Cryptogenic organizing pneumonia (COP) is an important subtype of idiopathic interstitial pneumonia, triggered by abnormal inflammatory responses of the lung to unknown stimuli. The disease shows good initial sensitivity to glucocorticoid therapy, but ~50% of patients experience recurrence after glucocorticoid dose reduction or withdrawal. Cases of multiple recurrences (≥3 times) are rarely reported clinically, and no unified consensus has been formed on their diagnosis and treatment strategies, making clinical management challenging. In the present study, a 53-year-old female patient was admitted to hospital for the first time, presenting with a recurrent cough for >1 month. Chest computed tomography (CT) revealed multiple areas of increased density in both lungs, with no notable absorption of the lesions following antimicrobial therapy. A CT-guided percutaneous lung biopsy revealed widened alveolar septa with numerous fibroblast clusters and foam cells within the alveolar spaces, consistent with a diagnosis of COP. Following treatment with methylprednisolone combined with anti-infective therapy, the lesions largely resolved. The patient was discharged on a tapering regimen of oral prednisone, completing a total treatment course of 2 months. After discontinuing medication for 18 months, the patient was readmitted due to chest tightness. CT revealed recurrence of the bilateral pulmonary lesions. Treatment with methylprednisolone combined with anti-infective therapy was administered. Following discharge, oral methylprednisolone therapy for 5 months resulted in complete resolution of the lesions. After 16 months of low-dose oral methylprednisolone maintenance therapy, the patient presented for a third visit with a cough and chest tightness. Chest CT confirmed recurrence of the COP. Symptoms improved after oral prednisone administration, but radiographic changes remained minimal. Multiple recurrent COP has clinical characteristics of migratory lesions, glucocorticoid sensitivity and easy recurrence, and a pathological biopsy is the key to its diagnosis. Long-term glucocorticoid maintenance and individualized dose reduction regimens may reduce the risk of recurrence, but the optimal treatment plan still needs to be explored in further clinical studies. The present case can provide a practical reference for the diagnosis and treatment of multiple recurrent COP.

Organizing pneumonia is a condition first described in 1941. Its symptoms are nonspecific (fever, arthromyalgia, etc.), and its diagnosis relies primarily on histology, when this is not feasible, it is based on a combination of clinical, laboratory, and radiological findings, in the absence of a differential diagnosis particularly active infections. It is a rare condition that may be secondary to another condition or primary and referred to as cryptogenic. Numerous causes of organized pneumonia have been described: post-infectious, associated with connective tissue diseases, drug-induced, etc. It affects all age groups, with the idiopathic form predominantly occurring between the ages of 50 and 60. A non-contrast chest CT scan may be suggestive but remains nonspecific and must rule out infection. Bronchoalveolar lavage can help rule out active infectious causes and provide diagnostic clues; however, the gold standard remains histological examination, which reveals endoalveolar buds, an inflammatory infiltrate of the alveolar walls, and thickening of the alveolar walls. Treatment is usually based on corticosteroid therapy for the cryptogenic form but must be tailored to the underlying condition when the disease is secondary. The prognosis is generally favorable in the cryptogenic form and depends on the cause in the secondary form.

Cerliponase alfa enzyme replacement therapy has transformed the management of neuronal ceroid lipofuscinosis type 2 (CLN2) disease but requires lifelong cerebrospinal fluid (CSF) access for repeated intraventricular infusions. Conventional scalp-based ventricular reservoirs are associated with complications including infection, mechanical deterioration, and device revision related to repeated puncture. Chest port-mediated ventricular access has emerged as a potential alternative; however, detailed operative descriptions remain limited. We describe operative considerations and stepwise technique for establishing ventricular access using a Rickham reservoir connected via shunt tubing to a subcutaneous chest port system for repeated intraventricular cerliponase alfa infusion. Illustrative clinical experience demonstrates durable long-term use of this configuration. Key technical elements include neuronavigation-guided ventricular catheter placement, creation of a subcutaneous or subfascial chest pocket depending on patient body habitus, incorporation of strain-relief loops to reduce catheter tension, and systematic testing of the completed construct. The resulting system allows reliable ventricular access via a chest port while preserving ventricular catheter integrity. Chest port-mediated ventricular access represents a feasible and durable strategy for long-term intraventricular therapy in CLN2 disease. Dissemination of operative technique may facilitate broader adoption and support reliable delivery of enzyme replacement therapy for patients requiring lifelong treatment.

