Tracheobronchitis and laryngitis associated with Crohn's disease.

We report a 68-year-old woman with tracheobronchitis and laryngitis associated with Crohn's disease (CD), which was discovered during the evaluation of suspected lung cancer. She had no symptoms induced by these upper airway diseases (UADs). Bronchoscopy revealed swelling of the epiglottis with edematous change and a mass like epiglottis fold. There were nodular and edematous changes in the trachea and bilateral main bronchus. Histological findings demonstrated infiltration by numerous lymphocytes and plasma cells. Dexamethasone as the premedication for chemotherapy against lung cancer was efficacious for these extraintestinal manifestations of CD. Our case was rare in that bronchial lesion and UADs appeared concomitantly.

Relapsing polychondritis with isolated tracheobronchial involvement complicated with Sjogren's syndrome: A case report.

BACKGROUND: Relapsing polychondritis (RP) is a rare, long-term, and potentially life-threatening disease characterised by recurrent paroxysmal inflammation that can involve and destroy the cartilage of the external ear, nose, larynx, and trachea. CASE SUMMARY: We here report a case of RP involving solely the tracheobronchial cartilage ring (and not the auricular. nasal or articular cartilage) complicated by Sjögren's syndrome in a 47-year-old female whose delayed diagnosis caused a sharp decline in pulmonary function. After corticosteroid treatment, her pulmonary function improved. CONCLUSION: In such cases, our experience suggested that 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) and fiberoptic bronchoscopy should be used to diagnose airway chondritis as relapsing polychondritis in the early phase of disease.

Pyoderma gangrenosum with pulmonary involvement: a pulmonary special report and literature review.

INTRODUCTION: Pyoderma gangrenosum (PG) is the prototypical neutrophilic dermatosis, commonly associated with inflammatory bowel disease, with pulmonary involvement being the commonest extracutaneous manifestation. PG with tracheobronchial involvement may present as upper airway obstruction and can be life-threatening. AREAS COVERED: To evaluate the clinical characteristics and predictors of PG with pulmonary involvement, we reported a case of PG with tracheobronchial involvement in China, and performed a literature retrieval on PG with pulmonary involvement. Demographic data, clinical presentations, underlying diseases, radiological and histopathological findings, treatments, and clinical outcomes were collected and subjected to statistical analysis. Forty-seven cases (including ours) were identified. Diseases associated with PG with pulmonary involvement were similar. Clinical presentation of PG with pulmonary involvement was nonspecific, with cough and dyspnea being the most common clinical symptoms, and pulmonary infiltrates and cavitation being the most common radiological signs. Further univariate analysis suggested stridor and young age (p < 0.01) may be predictors of tracheobronchial involvement in PG. EXPERT OPINION: PG with tracheobronchial involvement can be life-threatening, with young age and stridor being possible predictors. Therefore, prompt airway assessment and management are required in younger patients with PG with pulmonary involvement presenting with stridor.

Presentation, Diagnosis, and Management of Subglottic and Tracheal Stenosis During Systemic Inflammatory Diseases.

