frequency of tomographic findings and their distribution in the lung parenchyma. Materials and Methods: Thirteen patients (9 females and 4 males; age, 9 to 59 years; mean age, 34.5 years) were included in the present study. The HRCT images were independently evaluated by two observers whose decisions were made by consensus. The inclusion criterion was the presence of abnormalities typical of pulmonary alveolar microlithiasis at HRCT, which precludes lung biopsy. However, in 6 cases lung biopsy was performed. Results: Ground-glass opacities and small parenchymal nodules were the predominant tomographic findings, present in 100% of cases, followed by small subpleural nodules (92.3%), subpleural cysts (84.6%), subpleural linear calcifications (69.2%), crazy-paving pattern (69.2%), fissure nodularity (53.8%), calcification along interlobular septa (46.2%) and dense consolidation (46.2%). Conclusion: As regards distribution of the lesions, there was preferential involvement of the lower third of the lungs. No predominance of distribution in axial and anteroposterior directions was observed.

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Chest computed tomography findings of adult patients with antimelanoma differentiation-associated protein 5 antibody-positive interstitial lung disease

Modern Rheumatology ◽

10.1093/mr/roab006 ◽

2021 ◽

Author(s):

Yuko Waseda ◽

Takeshi Johkoh ◽

Helmut Prosch ◽

Stefan Nemec ◽

Keigo Saeki ◽

...

Keyword(s):

Computed Tomography ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Univariate Analysis ◽

Antibody Detection ◽

Ground Glass ◽

Ct Findings ◽

Ground Glass Attenuation ◽

Air Space ◽

Time Required

ABSTRACT Objectives Interstitial lung disease (ILD) associated with the antimelanoma differentiation-associated protein 5 (anti-MDA5) antibody is a rapidly progressive disease that requires timely, aggressive treatment. However, prompt diagnosis is difficult due to the longer time required for antibody detection. This study described the computed tomography (CT) findings of anti-MDA5 antibody-positive ILD (anti-MDA5-ILD). Methods CT findings of 20 patients (7 men, 13 women; mean age, 53.6 ± 13.5 years) with anti-MDA5-ILD were retrospectively reviewed. All patients had clinical diagnoses of dermatomyositis, and 14 patients presented with amyopathic findings. Results Bilateral ground-glass attenuation, air-space consolidation, and reticular shadows were observed in 20 (100%), 15 (75%), and 3 (15%) patients, respectively. The spread of air-space consolidation was 6.0 ± 5.6% (mean ± standard deviation). Univariate analysis revealed that high Krebs von den Lungen-6, high spread of consolidation, low partial pressure of oxygen, and low forced vital capacity were significant predictors for poor survival. The final radiological diagnoses were nonspecific interstitial pneumonia and organising pneumonia (OP) in 2 (10%) and 16 (80%) patients, respectively. Further, 30% of OP patients showed fibrosis. Conclusion The characteristic CT findings of patients with anti-MDA5-ILD were ground-glass attenuation, air-space consolidation, and less reticulation. These CT findings were compatible with those of OP.

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Pulmonary Alveolar Microlithiasis and Rheumatoid Arthritis: A Case Report and Review of the Literature

Case Reports in Rheumatology ◽

10.1155/2021/8811507 ◽

2021 ◽

Vol 2021 ◽

pp. 1-4

Author(s):

Waleed Hafiz ◽

Ahmedhusam Alahmed ◽

Mohammed Alahmadi ◽

Rakan Alotaibi ◽

Abdullah Alsharif ◽

...

