Endobronchial obstruction in connective tissue diseases: an uncommon but life threatening complication: two case reports.

BACKGROUND: Granulomatosis with polyangiitis and relapsing polychondritis are rare, multisystemic and potentially life-threatening connective tissue diseases. We present two cases of severe endobronchial obstruction in the aforementioned conditions and discuss difficulties with detection and treatment. Despite differing underlying pathophysiologies, endobronchial disease is a less frequently reported but serious complication of both conditions. CASE PRESENTATION: Case 1, a 31-year-old South Asian woman with relapsing polychondritis, required partial tracheal resection and reconstruction in combination with immunosuppressive therapy to achieve respiratory recovery following collapse of her right main bronchus and a stricture in her left main bronchus. Case 2, a 22-year-old Caucasian male with granulomatosis with polyangiitis, underwent surgical resection of an endobronchial growth causing occlusion of his right main bronchus. Although his respiratory status was initially stabilised with increased immunosuppression, he continues to have disease progression in spite of this. CONCLUSIONS: Our cases highlight the importance of a multidisciplinary approach combining immunosuppression with supportive care and judicious use of surgical interventions in select cases. A further review of the literature shows endobronchial obstruction is potentially under-reported due to overlap in connective tissue disease symptomatology and there is no consensus on best practice.

Respiratory Failure during BIS-Guided Sedation in a Patient with Relapsing Polychondritis: A Case Report.

Relapsing polychondritis (RP) is a rare autoimmune disorder that causes inflammation and deterioration of cartilaginous structures such as the ears, nose, joints and laryngotracheobronchial tree. A 42-year-old man receiving treatment for RP underwent open reduction and internal fixation of a femur fracture under spinal anesthesia and with sedation by propofol and remifentanil. The level of sedation was monitored via a bispectral index (BIS), and maintained at between 60 and 80. At the end of the operation, he lost consciousness and displayed weak respiratory effort. During mask ventilation, the patient was judged to have respiratory failure due to high end-tidal CO2 (EtCO2) concentration and respiratory acidosis in an arterial-blood-gas analysis (ABGA). Ventilation through a properly inserted laryngeal-mask-airway or endotracheal intubation were impossible; instead, a surgical tracheotomy was performed. After recovering from respiratory failure with ventilatory support in the intensive care unit (ICU), he experienced the same symptoms three more times, requiring ventilatory support. He was discharged with bilevel positive-airway-pressure (BiPAP), after successful adaptation.

Single-incision laparoscopic colectomy for ascending colon tumor with relapsing polychondritis.

A 69-year-old man underwent single-incision laparoscopic colectomy for a colon tumor. He had comorbid relapsing polychondritis, an uncommon and intractable chronic inflammatory disease that shows various symptoms and systemically invades the cartilaginous tissue throughout the body. In this case, the bronchial wall was edematous and the glottis was incompetent. Steroids were also administered. Although the patient had high-risk factors for surgical complications, none occurred during the perioperative period. Once the C-reactive protein value returned to within the normal range after surgery, we reduced the steroid dose. This is the first report of single-incision laparoscopic colectomy for a patient with relapsing polychondritis.

Concomitant aortic valve replacement, mitral valve replacement, and coronary artery bypass grafting for aortic stenosis and mitral regurgitation in a patient with relapsing polychondritis.

Relapsing polychondritis is a rare multi-system disease characterized by inflammation in cartilaginous structures and other connective tissues. Cardiovascular complications occur in 10-51% of the patients. We report a case of concomitant aortic valve replacement, mitral valve replacement, and coronary artery bypass grafting in a patient with relapsing polychondritis. A 71-year-old female with relapsing polychondritis on prednisolone (5 mg/day) for 15 years presented at our hospital for further evaluation of valvular disease. Severe aortic stenosis and severe mitral regurgitation were diagnosed. We performed aortic and mitral valve replacement. During surgery, we found connective tissue surrounding the intima of the sinus of Valsalva and stenosis of the right coronary artery ostium, which was not noted on preoperative coronary angiography. We removed the tissue and performed bypass grafting to the right coronary artery. Postoperative recovery was uneventful, and she was discharged 27 days after surgery.

Long-Term Outcome of Metallic Stenting for Central Airway Involvement in Relapsing Polychondritis.