BACKGROUND: Subglottic stenosis (SGS) and tracheal stenosis (TS) are characterized by a narrowing of the airways. The goal of this study was to describe the characteristics and prognosis of nontraumatic and nontumoral SGS or TS. RESEARCH QUESTION: What are the inflammatory etiologies of SGS and TS, and what are their characteristics and prognosis? STUDY DESIGN AND METHODS: This multicenter, observational retrospective study was performed in patients with SGS or TS that was neither traumatic nor tumoral. RESULTS: Eighty-one patients were included, 33 (41%) with granulomatosis with polyangiitis (GPA) and 21 (26%) with relapsing polychondritis (RP). GPA-related stenoses exhibited circumferential subglottic narrowing in 85% of cases, without calcifications. In contrast, RP-related stenoses displayed anterior involvement in 76%, in a longer distance from vocal cords (4 cm), with calcifications in 62%, and extension to bronchi in 86%. Other diagnoses included bullous dermatoses (n = 3), amyloidosis (n = 3), sarcoidosis (n = 2), and Crohn's disease (n = 2); the remaining stenoses (n = 15) were idiopathic. SGS/TS was the initial manifestation of the disease in 66% of cases, with a median interval from stenosis to disease diagnosis of 12 months (interquartile range, 0-48 months). Despite the use of glucocorticoids in 80%, combined with methotrexate in 49%, endoscopic procedures were required in 68% of patients. Relapses of stenoses occurred in 76% without any difference between causes (82% in GPA, 67% in RP, and 75% in idiopathic SGS/TS). Three patients died due to the stenosis, two of RP and one of GPA. INTERPRETATION: These data show that GPA and RP are the two main inflammatory diseases presenting with SGS/TS. GPA-related stenoses are mostly subglottic and circumferential, whereas RP-related stenoses are mostly tracheal, anterior, and calcified with a frequent extension to bronchi. Relapses of stenoses are common, and relapse rates do not differ between causes. Diagnosis and management of SGS/TS require a multidisciplinary approach.

Hereditary lysozyme amyloidosis with sicca syndrome, digestive, arterial, and tracheobronchial involvement: case-based review.

Lysozyme amyloidosis (ALys) is a rare autosomal dominant hereditary systemic amyloidosis associated with a large spectrum of clinical manifestations. ALys phenotype mainly involves the digestive tract, liver and spleen, kidneys, lymph nodes, skin, and lachrymal and salivary glands. Very recently, cardiac involvement and peripheral neuropathy associated with a new p.Leu102Ser variant of lysozyme have been documented. In the present observation, we extend the phenotypic heterogeneity of ALys to the tracheobronchial tree with histologically proven bronchial ALys-amyloid deposits. We report the case of a 62-year-old man of Italian origin (Piedmont) diagnosed with ALys associated with the p.Trp82Arg variant. The patient complained of upper digestive symptoms, sicca syndrome, and lately recurrent pulmonary infections. Thoracic endoscopy revealed a fragile, inflammatory, and granulomatous aspect of the bronchi. Amyloid deposits were observed in the upper digestive tract, salivary glands, temporal artery, and tracheobronchial tree. Symptomatic treatment was offered. Recurrent pulmonary infections occurred during the follow-up. Lung involvement in hereditary ALys has only been exceptionally described. Although vascular involvement has already been reported in ALys in many organs, it never concerned cranial arteries. This case highlights the systemic nature of the amyloid protein variant deposits and expands the spectrum of clinical manifestations to chest involvement. The literature review highlights that hereditary ALys with the p.Trp82Arg variant is frequent in patients coming from Piedmont (Italy). Due to diffuse organs involvement related to ALys, it is important not to misdiagnose ALys for AL amyloidosis, the most frequent form of amyloidosis.

Tracheal Involvement in Crohn Disease: the First Case in Korea.

Respiratory involvement in Crohn disease (CD) is rare condition with only about a dozen reported cases. We report the first case of CD with tracheal involvement in Korea. An 18-year-old woman with CD was hospitalized because of coughing, dyspnea, and fever sustained for 3 weeks. Because she had stridor in her neck, we performed computed tomography of the neck, which showed circumferential wall thickening of the larynx and hypopharynx. Bronchoscopy revealed mucosal irregularity, ulceration, and exudates debris in the proximal trachea, and bronchial biopsy revealed chronic inflammation with granulation tissue. Based on these findings, we suspected CD with tracheal involvement and began administering intravenous methylprednisolone at 1 mg/kg per day, after which her symptoms and bronchoscopic findings improved.