Keyword(s):

Rheumatoid Arthritis ◽

Autosomal Recessive ◽

Lung Parenchyma ◽

Systemic Autoimmune Disease ◽

Pulmonary Alveolar Microlithiasis ◽

Slow Progression ◽

Autosomal Recessive Condition ◽

Alveolar Microlithiasis ◽

Synovial Membranes ◽

Inflammatory Polyarthritis

Pulmonary alveolar microlithiasis is a rare autosomal recessive condition that is characterized by the formation of excessive calcium phosphate microliths in the alveoli. Most patients are diagnosed in adulthood due to the slow progression of the disease. Children with this disease are asymptomatic, and changes in the lung parenchyma are usually discovered incidentally. The diagnosis is made by the combination of a positive chest imaging and histological examination. Rheumatoid arthritis (RA) is a chronic systemic autoimmune disease characterized by chronic seropositive symmetrical inflammatory polyarthritis with numerous extra-articular manifestations. It targets the lining of the synovial membranes, frequently affects females more than males, and is treated with the disease-modifying antirheumatic drugs (DMARDs). If left untreated, it leads to increased morbidity, mortality, and socioeconomic burdens. In this case, we report a 19-year-old young man who presented with clinical and radiographic features of PAM associated with RA.

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Taxane induced pulmonary toxicity in breast cancer.

Journal of Clinical Oncology ◽

10.1200/jco.2020.38.15_suppl.e15522 ◽

2020 ◽

Vol 38 (15_suppl) ◽

pp. e15522-e15522

Author(s):

Hrishi Varayathu ◽

Hephzibah Sara Mathew ◽

Vinu Sarathy ◽

Beulah Elsa Thomas ◽

Santosh Elluru Kumar ◽

...

Keyword(s):

Breast Cancer ◽

Pulmonary Toxicity ◽

Large Population ◽

Ground Glass ◽

Ct Findings ◽

Ground Glass Attenuation ◽

Her 2 ◽

Pulmonary Changes ◽

Tumor Types ◽

Interlobular Septal Thickening

e15522 Background: Taxanes are an integral part of chemotherapy for a broad range of tumor types. The usual toxicities associated with Taxane use are peripheral neuropathy, myelosuppression and musculoskeletal adverse effects. Very few studies have reported taxane induced pulmonary toxicity objectively. In our study we explored Taxane induced pulmonary toxicity based on discrete CT findings. Methods: 40 non-smokers diagnosed with Her-2 negative breast cancer who received Taxane monotherapy from 2017 to 2018 were retrospectively analyzed for pre and post therapy CT changes. Following CT findings were considered significant as pulmonary toxicity; (a) Nonspecific area with ground-glass attenuation, (b) multifocal areas of airspace consolidation, (c) patchy distribution of ground-glass attenuation accompanied by interlobular septal thickening and (d) extensive bilateral ground-glass attenuation or airspace consolidations with traction bronchiectasis. The CT findings were assessed independently by two radiologists and one pulmonologist. Results: Our study showed that 5 patients (12.5%) developed pulmonary changes on chest CT post Taxane therapy as summarized in Table. Conclusions: Our study shows that taxane has more potential to induce pulmonary toxicity than reported in present literature. Given that taxanes are the most widely used chemotherapy, it is of utmost necessity to be cognizant of this under-reported potentially fatal adverse effect in a large population. Further studies should be conducted to better understand the true potential, mechanisms and patterns of taxanes induced pulmonary toxicity. [Table: see text]

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Case series of Pulmonary Alveolar Microlithiasis from India

BMJ Case Reports ◽

10.1136/bcr-2018-227406 ◽

2019 ◽

Vol 12 (3) ◽

pp. e227406 ◽

Cited By ~ 1

Author(s):

Manu Chopra ◽

Manjit Sharad Tendolkar ◽

Vasu Vardhan

Keyword(s):

Rare Disease ◽

Clinical Symptoms ◽

Case Series ◽

Lung Parenchyma ◽

Response To Treatment ◽

Definitive Treatment ◽

Alveolar Type ◽

Pulmonary Alveolar Microlithiasis ◽

Alveolar Microlithiasis ◽

Calcific Deposits

Pulmonary alveolar microlithiasis (PAM) is a rare disease characterised by calcific deposits in lung parenchyma. PAM being a progressive disease with dissociation between severity of clinical symptoms and radiological picture, it is often detected incidentally. Mutations in the SLC34A2 gene encoding the type IIb sodium phosphate cotransporter in alveolar type II cells are considered to be involved in the pathogenesis of PAM. The majority of the patients are diagnosed usually between the ages of 20 and 40 years, although paediatric PAM has also been reported. Diagnosis is confirmed by combination of radiological features, bronchial lavage and histopathological testing. At present, lung transplant is the only definitive treatment available. Though rare, the prevalence of PAM is worldwide. Till June 2018, 86 cases have been reported from India and 1042 cases have been reported worldwide. We report three cases from India, including a student, cement factory worker and a tailor, which will highlight the varied clinical and radiological presentations of this rare disease along with the response to treatment.

