PO:25:082 | Progressive interstitial lung disease secondary to Sjögren's disease in a patient with inclusion body myositis complicated by dysphagia: a multidisciplinary approach and therapeutic challenges

Emanule Cocchiara¹, Francesco Falco¹, Lucia Spaggiari³, Francesca Cozzini^1|2, Lucia Dardani¹, Gianluigi Bajocchi¹, Andreina Manfredi^1|2, Carlo Salvarani^1|2 | ¹Dipartimento di Medicina, Clinica Reumatologica, Udine; ²Università degli studi di Modena e Reggio Emilia, Modena; ³AUSL area NORD, Guastalla-Montecchio, Italy 

Background. Inclusion Body Myositis (IBM) is a rare idiopathic inflammatory myopathy, characterized by an insidious onset with progressive muscle weakness and dysphagia. While its association with Sjögren’s syndrome (pSS) has been reported, this overlap is uncommon and often challenging to manage. We present the case of a patient affected by this dual condition, treated at our center.

Case report. A 70-year-old man, a former smoker, with a medical history of hypertension, hypothyroidism, transient ischemic attack, and prior intestinal obstruction surgery, presented to rheumatologic evaluation in 2017 with progressive dysphagia and muscle weakness. A diagnosis of IBM was established and confirmed by muscle biopsy. The patient was subsequently treated with intravenous immunoglobulins and pyridostigmine, achieving clinical improvement. In 2023, the patient developed new-onset xerophthalmia, xerostomia, and progressive exertional dyspnea. The comprehensive diagnostic work-up included a chest CT scan showing subpleural reticular thickening, mainly in the posterior-basal segments of the lower lobes, in the lateral segments of the middle lobe, and in the superior segments of the lingula. Pulmonary function testing showed a restrictive ventilatory defect and a moderate drop in DLCO. Serological analysis showed anti-Ro52 antibody positivity, and minor salivary gland biopsy demonstrated a focus score of 1. The overall findings were consistent with pSS. In 2024, due to worsening dyspnea, a new chest CT was performed, showing disease progression with the appearance of pulmonary fibrosis (radiologic UIP pattern). The case was discussed during a multidisciplinary meeting involving radiologists, pulmonologists, and rheumatologists. Due to the clinical and radiological deterioration of pSS-associated interstitial lung disease, treatment with nintedanib 150 mg twice daily, prednisone 50 mg with gradual tapering, and cyclophosphamide according to the NIH protocol was initiated. Maintenance therapy with mycophenolate mofetil 2 g/day was subsequently introduced. At follow-up spirometry, pulmonary function remained stable, although CT imaging showed mild disease progression with increased bronchiectasis. The patient’s clinical and therapeutic management is ongoing under multidisciplinary follow-up.

Conclusions. This case highlights two key aspects: the intrinsic complexity in managing inclusion body myositis associated with pSS, and the essential role of a multidisciplinary approach in defining not only the initial treatment but also subsequent therapeutic adjustments based on clinical and instrumental evolution. Furthermore, the combination of an immunosuppressive drug with an antifibrotic agent may represent an effective therapeutic strategy to achieve clinical, radiological, and functional stabilization of interstitial lung disease in patients with pSS.