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Pulmonary puzzle
Twenty-year-old patient with polyarthritis since childhood showing cysts and ground glass attenuation on HRCT
  1. Marília da Cruz Fagundes1,
  2. Thais Bianco1,
  3. Daniella Porfírio Nunes2,
  4. Tayza Katelline Danilau Ostroski3,
  5. Guilherme das Posses Bridi4,
  6. Alexandre Melo Kawassaki4,
  7. Carmen Silvia Valente Barbas4,
  8. Leonardo Oliveira Mendonça5,6,
  9. Samar Freschi Barros5,6,
  10. Jorge Kalil5,6,
  11. Anthony K Shum7,
  12. Dante Luiz Escuissato1
  1. 1 Department of Radiology, Universidade Federal do Parana Hospital de Clinicas, Curitiba, Brazil
  2. 2 Department of Pneumology, Universidade Federal do Parana Hospital de Clinicas, Curitiba, Brazil
  3. 3 Department of Pathology, Universidade Federal do Parana Hospital de Clinicas, Curitiba, Brazil
  4. 4 Department of Pneumology, Universidade de Sao Paulo Hospital das Clinicas, Sao Paulo, Brazil
  5. 5 Division of Clinical Immunology and Allergy, Universidade de Sao Paulo Hospital das Clinicas, Sao Paulo, Brazil
  6. 6 Laboratory of Immunology (LIM-19), Universidade de Sao Paulo Instituto do Coracao, Sao Paulo, Brazil
  7. 7 Department of Pulmonary and Critical Care, University of California, San Francisco, California, USA
  1. Correspondence to Marília da Cruz Fagundes, Department of Radiology, Universidade Federal do Parana Hospital de Clinicas, Curitiba, Parana, Brazil; mfagundes_90{at}hotmail.com

https://doi.org/10.1136/thorax-2023-220798

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    Clinical presentation

    We present a case of a 20-year-old woman reporting polyarthritis, muscle weakness, dyspnoea and multiple intensive care unit admissions due to respiratory failure, without haemoptysis, since 4 months of age. The mother had systemic erythematous lupus. On physical examination, digital clubbing and mild desaturation were found. Laboratory tests demonstrated 1:640 nuclear homogeneous pattern antinuclear antibodies (ANA) and erythrocyte sedimentation rate (ESR) of 61 mm. Spirometry exhibited forced vital capacity of 53%.

    High-resolution CT (HRCT) showed cysts and ground glass with reticular infiltrates (figure 1A-D). In 2006, she underwent a pulmonary biopsy, which revealed follicular bronchiolitis (figure 2A-D), without features of haemorrhage on biopsy such as haemosiderin laden macrophages.

    Figure 1

    HRCT appearances. (A, B) Multiple well-circumscribed cysts in the parenchyma (arrows) and parenchymal reticulation with architectural distortion (arrowheads); (C, D) scattered ground glass opacities, more extensive in the lower lobes (circles). These findings correspond to an interstitial lung disease of unclassifiable pattern with parenchymal cysts. HRCT, high-resolution CT.

    Figure 2

    Lung biopsy revealing. (A) Pulmonary parenchyma with lymphoid aggregates; (B) peribronchiolar (arrowhead) lymphoid aggregate with prominent germinative centre (arrow); (C) proliferation …

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    Footnotes

    • Contributors MdCF contributed to the acquisition, analysis and interpretation of data, writing and manuscript review. TB contributed to the acquisition, analysis and interpretation of data, writing and manuscript review. DPN contributed to the study design, acquisition, analysis and interpretation of data, writing and manuscript review. TKDO contributed to the analysis and interpretation of data and manuscript review. GdPB contributed to the analysis and interpretation of data, writing and manuscript review. AdMK contributed to the analysis and interpretation of data, writing and manuscript review. CSVB contributed to the analysis and interpretation of data, writing and manuscript review. LOM contributed to the acquisition, analysis and interpretation of data, writing and manuscript review. SFB contributed to the acquisition, analysis and interpretation of data, writing and manuscript review. JK contributed to the acquisition, analysis and interpretation of data, writing and manuscript review. AKS contributed to the analysis and interpretation of data and manuscript review. DE contributed to the study design, acquisition, analysis and interpretation of data, writing and manuscript review. All authors approved the final version of the article.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.