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Pulmonary alveolar microlithiasis in children: radiographic and high-resolution CT findings.

American Journal of Roentgenology ◽

10.2214/ajr.168.1.8976922 ◽

1997 ◽

Vol 168 (1) ◽

pp. 63-65 ◽

Cited By ~ 35

Author(s):

T H Helbich ◽

C Wojnarovsky ◽

P Wunderbaldinger ◽

G Heinz-Peer ◽

I Eichler ◽

...

Keyword(s):

High Resolution ◽

Pulmonary Alveolar Microlithiasis ◽

High Resolution Ct ◽

Ct Findings ◽

Alveolar Microlithiasis

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Pulmonary Alveolar Microlithiasis: An Isolated Case in a Hispanic Male

Case Reports in Pathology ◽

10.1155/2020/6247920 ◽

2020 ◽

Vol 2020 ◽

pp. 1-4

Author(s):

Preethi Dileep Menon ◽

Sarah Hackman

Keyword(s):

Time Course ◽

Miliary Tuberculosis ◽

Lung Parenchyma ◽

Gene Encoding ◽

Pulmonary Alveolar Microlithiasis ◽

Bilateral Lung Transplantation ◽

Radiologic Findings ◽

Bilateral Lung ◽

Oxygen Requirements ◽

Alveolar Microlithiasis

Pulmonary alveolar microlithiasis (PAM) is an uncommon hereditary lung disease characterized by widespread deposition of calcium phosphate microliths within the alveolar spaces. It is considered an autosomal recessive disease with a mutation in a gene encoding a sodium phosphate cotransporter. The imaging findings in the early phase of disease can be mistaken for miliary tuberculosis or sarcoidosis. However, the classic radiologic findings in the later phases of disease show numerous opacities causing a “snowstorm” appearance to the lungs that corresponds with widespread deposition of microliths throughout the lung parenchyma. Although the disease often progresses over a slow time course, there are no effective therapies, and bilateral lung transplantation is recommended when there are increasing oxygen requirements or evidence of pulmonary hypertension.

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Pulmonary Alveolar Microlithiasis: A Case Report with Emphasis on Imaging Findings

Case Reports in Medicine ◽

10.1155/2010/819242 ◽

2010 ◽

Vol 2010 ◽

pp. 1-4 ◽

Cited By ~ 2

Author(s):

Guilherme Abdalla ◽

Edson Marchiori ◽

Gláucia Zanetti ◽

Antonio Mucillo ◽

Mariana Leite Pereira ◽

...

Keyword(s):

Case Report ◽

High Resolution ◽

Lung Biopsy ◽

Shortness Of Breath ◽

Imaging Findings ◽

Alveolar Space ◽

Pulmonary Alveolar Microlithiasis ◽

Ground Glass Attenuation ◽

History Of ◽

Alveolar Microlithiasis

Pulmonary alveolar microlithiasis (PAM) is a rare disease characterized by the presence of small calculi in the alveolar space. The authors report a case of a 21-year-old man with a 2-year history of shortness of breath on exertion and dry cough. Physical examination was altered only for crackles at auscultation. Pulmonary function revealed a mild restrictive ventilatory defect and the chest radiograph demonstrated paracardiac confluence of dense micronodular infiltrate. High-resolution CT scan revealed diffuse ground glass attenuation and septal thickening, more pronounced in lower pulmonary regions, with calcifications along the interlobar septa and subpleural regions. A transbronchial lung biopsy confirmed the diagnosis of PAM.

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