Idiopathic Pulmonary Fibrosis Complicated by Adenocarcinoma and Organizing Pneumonia

Abstract

We describe a case of a 77-year-old male with idiopathic pulmonary fibrosis (IPF) complicated by lung adenocarcinoma and organizing pneumonia (OP). On initial examination, physical examination revealed fine crackles in both sides of his chest. There were no physical findings suggestive of collagen disease. Blood tests showed no elevation of C-reactive protein, and lactate dehydrogenase and Krebs von den Lungen-6 (KL-6) were within normal limits. A high-resolution CT (HRCT) of the chest showed multiple ground-glass opacities (GGOs) in both lungs, with consolidation and traction bronchiectasis in the left lower lobe. Although a bronchoscopy was performed, no diagnosis could be made. Bronchoalveolar lavage showed elevated lymphocytes, and treatment with prednisolone was started for the possibility of OP. Subsequent chest X-ray and chest CT showed worsening of the shadows over time, and shortness of breath on exertion progressed. Surgical lung biopsy revealed IPF complicated by adenocarcinoma and OP. Although the patient was treated with pemetrexed and carboplatin combination therapy, respiratory failure progressed, and palliative care was decided. There is no report of IPF complicated by adenocarcinoma and OP, and early surgical lung biopsy may be important for diagnosis.

Keywords: adenocarcinoma; idiopathic pulmonary fibrosis; lung cancer; organizing pneumonia; usual interstitial pneumonia.

Figure 1. Radiological findings at initial examination.

(A) Chest X-ray image. (B–E) Chest CT images showing multiple ground-glass opacities (GGOs) in both lungs (arrowheads), with consolidation and traction bronchiectasis in the left lower lobe (arrows).

Figure 2. Clinical course of the patient.

Although clarithromycin 200 mg and vonoprazan 20 mg were started for the possibility of chronic bronchitis and bronchiectasis due to aspiration or some other cause, no improvement was observed. BALF from left B5 showed elevated lymphocytes, and treatment with prednisolone 20 mg was started for the possibility of organizing pneumonia. The chest X-ray showed multiple GGOs that spread over time, and shortness of breath on exertion progressed.

Figure 3. Chest CT images of the patient over time and location of partial lung resection. The resection site was described by the bronchopulmonary segments (S1-10).

(A–C) The chest CT showed multiple GGOs that spread over time. Partial lung resection was performed from two locations (arrows): from the right middle lobe (S4), which was near normal grossly, and the right lower lobe (S10), which was highly fibrotic.

Figure 4. Pathological findings from the right middle lobe (S4).

Hematoxylin and eosin staining: (A) ×40, (B) ×100, and (C,D) ×400. (A,B) Adenocarcinoma (dotted lines) and OP (arrows). (C) Acinar-predominant adenocarcinoma. (D) Lepidic-predominant adenocarcinoma.

Figure 5. Pathological findings from the right lower lobe (S10).

Hematoxylin and eosin staining: (A,C) ×20 and (B,D) ×400. (A) Probable UIP pattern. (B) Fibroblastic foci. (C,D) Atypical cells (dotted line).