Pulmonary manifestation of VEXAS syndrome.

This case report presents the diagnostic journey of a man in his mid-70s who experienced shortness of breath, cough, recurrent episodes of fever, weight loss, pruritic erythroderma, uveitis and macrocytic anaemia. The initial diagnosis of cryptogenic organising pneumonia was made based on antibiotic refractory infiltrates seen in the lung CT scan. The patient initially responded favourably to immunosuppression but experienced a recurrence of symptoms when the corticosteroid dose was tapered. Despite ongoing systemic inflammation and refractory symptoms, it took nearly a year to establish the diagnosis of VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory and somatic) syndrome. This case highlights the challenges in diagnosing and managing VEXAS syndrome due to its recent discovery and limited awareness in the medical community, as well as the need to consider this syndrome as a rare differential diagnosis of therapy-refractory pulmonary infiltrates.

[Clinical analysis of 4 children with cryptogenic organizing pneumonia].

Objective: To analyze the clinical features of children with cryptogenic organizing pneumonia (COP) confirmed by pathology. Methods: The clinical manifestations, imaging, pathology, treatment and outcome data of 4 children with COP confirmed by thoracoscopic lung biopsy were retrospectively analyzed, who were hospitalized at Respiratory Department of Shenzhen Children's Hospital from January 2004 to December 2022. Results: All of the 4 patients were male, the age ranged from 1 year 3 months to 14 years. The time from onset to diagnosis was 3 months to 3 years. The follow-up duration was 6 months to 6 years. All the 4 cases had cough, 2 cases had tachypnea and wheezing, 1 case had expectoration, 1 case had chest pain, 1 case had decreased activity tolerance and weight loss. Rales in 2 cases and hypoxemia in 1 case. Pulmonary high resolution CT showed diffuse distribution, involvement of both lungs in 3 cases, and single lung combined migration in 1 case.Three cases showed ground-glass opacity, consolidation, patchy or fibrous strips, and 1 case presented air bronchogram and "reversed halo sign". All the 4 cases were performed thoracoscopic lung biopsy, and the pathological findings showed cellulose exudate or small nodules filled with granulation tissue or fibroblasts in the alveolar cavity and small airways, and 1 case was Masson corpuscle positive. Three patients achieved remission after glucocorticoid therapy. Spontaneous remission without treatment was seen in 1 patient.Two cases were followed up for 17 months and 6 years, respectively, who had excellent outcome. Conclusions: The manifestations of COP in children include cough, expectoration and chest pain. Infants and young children may have tachypnea and wheezing. The most common chest CT findings are diffuse distribution of ground-glass opacity, patchy and consolidation in both lungs. Diagnosis should depend on pathological examination. The effect of glucocorticoid therapy is good.

Cryptogenic organizing pneumonia after withdrawal of systemic corticosteroids for chronic eosinophilic pneumonia and severe asthma under benralizumab treatment.

A 79-year-old woman with severe asthma developed chronic eosinophilic pneumonia (CEP). After CEP resolved with oral prednisolone at 30 mg/day, prednisolone was tapered and discontinued under introduction of benralizumab for her severe asthma. However, 8 weeks later, symptoms and bilateral patchy infiltrates on chest radiography appeared. Lymphocytosis without eosinophilia was seen in bronchoalveolar lavage fluids, and transbronchial biopsy indicated organizing pneumonia. Cryptogenic organizing pneumonia (COP) was diagnosed and resolved with prednisolone at 30 mg/day. Prednisolone was tapered to 3 mg/day without relapse of CEP or COP. This case suggests the overlap and similar pathogenesis of CEP and COP.

A case report: Acute fibrinous and organizing pneumonia.