Tracheal Involvement in Crohn Disease: the First Case in Korea

Respiratory involvement in Crohn disease (CD) is rare condition with only about a dozen reported cases. We report the first case of CD with tracheal involvement in Korea. An 18-year-old woman with CD was hospitalized because of coughing, dyspnea, and fever sustained for 3 weeks. Because she had stridor in her neck, we performed computed tomography of the neck, which showed circumferential wall thickening of the larynx and hypopharynx. Bronchoscopy revealed mucosal irregularity, ulceration, and exudates debris in the proximal trachea, and bronchial biopsy revealed chronic inflammation with granulation tissue. Based on these findings, we suspected CD with tracheal involvement and began administering intravenous methylprednisolone at 1 mg/kg per day, after which her symptoms and bronchoscopic findings improved.

Granulomatosis with polyangiitis: endoscopic management of tracheobronchial stenosis: results from a multicentre experience.

OBJECTIVES: Tracheobronchial stenosis (TBS) is noted in 12-23% of patients with granulomatosis with polyangiitis (GPA), and includes subglottic stenosis and bronchial stenosis. We aimed to analyse the endoscopic management of TBS in GPA and to identify factors associated with the efficacy of endoscopic interventions. METHODS: We conducted a French nationwide retrospective study that included 47 patients with GPA-related TBS. RESULTS: Compared with patients without TBS, those with TBS were younger, more frequently female and had less frequent kidney, ocular and gastrointestinal involvement and mononeuritis multiplex. Endoscopic procedures included 137 tracheal and 50 bronchial interventions, mainly endoscopic dilatation, local steroid injection and conservative laser surgery, and less frequently stenting. After the first endoscopic procedure, the cumulative incidence of endoscopic treatment failure was 49% at 1 year, 70% at 2 years and 80% at 5 years. Factors significantly associated with a higher cumulative incidence of treatment failure were a shorter time from GPA diagnosis to endoscopic procedure [hazard ratio (HR) 1.08 (95% CI 1.01, 1.14); P = 0.01] and a bronchial stenosis [HR 1.96 (95% CI 1.28, 3.00); P = 0.002]. A prednisone dose ≥30 mg/day at the time of the procedure was associated with a lower cumulative incidence of treatment failure [HR 0.53 (95% CI 0.31, 0.89); P = 0.02]. CONCLUSION: TBS represents severe and refractory manifestations with a high rate of restenosis. High-dose systemic CSs at the time of the procedure and increased time from GPA diagnosis to bronchoscopic intervention are associated with a better event-free survival. In contrast, bronchial stenoses are associated with a higher rate of restenosis than subglottic stenosis.

A Case of Relapsing Polychondritis Presenting with Diffuse Tracheobronchial Involvement

Relapsing polychondritis is a rare autoimmune disease involving the cartilaginous structures of the whole body. Its diagnosis can be difficult when the typical clinical features such as auricular chondritis are absent. Here, we report on a case of a 51-year-old female who presented with cough, dyspnea, and polyarthritis. Chest computed tomography showed the diffuse involvement of tracheobronchial cartilage. According to Damiani's criteria, she was diagnosed as relapsing polychondritis even though there was no unique involvement of auricular cartilage, and high dose steroid and immunosuppressive therapy were then started. This case indicated that patients who have tracheobronchial cartilage involvement without definite auricular chondritis should be considered for relapsing polychondritis as a differential diagnosis.

Extranodal Marginal Zone Lymphoma Occurring along the Trachea and Central Airway

Extranodal marginal zone lymphoma is a low-grade B cell lymphoma that presents with an indolent clinicopathologic nature. Although this tumor can occur in various sites, including the gastrointestinal tract and lungs, it develops and spreads extremely rarely along the trachea and central airway. We report a case of extranodal lymphoma of mucosa-associated lymphoid tissue with tracheobronchial involvement. An 83-year-old woman presented with a cough and dyspnea. Bronchoscopic evaluation confirmed diffuse, multiple nodular lesions in both the trachea and large bronchi, and she was diagnosed with an extranodal marginal zone lymphoma of the tracheobronchial tree. After systemic chemotherapy, she survived for more than 18 months.