UNASSIGNED: Relapsing polychondritis (RP) is an uncommon autoimmune disease that causes inflammation of the cartilage and proteoglycan-rich structures, including the ear, nose, and airway. Paraneoplastic RP is a subset of RP that occurs in some individuals following the detection and treatment of certain types of cancers. FDG PET/CT helps with early diagnosis of RP, identifying inflammatory areas even in the absence of symptoms, and guiding the selection of appropriate biopsy sites. Here, we present a case of adenocarcinoma of the lung presenting with paraneoplastic symptoms of RP as initial presentation, and symptoms were resolved after 3 cycles of chemotherapy.

We present the case of a 61-year-old man who developed coronavirus disease 2019 (COVID-19) and died during treatment for relapsing polychondritis. The patient was intubated and treated with steroid pulse therapy, remdecivir, antibacterial agents, baricitinib, and tocilizumab. However, his respiratory condition worsened, and he died 108 days after disease onset. An autopsy revealed diffuse alveolar damage in the fibrotic phase in all lung lobes, diffuse pulmonary ossification, and cytomegalovirus-infected cells in the middle lobe of the right lung. We herein discuss the clinical features and pathological findings of COVID-19 in immunosuppressed patients.

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.
METHODS: 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.

暂无摘要。

Relapsing polychondritis (RP) is a rare autoimmune disease characterized by inflammation of the cartilage structures of the body with typical features of auricular chondritis, nasal and ocular inflammation, audio-vestibular damage, as well as respiratory tract manifestations. It is associated with several autoimmune diseases and many other disorders. Tumor necrosis factor alpha (TNFα) inhibitors treat many chronic inflammatory disorders. They have proven effective and relatively safe in many clinical trials and observational studies. However, several autoimmune phenomena and paradoxical inflammation have been described with TNFα inhibitors, among them RP. This report presents a 43-year-old man with psoriatic arthritis treated with ABP-501 (Amgevita), an adalimumab (ADA) biosimilar and who developed RP, 8 months after the initiation of the treatment. This, is the first report of RP development during TNFα inhibitors biosimilar. We concluded that rheumatologists dealing with patients treated with TNFα inhibitors (originators or biosimilars), should be aware of several paradoxical reactions which may emerge and RP, is one of them.

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare chronic inflammatory disease. The main clinical manifestation of SAPHO syndrome is an osteoarthropathy with cutaneous involvement. Relapsing polychondritis (RP) characterized by chronic inflammation and cartilage degeneration is a rare systematic autoimmune disease. Here we report a RP case in a SAPHO syndrome patient, in which auricularitis happened 10 years after the diagnosed as SAPHO syndrome. Tofacitinib treatment can alleviate the symptoms.

BACKGROUND: Relapsing polychondritis (RP) is a chronic and recurrent inflammatory disease of the cartilage tissues in the body. The cause of RP is unknown, and since it is a rare disease with symptoms that affect multiple organs, diagnosis is often delayed.
METHODS: A 62-year-old woman with no smoking history visited our institution complaining of fever, cough, and dyspnoea. Chest CT showed a stenosis from the left main bronchus to the left lower lobe branch. Bronchoscopy visualised intense erythema and oedema at the left main bronchus, with airway narrowing. Biopsy of the ear revealed degenerative vitreous cartilage and fibrous connective tissue with a mild inflammatory cell infiltrate. She was subsequently diagnosed with RP and administered systemic corticosteroid therapy. Her symptoms improved rapidly, and post-treatment bronchoscopy revealed that although mild erythema of the airway epithelium remained, oedema markedly improved, and the airway stenosis was resolved.
CONCLUSIONS: We report a case where pre-treatment bronchoscopy was able to visually confirm RP at the acute stage. Since RP is difficult to diagnose, severe airway narrowing can occur prior to diagnosis. Therefore, to determine the stage of the disease, it is helpful to perform bronchoscopic observation before treatment. However, bronchoscopic observation before treatment should be performed by experienced bronchoscopists due to the risk of airway obstruction.

暂无摘要。

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.

The present study reports the clinical data of a patient with small cell lung cancer who developed relapsing polychondritis. We report a case of a 57-year-old female presented with cough, expectoration, and fever. A Computed Tomography (CT) scan performed at the hospital revealed diffuse thickening of bronchial walls in both lungs. Bronchoscopy revealed that the tracheal mucosa was thickened, narrowed, and collapsed, and the bronchoscope could pass through. The bronchial mucosa on both sides was thickened and edematous, the surface was rough, each bronchus was narrow, and the intervertebral ridges were widened. Needle biopsy: considering small cell carcinoma in combination with immunohistochemical results. Her symptom was not improved after anti-infective therapy. The left auricle was red and swollen, the auricle collapsed, and the left eye had subconjunctival hemorrhage during her hospitalization without obvious cause. After multidisciplinary consultation, pulmonary small cell lung cancer cT0N2Mx rumen lymph node metastasis and RP were considered. Treatment: Prednisone, orally for RP. Chemotherapy combined with radiotherapy was given for small cell lung cancer. The chemotherapy regimen was carboplatin combined with etoposide. The patient has already been followed for 1 year after receiving chemoradiotherapy; the condition of the patient is stable at present. Based on the case of our patient, for cases of RP with symptoms such as auricle chondritis, ocular inflammatory disease, and nasal chondritis, we should pay great attention to whether the case is caused by lung cancer with relapsing polychondritis. Because of the rarity of the disease, the clinician should improve the recognition of the disease in order to strive for early diagnosis and therapy.