Respirology Case Reports最新文献

We report a 67-year-old man with a relapse characterised by fever, respiratory failure and bilateral infiltrates following completion of a short-term empirical methylprednisolone regimen for a similar episode. Serum, upper respiratory tract and bronchoalveolar lavage samples were negative for infection, whereas an extensive antibody panel showed no remarkable findings. The presence of migratory opacities on chest imaging, a mixed cellular lavage pattern and prior steroid responsiveness supported a provisional diagnosis of cryptogenic organising pneumonia. During outpatient follow-up, frequent exacerbations occurred, barring any steroid tapering attempts. An individualised pathophysiological hypothesis is proposed for the recalcitrant course following incidental detection of mannose-binding lectin deficiency. Given the potential role of mannose binding lectin in apoptotic cell clearance and modulation of inflammation, we postulate an impingement on the disease trajectory, which has been previously observed in chronic obstructive pulmonary disease, bronchiectasis and bronchiolitis obliterans post-transplant.

Sampling aortopulmonary window lymph nodes is technically challenging and often requires surgical approaches. We report a minimally invasive robotic-assisted transbronchial biopsy of AP window lymph nodes using cone-beam CT guidance, enabling diagnosis of small cell lung cancer without surgical mediastinal biopsy.

A 74-year-old man with prior stage IE gastric MALT lymphoma presented with blood-stained sputum. Autofluorescence imaging (AFI) bronchoscopy revealed bilateral endobronchial lesions; with biopsies confirmed MALT lymphoma. Positron emission tomography computed tomography showed right upper lobe lesion without nodal disease. He received rituximab, with complete metabolic response. AFI facilitated early airway-focused diagnosis.

IgG4-related disease (IgG4-RD) is a fibroinflammatory disease characterised by elevated serum IgG4 levels and marked infiltration of IgG4-positive plasma cells. Here, we present the case of a 49-year-old man who presented with dysphagia and hoarseness caused by an anterior mediastinal mass. As the fluorodeoxyglucose positron emission tomography (FDG-PET) SUVmax was 6.68 (early and delayed), malignancy was initially suspected. Serum IgG4 levels were slightly elevated at 148 mg/dL. Surgical biopsy revealed dense lymphoplasmacytic infiltration with storiform fibrosis and numerous IgG4-positive plasma cells, fulfilling the diagnostic criteria for IgG4-RD. Treatment with high-dose prednisolone (60 mg/day) rapidly resolved symptoms and markedly reduced the mass within 6 months. This case highlights the importance of considering IgG4-RD in the differential diagnosis of mediastinal tumours, even when FDG-PET shows abnormal accumulation and serum IgG4 levels are not particularly high.

Behcet's disease is a chronic, multisystem variable vessel vasculitis characterised by recurrent oral and genital ulcers, ocular inflammation and a wide range of systemic manifestations. Pseudo-Behcet's syndrome refers to a condition that mimics these clinical features but arises from distinct etiologies. We present a case of a 33-year-old male with year-long recurrent oral ulcers and intermittent abdominal pain, followed by scrotal ulcers, severe fatigue, weight loss and appetite loss. Chest imaging demonstrated bilateral consolidations with cavitation and right-sided pleural effusion, prompting consideration of a Behcet's mimic. Thoracoscopic pleural biopsy revealed acid-fast bacilli, confirming tuberculosis. A diagnosis of Pseudo-Behcet's secondary to tuberculosis was made. Initiation of anti-tubercular therapy led to complete resolution of symptoms within 4 months. A literature review identified nine cases (including the index case) in which tuberculosis presented with Behcet's-like features, underscoring the complex association between Behcet's disease and tuberculosis. This case underscores the need to consider tuberculosis as a Behcet's disease mimic, particularly in endemic areas, to prevent misdiagnosis and inappropriate immunosuppression.

Pulmonary involvement of pyoderma gangrenosum (PG) is rare and has been observed in few patients with rheumatoid arthritis (RA). We describe the case of a 72-year-old woman with RA who simultaneously developed multiple cavitary pulmonary nodules and worsening cutaneous PG. Differential diagnoses included PG-related pulmonary lesions and rheumatoid nodules, which may cavitate and are difficult to distinguish radiologically and histologically. Bronchoscopy and video-assisted thoracoscopic surgery were performed. A histopathological evaluation revealed nonspecific findings, and infection was considered unlikely. Infliximab (IFX) was initiated because limited reports have suggested its potential benefit for pulmonary PG and potential usefulness for lesions that represent rheumatoid nodules. To the best of our knowledge, this is the first report of pulmonary involvement in a patient with PG and RA that was successfully treated with IFX. This case highlights diagnostic challenges and suggests that tumour necrosis factor-α inhibitors may be a promising therapeutic option for select cases.