RATIONALE: Acute fibrinous and organizing pneumonia (AFOP) is a rare acute or subacute interstitial lung disorder characterized by the deposition of fibrin within the alveoli and organizing pneumonia with a patchy distribution. The clinical features of AFOP are nonspecific, and it is often misdiagnosed as pneumonia, cancer, tuberculosis, or other lung disorders. PATIENT CONCERNS: In this case report, a 58-year-old woman presented with chest tightness, shortness of breath, cough and sputum. A chest CT scan showed multiple patchy shadows in both lungs. She was initially diagnosed with community-acquired pneumonia. Her purified protein derivative skin test was positive, but sputum was negative for acid-fast bacilli. DIAGNOSES: AFOP was diagnosed by bronchoscopic lung biopsy and histopathology. INTERVENTIONS: Following AFOP diagnosis, all anti-infective drugs were discontinued, and replaced by methylprednisolone and prednisone. OUTCOMES: After 1 week of treatment with methylprednisolone 40 mg daily, the patient chest CT and clinical symptoms improved. After 1 month, the patient symptoms had demonstrated dramatic improvement and CT scan revealed complete absorption of lesions in both lungs. After 5 months of follow-up, the patient symptoms completely disappeared. LESSONS: Acute AFOP is an uncommon lung condition with poor prognosis; hence, early diagnosis and identification are particularly important. Definitive diagnosis requires histopathological findings. Currently, there is no unified treatment guideline for AFOP, and treatment must be tailored based on the etiology and severity of each individual patient disease. Subacute AFOP shows a good response to corticosteroid treatment.

Minimal effective dose of maintenance steroid therapy for relapse of cryptogenic organizing pneumonia.

BACKGROUND: Long-term maintenance steroid therapy (MST) is frequently required for repeated relapses of cryptogenic organizing pneumonia (COP); however, the optimal minimal dose has not been clarified. Therefore, this study evaluated the minimal MST dose required to prevent repeated relapses and identify relapse predictors. METHODS: We retrospectively reviewed the medical records of patients with steroid-treated COP and compared background factors between the non-relapse and relapse groups. We also reviewed the treatment course in the relapse group and determined the minimal effective steroid dose based on the MST dose at relapse events and the current relapse prevention dose. RESULTS: In total, 48 patients were identified, including 27 (56%) in the non-relapse group and 21 (44%) in the relapse group. Receiver operating characteristic curve analysis identified prednisolone at 5 mg/day as the optimal cut-off value in the relapse group. Relapse-free time in patients with relapsed COP was significantly longer in the MST dose ≥5 mg/day group than in the <5 mg/day group (log-rank P = 0.003; hazard ratio, 0.19; 95% confidence interval [CI], 0.04-0.60). Multivariate logistic regression analysis demonstrated that a high eosinophil percentage and CD4/CD8 ratio in bronchoalveolar lavage fluid (BALF) were predictors of relapse (odds ratio [OR], 1.12; 95% CI, 1.02-1.23; P = 0.008 and OR, 3.87; 95% CI, 1.29-11.6; P = 0.008, respectively). CONCLUSIONS: Our results indicate that 5 mg/day of prednisolone may be the minimal effective dose for preventing repeated relapses, and a high BALF eosinophil percentage and CD4/CD8 ratio are independent predictors of relapse.

Spontaneous resolution of cryptogenic organizing pneumonia: Observational study.

Cryptogenic organizing pneumonia (COP) is an idiopathic interstitial pneumonia generally requiring steroid therapy, and spontaneous resolution has been reported in patients with mild disease. However, evidence supporting the need for COP treatment is poor. Therefore, we investigated the characteristics of patients with spontaneous resolution. We retrospectively collected data from 40 adult patients who were diagnosed with COP through bronchoscopic examination at Fukujuji Hospital from May 2016 to June 2022. Sixteen patients who improved without steroid therapy (the spontaneous resolution group) and 24 patients who required steroid therapy (the steroid therapy group) were compared. Patients in the spontaneous resolution group showed a lower C-reactive protein (CRP) concentration (median 0.93 mg/dL [interquartile range [IQR] 0.46-1.91] vs median 10.42 mg/dL [4.82-16.7], P < .001), a higher lymphocyte ratio (median 21.7% [18.2-25.2] vs median 13.3% [8.8-19.8], P = .002), and a longer duration from symptom onset to diagnosis of COP (median 51.5 days [24.5-65.3] vs 23.0 days [17.3-31.8], P = .009) than those in the steroid therapy group. Within 2 weeks, all patients in the spontaneous resolution group showed relief of symptoms and alleviated radiographic findings. The area under the receiver operating characteristic (ROC) curve was 0.859 (95% confidence interval [CI]: 0.741-0.978) in CRP. When we arbitrarily determined the cutoff values, including CRP levels of ≤3.79 mg/dL, the sensitivity, specificity, and odds ratio were 73.9%, 93.8%, and 39.8 (95% confidence interval: 4.51-1968.9), respectively. Only 1 patient in the spontaneous resolution group showed recurrence but did not require steroid therapy. Conversely, 4 patients in the steroid therapy group showed recurrence and were treated by an additional course of steroids. The characteristics of COP with spontaneous resolution and factors that determine the patients in whom steroid therapy might be avoided is detailed in this study.