BACKGROUND: Placement of uncovered self-expandable metallic stents was found to successfully alleviate critical airflow limitation in patients with relapsing polychondritis (RP) with central airway involvement by several reports. However, the long-term outcome of airway metallic stenting in patients with RP remain unclear. METHODS: We retrospectively analyzed patients with RP who underwent airway metallic stenting with the use of fiberoptic bronchoscopy between September 1, 2009, and October 1, 2017, in Shanghai. Outcome measurements, including modified Medical Research Council (mMRC) dyspnea score, 6-minute walk distance (6MWD), spirometry, and bronchoscopic findings, as well as adverse events after stent placement, were collected. RESULTS: A total of 27 patients were included; the median patient age was 58 years (range: 41 to 74 years), and 19 were men (70.4%). Nineteen uncovered self-expandable metallic stents were placed in the trachea and 39 in the main bronchi. The median follow-up time was 50.5 months (range: 6 to 100 months). The baseline forced expiratory volume in 1 second (FEV1) percentage predicted (%pred), FEV1/forced vital capacity (FVC), and peak expiratory flow (PEF) was 24.2 ± 3.7, 27.2 ± 5.6, and 0.99 ± 0.21 L/min, respectively. One day after the procedure, improvement from baseline in FEV1 %pred, FEV1/FVC, and PEF was 17.9 ± 8.9 (p = 0.001), 19.8 ± 10.9 (p = 0.002), and 0.69 ± 0.44 L/min (p = 0.001), respectively. Changes in the following variables were also statistically and clinically significant: 6MWD of 193.7 ± 83.4 m; mMRC dyspnea score of -1.2 ± 0.4 points (both p < 0.05). The improvements were maintained at 5 years: a mean change in FEV1 %pred, FEV1/FVC, PEF, 6MWD, and mMRC score was 19.5 ± 6.7, 13.9 ± 5.0, 0.82 ± 0.40 L/min, 134.7 ± 66.2 m, and -0.83 ± 0.29 points, respectively. Cough, foreign body sensation, mucus production, and granulomas were common adverse events, occurring in 48.1% (13 of 27), 40.7% (11 of 27), 29.6% (8 of 27), and 25.9% (7 of 27) of the subjects. However, none of those complications were severe enough to require urgent bronchoscopic interventions. CONCLUSIONS: Airway metallic stenting in patients with RP with central airway involvement resulted in long-term clinical benefits in lung function, exercise tolerance, and dyspnea with an acceptable safety profile.

Heart Transplantation as Last Resort Treatment for Relapsing Polychondritis With Severe Cardiac Involvement.

Relapsing polychondritis is a rare multisystem autoimmune disease characterized by recurrent inflammation and destruction of different cartilaginous and proteoglycan-rich structures like cardiovascular system. We reported a case of heart transplantation in a 20-year-old man having a relapsing polychondritis with severe multiple cardiovascular involvement. The immunosuppressive treatment used to prevent graft rejection allowed to turn out the inflammatory disease. Cardiac transplantation may be considered as last resort treatment option in relapsing polychondritis with intractable extensive heart lesions.

Laryngotracheal reconstruction for relapsing polychondritis: case report and review of the literature.

BACKGROUND: Relapsing polychondritis is a multi-system autoimmune disease characterised by the inflammation and destruction of cartilaginous structures. The most common sites are the pinna, nose, laryngotracheobronchial tree and peripheral joints. Airway involvement occurs in up to half of patients affected, at any disease stage. It is the most severe and life-threatening aspect of the disease, and proves to be a therapeutic challenge. OBJECTIVES: This article reports our experience of performing laryngotracheal reconstruction in a patient with relapsing polychondritis. A review of the literature is presented, with a focused discussion of airway treatment options. METHODS: Laryngotracheal reconstruction for relapsing polychondritis was performed using hyoid bone pedicled on sternohyoid muscle. CONCLUSION: Airway management in relapsing polychondritis can improve quality of life and palliate patients effectively.

Surgical reconstruction of auricular relapsing polychondritis.

Relapsing polychondritis (RP) is a multiple system disease of unknown cause characterized by episodic inflammation of cartilage and potentially progressive degeneration of cartilaginous tissue. Solitary auricular cartilage involvement without any inflammatory symptoms is very uncommon and surgical treatment is rarely recommended. We present a 53-year-old Chinese male patient who had undergone a mass resection on the right ear in December 2008 and 1 year later had developed another mass at the same spot. A mass resection and reconstructive surgery were performed and the pathologic result of the mass was auricular RP. Although RP is rarely seen by plastic surgeons, we should have sufficient information about this disease. This case demonstrates a simple and straight surgical approach to obtain an excellent ear appearance.

Laryngotracheal reconstruction at relapsing polychondritis.

Relapsing polychondritis (RP) is characterized by chronic, recurrent episodes of inflammation with eventual degeneration of cartilaginous tissues including the ears, nose, larynx, trachea, and so on. Tracheobronchomalacia and airway stenosis may be seen in RP. In this report, we describe 3 female patients presenting with progressive dyspnea owing to laryngotracheal involvement of RP who underwent laryngotracheal reconstruction.