Collagen vascular disease encompasses a diverse group of immunologically mediated entities that share overlapping clinical and histopathologic features as well as manifest in characteristic patterns of interstitial lung disease. The collagen vascular diseases which commonly affect the pulmonary system include rheumatoid arthritis (RA), systemic sclerosis (SSc), systemic lupus erythematosus (SLE), polymyositis (PM), dermatomyositis (DM), mixed connective tissue disease (MCTD), and Sjogren’s syndrome (SS). The degree of lung involvement and spectrum of thoracic findings vary among the disorders, may be seen with clinically early or late disease, and portends certain patient outcomes. Identification of pulmonary involvement has important therapeutic and prognostic implications. Collagen vascular disease may be associated with usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia (COP), diffuse alveolar damage (DAD), and lymphocytic interstitial pneumonia (LIP).[1]

This study evaluates the incidence and clinical impact of late- onset non-infectious pulmonary complications (LONIPCs) in adults undergoing allo-HCT with post-transplant cyclophosphamide (PTCy). In addition, it assesses the prognostic value of pre-transplant pulmonary function tests (PFTs) for overall mortality in this population. A total of 317 consecutive adults transplanted between 2014 and 2024 were retrospectively included. LONIPC occurred in 10.6% of patients. The most frequent diagnoses were cryptogenic organizing pneumonia (COP, 4.1%), bronchiolitis obliterans syndrome (BOS, 5.9%), and pulmonary thromboembolism (0.6%). Risk factors for LONIPC included smoking history, pre-existing bronchial asthma, mismatched donors, and grade III-IV acute GVHD. The diagnosis of LONIPC was strongly associated with adverse outcomes in both univariate (OS: HR 9.37, P < 0.001; NRM: HR 4.19, P = 0.001) and multivariate analyses (OS: HR 8.07, P < 0.001; NRM: HR 3.19, P = 0.033). Based on pre-transplant PFTs and the HCT-CI scoring system, patients were classified into low- (24.3%), moderate- (35.0%), or high-risk groups (40.7%). High-risk patients had significantly poorer overall survival (OS) (3-year OS: 58.8% vs. 72.0% and 70.0%, P = 0.027) and higher non-relapse mortality (NRM) (3-year NRM: 19.0% vs. 7.9% and 15.1%) compared with those in the low- and intermediate-risk groups. No differences were observed regarding pre-transplant PFTs between patients developing LONIPC and those who did not. In conclusion, the incidence of LONIPC occurs in 10% of adults undergoing allo-HCT with PTCy. Its diagnosis, as well as the presence of significant pre-transplant PFT abnormalities, were associated with worse outcomes, underscoring their prognostic importance in the contemporary PTCy-based allo-HCT setting.

Air-leak syndrome (ALS), including pneumothorax, pneumomediastinum, and subcutaneous emphysema, is a rare but life-threatening complication after allogeneic hematopoietic stem cell transplantation (HSCT). However, no standard treatment has yet been established. Here, we report the case of a 60-year-old man with acute myeloid leukemia who underwent allogeneic HSCT. He developed chronic graft-versus-host disease (GVHD) and cryptogenic organizing pneumonia (COP) on day 126 after transplantation. Although initial corticosteroid therapy improved the COP, the relapse occurred during tapering, and repeated steroid treatment failed to achieve sustained disease control. The patient subsequently developed pneumomediastinum, consistent with ALS. Ruxolitinib treatment was initiated on day 265 for steroid-dependent chronic GVHD and organizing pneumonia. Following treatment, oxygenation improved, pneumomediastinum improved, and corticosteroids were successfully tapered and discontinued without COP recurrence. Serum Krebs von den Lungen-6 (KL-6) levels decreased in parallel with clinical and radiological improvement. The introduction of ruxolitinib is associated with improvements in ALS and enabled the discontinuation of steroids. Adverse events included cytopenia and mild liver dysfunction, consistent with known safety profiles. The patient died of acute respiratory failure after transfusion, which was not considered a direct complication of the ruxolitinib treatment. This case suggests that ruxolitinib may facilitate corticosteroid tapering and radiographic improvement of organizing pneumonia-associated ALS after allogeneic transplantation.

Transcranial magnetic stimulation (TMS) is increasingly used for language mapping in epilepsy surgery. However, due to small sample sizes and inconsistent definitions of overlap, when compared to direct cortical stimulation (DCS), TMS has shown variable sensitivity and specificity. Furthermore, performance of TMS in pediatric cohort is not well understood. This study aimed to standardize the TMS-DCS overlap criteria using a Euclidean distance method and then document the efficacy of TMS in pediatric and adult patients. Using this approach, we evaluated the statistical performance of TMS language localization in eight children (mean age 15) and seven adults (mean age 28) with refractory epilepsy all of whom underwent extra-operative DCS mapping. Overall, TMS showed 89.6% specificity, 81.5% negative predictive value, and 75% accuracy, with high false negatives and positives, resulting in 12.7% sensitivity and 22% positive predictive value (PPV). In subgroup analyses, children showed significantly higher sensitivity (16.3% vs. 5.3%; p = 0.026) and numerically higher PPV (28.9% vs. 8.7%) than adults. The results of this study add to prior evidence TMS may be most useful for predicting language-negative regions for surgery. In children, TMS can still provide useful adjunctive information where other language mapping modalities are less effective. The proposed validation method can be readily adopted across centers to optimize TMS parameters, aimed at improving its sensitivity and overall accuracy.