Correct!
5. More than one of the above

Rheumatologic consultation with the initiation of immunomodulatory therapy is appropriate given the diagnosis of rheumatoid arthritis. Given that the patient’s thoracic symptoms are unabated and the right pleural effusion is recurrent following thoracoscopic surgery and repeated ultrasound-guided thoracenteses, and suspicion of trapped lung is present, surgical consultation for decortication is appropriate.
Rheumatologic consultation was obtained, and careful questioning did disclose past complaints of joint pain; radiography of the hands, elbows and feet did not disclose erosive change. She was begun on oral leflunomide therapy. Surgical consultation was again obtained and the consensus among the pulmonologist, rheumatologist, and thoracic surgeon was that decortication was appropriate. The patient underwent this surgery (Figure 9).

Figure 9. Intraoperative image during decortication of the right pleural space shows thickened pleura with adhesions to the underlying lung.

The resultant pathology of the surgery (Figure 10) showing chronic fibrosing pleuritis with old fibrous pleural peel with adhesions.

Figure 10. Histopathological specimen from thoracoscopic decortication (Hematoxylin & Eosin, 40x) shows a fibrous “pleural peel.”

Biopsy of the underlying right lung parenchyma showed foci chronic small airway remodeling with follicular bronchiolitis, mild chronic interstitial inflammation, and patchy interstitial fibrosis consistent with an early or evolving interstitial pneumonia. The pattern of interstitial fibrosis was mixed, with hybrid features of non-specific interstitial pneumonia and usual interstitial pneumonia.
The patient recovered well after surgery. Leflunomide therapy was stopped and rituximab was initiated. The presence of follicular bronchiolitis prompted therapy with inhaled corticosteroid and a long-acting beta-agonist, montelukast, and azathioprine. The patient is undergoing monitoring for therapy effectiveness and to assess need for a second immunosuppressive agent should the interstitial lung disease progress.
Diagnosis: Rheumatoid arthritis with rheumatoid-associated pleuritis, connective tissue associated interstitial lung disease.

References

  1. Aquino SL, Webb WR, Gushiken BJ. Pleural exudates and transudates: diagnosis with contrast-enhanced CT. Radiology. 1994 Sep;192(3):803-8. [CrossRef] [PubMed]
  2. Krishna R, Antoine MH, Rudrappa M. Pleural Effusion. 2024 Mar 6. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan–. [PubMed]
  3. Graham WR. Rheumatoid pleuritis. South Med J. 1990 Aug;83(8):973-5. [CrossRef] [PubMed]
  4. De Zorzi E, Spagnolo P, Cocconcelli E, et al. Thoracic Involvement in Systemic Autoimmune Rheumatic Diseases: Pathogenesis and Management. Clin Rev Allergy Immunol. 2022 Dec;63(3):472-489. [CrossRef] [PubMed]

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