Long-term radiographic stability of diffuse pulmonary meningotheliomatosis
Danielle Victoria Lefebvre1, Saihariharan Nedunchezhian2, Rohan Srivastava3
1Internal Medicine, The George Washington University, Washington, District of Columbia, USA dlefebvre@mfa.gwu.edu.
Abstract:
Diffuse pulmonary meningotheliomatosis (DPM) is a rare lung disease characterised by numerous, bilateral, minute pulmonary meningothelial-like nodules that appear as small ground glass opacities which are often asymptomatic and of unclear clinical significance. This case involves a woman in her 80s presenting with progressive dyspnoea on exertion and a complicated medical history including anaemia and multi-valvular heart disease. Her investigations revealed stable radiographic findings of random, diffuse, bilateral ground glass nodules unchanged over 7 years and mild restrictive deficits on pulmonary function tests. Differential diagnoses included malignancy, infection and granulomatous diseases, but the findings favoured DPM due to the stable nature of the nodules. No biopsy was pursued, and the diagnosis was made radiographically after a multidisciplinary discussion. Her subacute symptoms were attributed to iron-deficiency anaemia and resolved with treatment. This case highlights the indolent nature of DPM with years of radiographic and functional stability.
Insights
Diffuse pulmonary meningotheliomatosis (DPM) is a rare lung condition presenting as small nodules. This case study shows DPM is often asymptomatic and stable over many years, with symptoms potentially linked to other conditions.
Area of Science:
- Pulmonology
- Radiology
- Pathology
Background:
- Diffuse pulmonary meningotheliomatosis (DPM) is a rare condition characterized by numerous small nodules in the lungs.
- These nodules often appear as ground-glass opacities and their clinical significance is frequently unclear.
- DPM is typically asymptomatic and may remain stable over extended periods.
Purpose of the Study:
- To present a case of DPM in an elderly patient with progressive dyspnea.
- To discuss the diagnostic challenges and radiographic features of DPM.
- To highlight the indolent nature and long-term stability of DPM.
Main Methods:
- Case report of an 80-year-old woman with a history of anemia and heart disease.
- Review of radiographic findings showing stable, diffuse, bilateral ground-glass nodules over 7 years.
- Pulmonary function tests revealing mild restrictive deficits.
- Multidisciplinary discussion for diagnosis, excluding malignancy, infection, and granulomatous diseases.
Main Results:
- Radiographic findings were stable over 7 years, favoring DPM over other diagnoses.
- Pulmonary function tests showed mild restrictive deficits.
- The patient's dyspnea resolved with treatment for iron-deficiency anemia, suggesting symptoms were unrelated to DPM.
- A diagnosis of DPM was made radiographically without biopsy.
Conclusions:
- DPM is an indolent lung disease with a typically stable radiographic and functional course.
- Symptoms in patients with DPM may be attributable to coexisting conditions.
- Radiographic diagnosis after multidisciplinary discussion is feasible for DPM.
