A 70-year-old female with spontaneous lung torsion secondary to hypocomplementemic urticarial vasculitis was successfully treated with lung volume reduction, resection, and rituximab.
Case Report (n=1)
Spontaneous lung torsion can be a rare complication of hypocomplementemic urticarial vasculitis (HUVS) requiring surgical intervention and targeted immunosuppression.
Abstract Lung torsion is an exceedingly rare pulmonary complication, with a reported incidence between 0.089% to 0.3%. It typically occurs following thoracotomies or video-assisted thoracoscopic surgeries (VATS). 1 There are few case reports of spontaneous lung torsions, mostly from anatomic anomalies and disturbances within the pulmonary vasculature and lung tissue (e.g. tumors, pleural effusions, pneumothoraces). 2 The treatment consists of detorsion or lobectomy, in conjunction with treatment/avoidance of the underlying etiology. 3 We present a case of spontaneous lung torsion secondary to hypocomplementemic urticarial vasculitis (HUVS), treated with lung volume reduction and rituximab. A 70-year-old female presented to the office with dyspnea, described as abrupt in onset one year prior. Her pulmonary history was significant for asthma, allergic rhinitis, seasonal allergies, and COPD (FEV1/FVC ratio of 0.55, FEV1 25%, RV/TLC 194% on PFTs) despite being a never smoker. CT chest was performed for endobronchial valve (EBV) consideration; it revealed right bronchial torsion involving RUL and RML, RLL hyperinflation, and diffuse emphysema, greatest in the lower lobes. She subsequently underwent a right VATS, converted to thoracotomy, upper lobe lung volume reduction with resection of torsed lung, and mechanical pleurodesis. Pathology revealed panacinar emphysema, pulmonary vasculopathy, and small vessel vasculitis, suggestive of pulmonary HUVS. This was confirmed by Rheumatology and Immunology evaluations, which revealed low serum complements and CH50, and elevated anti-C1q antibodies. The patient was treated with budesonide/glycopyrrolate/formoterol, prednisone, and rituximab. Lung torsion is a rare diagnosis made via CT scan or bronchoscopy. It is life-threatening, with complications including bronchovascular compromise, airway collapse, and necrosis of the lung. 1 As seen in our case, an interprofessional care team was imperative to perform VATS, establish a diagnosis, and quickly initiate rituximab therapy. HUVS produces circulating immune complexes, which can deposit in pulmonary microvasculature and lead to destruction of alveolar walls. This causes emphysematous physiology, leading to severe bullous disease, loss of normal lung architecture, and anatomic disturbances that may predispose to lung torsion. The standard of treatment is detorsion of the lung, or lobectomy if detorsion fails. The underlying cause should be identified and treated to prevent recurrence. In our case, rituximab was used to treat HUVS by depleting the patient’s CD20 B-cells, thereby reducing the production of pathogenic autoantibodies and limiting further emphysematous progression. In any patient with spontaneous lung torsion, the decision to undergo surgery and treatment should be carefully considered, with a thorough risk-benefit discussion involving the patient’s multidisciplinary team. This abstract is funded by: None
Dunne et al. (2026) conducted a case report in Spontaneous lung torsion secondary to hypocomplementemic urticarial vasculitis (HUVS) (n=1). Lung volume reduction, resection, and rituximab was evaluated. A 70-year-old female with spontaneous lung torsion secondary to hypocomplementemic urticarial vasculitis was successfully treated with lung volume reduction, resection, and rituximab.