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.

Severe respiratory dysfunction in a patient with relapsing polychondritis: should we treat the saddle nose deformity?

SUMMARY: We report a case of surgical correction of a saddle nose deformity, causing severe ventilation restrictions in a 42-year-old man diagnosed with relapsing polychondritis. Relapsing polychondritis is an autoimmune disorder, in which antibodies to type II collagen cause an inflammatory destruction of cartilage. If septal cartilage of the nose is involved, destruction leads to collapse of the dorsum of the nose, causing a saddle nose deformity. Patients suffer from a ventilation disorder of varying degree depending on the response to or onset of immunosuppressive therapy. In the described patient, the destruction of the nasal septum, in addition to unstable tracheal cartilage, caused a severe restriction in ventilation, with total collapse of the internal nasal valves during forced inspiration. To improve the function of the external airways the patient underwent surgery to reconstruct the nasal septum. Although cartilage grafts are the state of the art to reconstruct the nasal septum, we used a bone graft from the iliac crest, because the autoimmune polychondritis precludes cartilage grafting due to expected cartilage destruction. At follow up 2 years postoperatively no signs of bone resorption or deterioration of the improved airway were observed. We conclude that the use of bone grafts is a promising method to restore and improve ventilation disorders caused by a saddle nose deformity in relapsing polychondritis.

[Multiple aortic aneurysms in chronic atrophic polychondritis]. / Anévrysmes aortiques multiples au cours d'une polychondrite atrophiante.

BACKGROUND: Although rare, cardiovascular involvement is the second most frequent cause of mortality in chronic relapsing polychondritis behind tracheobronchial tree chondritis. The most frequent cardiovascular complications are valvulopathy and aortic aneurysm. CASE REPORT: We report a case of chronic relapsing polychondritis with multiple aortic aneurysms that were clinically silent but continued to progress despite systemic corticosteroids and immunosuppressive therapy. DISCUSSION: Progression of aortic aneurysms and extravascular disease do not appear to be correlated. Although the disease may appear to be in remission, vascular lesions can continue to progress independently. This case shows that medical treatment has little effect on the progression of these aneurysms. Consequently, it is necessary to opt for surgical therapy at the opportune moment.

Endobronchial stenting for respiratory complications in relapsing polychondritis.

Relapsing polychondritis is characterized by recurrent inflammation and destruction of the cartilage and connective tissue. Respiratory complications are frequently severe during the course of the disease and usually signal a poor prognosis. We report a case of a 47-year-old man with known relapsing polychondritis who presented with bilateral narrowing of the airways complicated by refractory cavitary Pseudomonas aeruginosa pneumonia. Despite an aggressive antibiotic regimen, the patient's pneumonia did not improve until bronchial stenting 4 months later.

Long-term management of polychondritis with serial tracheobronchial stents.

A 56-year-old woman was referred to our hospital for airway compromise. Relapsing polychondritis was diagnosed by bronchial cartilage biopsy. Bronchoscopy showed tracheobronchial malacia due to the disappearance of bronchial cartilage as a result of chondritis. Stent insertion using a Dynamic stent (Rüsch; Kernen, Germany), Ultraflex stent (Boston Scientific International, Colombes, France) and TM stent (Fuji Systems Corp, Tokyo, Japan) was repeated for more than 7 years. Dramatic improvement of dyspnea was obtained each time. The patient survived during this period without oxygen inhalation or mechanical ventilation before she suddenly died due to suffocation caused by difficulty of sputum discharge.

[Chronic atrophic polychondritis. A case report]. / Manifestations respiratoires de la polychondrite atrophiante.

Chronic atrophic polychondritis is a rare systemic disease characterized by recurring inflammation of cartilage of the ear, nose, larynx and of the tracheobronchial tree. Nowadays, it is considered to be an autoimmune disease. The case of a 25-year-old female patient hospitalized in our department for treatment of a dyspnea is herein presented. ORL laryngoscopic examination revealed a small sized larynx with a pronounced narrowing of its lumen and very "floppy" cartilage. Bronchoscopic findings were a circumferential stenosis along the entire trachea and the right major bronchi. Results from biopsies performed on the trachea and thyroid cartilage were in favor of chronic atrophic polychondritis. The course of the disease was characterized by an aggravation of the dyspnea with episodes of asphyxia resulting in a tracheotomy. The tracheotomy has not been closed to this day, due to the underlined disease of the patient.

Metallic tracheal stents: complications associated with long-term use in the upper airway.

The use of metallic airway stents for the treatment of benign airway stenosis is increasingly advocated; however, the long-term safety and efficacy of these devices has not been established. Three case studies involving late but significant proximal tracheal stent complications are reported: 1 related to proximal mechanical stent failure and 2 related to obstructing granulation tissue and stenosis at the proximal stent orifice. Placement of these stents in close proximity to the cricotracheal junction is thought to be primarily responsible for these complications as a result of the excess shearing forces created at the stent-mucosal interface by the differential motion of the stent relative to the rigid subglottic airway and the more distensible trachea. Although the use of metallic stents remains appropriate in cases in which there is a defined and relatively short-term end point for treatment, caution is urged in using them in the proximal trachea for long-term management of benign airway disease.

[Anesthesia in a patient with relapsing polychondritis].

A 46-year-old female with relapsing polychondritis (RP) was scheduled for surgical repair of saddle-nose and peri-chondritic ear. RP is a rare systemic, inflammatory, and destructive disease of the cartilaginous structures leading to multiple functional disorders in the affected organs. Preoperatively, her chest radiogram of the trachea showed narrowing in diameters which altered about 2 mm with expiration and inspiration. Propofol administered intravenously was given as induction and anesthesia was maintained with nitrous oxide-oxygen-sevoflurane with spontaneous ventilation using laryngeal mask airway. The operation was over uneventfully. Anesthesiologic management requires careful preoperative evaluation of vital organ functions, in particular, respiration.