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Case Reports
. 2021 Jan 6;21(1):13.
doi: 10.1186/s12890-020-01386-2.

Successful treatment of severe interstitial pneumonia by removal of circulating autoantibodies: a case series

Philipp Eller  1 , Holger Flick  2 , Gernot Schilcher  3 , Florentine Moazedi-Fürst  4 , Kathrin Eller  5 , Emina Talakic  6 , Josef Hermann  4 , Yannick Allanore  7 , Horst Olschewski  2
Affiliations
Case Reports

Successful treatment of severe interstitial pneumonia by removal of circulating autoantibodies: a case series

Philipp Eller et al. BMC Pulm Med. .

Abstract

Background: There is only limited clinical data on the benefit of intense immunosuppression in patients with severe interstitial pneumonia associated with autoimmune features or new-onset connective tissue disease.

Case presentation: We here report a series of three consecutive patients suffering from severe interstitial lung disease necessitating endotracheal intubation and mechanical ventilation. The first two patients fulfilled many diagnostic criteria for new-onset antisynthetase syndrome, the third patient for systemic lupus erythematosus. We decided to implement aggressive immunosuppressive strategies in these critically-ill patients including therapeutic plasma exchange, immunoadsorption, cyclophosphamide and rituximab. All three patients improved from respiratory failure, were successfully weaned from the respirator, and eventually dismissed from hospital with ongoing immunosuppressive therapy.

Conclusion: Patients suffering from severe connective tissue disease-associated interstitial lung disease and respiratory failure may benefit from an aggressive immunosuppressive regimen and extracorporeal blood purification with rapid reduction of circulating autoantibodies. The impressive clinical responses in this small case series warrant a controlled clinical trial.

Keywords: Autoimmunity; Immunosuppression; Interstitial lung disease; Radiography; Vasculitis.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Patient 1. Chest computed tomography scan showing interstitial pneumonia on day 2 (a) and day 10 (b) with mainly axial distribution, subpleural consolidations and no honeycombing. Improvement in the chest computed tomography scan on day 37 (c). There was a close temporal association of the initiation of therapeutic plasma exchange on day 14 (blue arrow), and the nine immunoadsorptions (green arrows from day 19 until day 30) with recovery of oxygenation index (closed squares) (d)
Fig. 2
Fig. 2
Patient 2. Chest computed tomography showing progressive interstitial pneumonia on day 17 (a) and day 28 (b) with dense and partly consolidated infiltrates, fine reticular pattern and no honeycombing. Dramatic improvement in the chest computed tomography scan on day 71 (c). There was a close temporal association of the initiation of therapeutic plasma exchange on day 24 (blue arrow), and the infusion of cyclophosphamide (red arrow) and rituximab (violet arrow) on day 28 and 42 with recovery of oxygenation index (closed squares) (d)
Fig. 3
Fig. 3
Patient 3. Chest computed tomography showing interstitial pneumonia on day 2 (a), day 15 (b). Immunosuppression with cyclophosphamide (red arrow) was initiated on day 43, causing impressive clinical improvement. A control chest computed tomography was performed after 15 months (c). There was a close temporal association of the initiation of cyclophosphamide (red arrow) on day 43 with recovery of oxygenation index (d)
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References

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