Methicillin-susceptible Staphylococcus aureus (MSSA) necrotizing pneumonia can progress to severe disease, particularly when Panton-Valentine leukocidin (PVL) is involved. Here we report the case of a previously healthy 56-year-old man who developed severe necrotizing pneumonia due to MSSA despite the absence of major toxin genes including PVL. The patient reported a 10-day history of low-grade fever and was admitted for bilateral pneumonia with sepsis. Despite antibiotic therapy, his condition rapidly progressed, and chest computed tomography revealed extensive ground-glass opacities and multiple cavitary lesions. MSSA was isolated from blood and pleural fluid cultures, and polymerase chain reaction confirmed negativity for PVL, toxic shock syndrome toxin-1, and exfoliative toxin genes. Although the infection improved with antibiotics including clindamycin, a potent toxin suppressor, the patient experienced recurrent refractory pneumothorax due to persistent cavitary lesions that led to prolonged respiratory failure. PVL-negative MSSA necrotizing pneumonia can be a life-threatening disease with long-term complications.

This report describes a rare case of a bilio-bronchial fistula in a 60-year-old man with a complex history of hepatocellular carcinoma, treated with resection, trans-arterial chemoembolisation and microwave ablation. Following ablation, a pre-existing sterile liver seroma became infected, forming an abscess that eroded through the diaphragm, culminating in a fistula between the biliary tree and the bronchial system. The pathognomonic presentation of bilioptysis (bilirubin-rich sputum) confirmed the diagnosis, which was further visualised on endoscopic retrograde cholangiopancreatography. Management successfully focused on biliary system decompression via endoscopic stent placement into the culprit duct. This intervention resulted in the immediate cessation of bilioptysis and subsequent fistula healing, thereby avoiding high-risk surgical repair. This case highlights that bilio-bronchial fistula is a potential complication of liver ablation therapies and underscores the critical role of endoscopic biliary drainage as a first-line, minimally invasive treatment to reduce intrabiliary pressure and promote spontaneous closure.

A right post-eparterial bronchus (PEB) is a rare anatomical variant where an accessory bronchus arises inferior to the standard right upper lobe bronchus to supply the right upper lobe (RUL). We present a case of a 13 mm solitary RUL nodule in a 36-year-old immunosuppressed patient, uniquely supplied by a displaced PEB. Successful access was achieved using an ultrathin bronchoscope (UTB) due to the PEB's narrow lumen and acute angulation. The lesion was localised with radial endobronchial ultrasound, and definitive tool-in-lesion confirmation was obtained using a mobile Cone Beam CT system. Cryobiopsy revealed a non-caseating granuloma, with fungal culture identifying Penicillium species. This case highlights the essential role of advanced guided bronchoscopy techniques-specifically UTB navigation and intra-procedural 3D imaging-in successfully accessing and diagnosing infective pulmonary lesions situated in rare, anatomically complex airways, which may be susceptible to infection due to impaired sputum clearance.

Anti-eukaryotic initiation factor 2B (anti-eIF2B) is a rare systemic sclerosis (SSc)-related autoantibody. Longitudinal treatment data for anti-eIF2B-positive SSc-associated interstitial lung disease (ILD) remain limited. We herein describe the case of a 46-year-old man with limited cutaneous SSc who developed lower-lobe-predominant fibrotic ILD. Major SSc autoantibodies were negative, and an antigen-specific panel identified anti-eIF2B. Mycophenolate mofetil was initiated; however, serial high-resolution computed tomography showed incremental fibrotic progression, and the diffusing capacity declined, consistent with progressive SSc-ILD, prompting the addition of nintedanib to the ongoing therapy. This case supports testing for minor specificities when SSc-ILD is suspected despite negative results for major autoantibodies and adds new information on the post-treatment disease course and therapeutic selection in this serological subset. Further case accumulation with standardised longitudinal outcomes is needed to define the prognosis and treatment response.