Resolution of an insidious and migratory Mycobacterium tuberculosis-associated secondary organizing pneumonia: a case report and literature review.

BACKGROUND: Organizing pneumonia (OP) is a rare interstitial lung disease. Secondary organizing pneumonia (SOP) caused by Mycobacterium tuberculosis (MTB) is extremely rare. Migratory MTB-associated SOP is more deceptive and dangerous. When insidious tuberculosis (TB) is not recognized, SOP would be misdiagnosed as cryptogenic organizing pneumonia (COP). Use of steroid hormone alone leads to the progression of TB foci or even death. Clues of distinguishing atypical TB at the background of OP is urgently needed. CASE PRESENTATION: A 56-year-old female patient was hospitalized into the local hospital because of cough and expectoration for more than half a month. Her medical history and family history showed no relation to TB or other lung diseases. Community-acquired pneumonia was diagnosed and anti-infection therapy was initialized but invalid. The patient suffered from continuous weigh loss. More puzzling, the lesions were migratory based on the chest computed tomography (CT) images. The patient was then transferred to our hospital. The immunological indexes of infection in blood and pathogenic tests in sputum and the bronchoalveolar lavage fluid were negative. The percutaneous lung puncture biopsy and pathological observation confirmed OP, but without granulomatous lesions. Additionally, pathogen detection of the punctured lung tissues by metagenomics next generation sequencing test (mNGS) were all negative. COP was highly suspected. Fortunately, the targeted next-generation sequencing (tNGS) detected MTB in the punctured lung tissues and MTB-associated SOP was definitely diagnosed. The combined therapy of anti-TB and prednisone was administrated. After treatment for 10 days, the partial lesions were significantly resorbed and the patient was discharged. In the follow-up of half a year, the patient was healthy. CONCLUSIONS: It is difficult to distinguish SOP from COP in clinical practice. Diagnosis of COP must be very cautious. Transient small nodules and cavities in the early lung image are a clue to consider TB, even though all pathogen tests are negative. tNGS is also a powerful tool to detect pathogen, ensuring prompt diagnosis of TB-related SOP. For clinicians in TB high burden countries, we encourage them to keep TB in mind before making a final diagnosis of COP.

Pulmonary tuberculosis associated acute fibrinous and organizing pneumonia: A case report and literature review.

BACKGROUND: Acute fibrinous and organizing pneumonia (AFOP) is a rare histological interstitial pneumonia pattern characterized by patches of "fibrin balls" distributed within the alveoli and organizing pneumonia. Currently, there is no consensus on the diagnosis and treatment of this disease. METHODS: We present the case of a 44-year-old male with AFOP secondary to Mycobacterium tuberculosis. We have further reviewed organizing pneumonia (OP) and AFOP caused by tuberculosis. CONCLUSION: Tuberculosis secondary to OP or AFOP is rare and challenging to diagnose. We need to constantly adjust the treatment plan based on the patient's symptoms, test results, and response to treatment in order to arrive at an accurate diagnosis and maximize treatment efficacy.

Flecainide-induced pneumonitis: a case report.

BACKGROUND: We report a case of acute respiratory distress associated with a histological pattern of acute fibrinous and organizing pneumonia, and discuss the possible responsibility of flecainide therapy. CASE PRESENTATION: A 61-year-old African woman developed a rapidly progressive dyspnea and required admission in the intensive care unit for orotracheal intubation and mechanical ventilation. Chest X-ray examination revealed bilateral infiltrates predominating in the basal part of both lungs. Lung computed tomography disclosed bilateral ground-glass opacities and septal thickening. After exclusion of the most common causes of infectious or immune pneumonia, a toxic origin was investigated and flecainide toxicity was considered. Lung biopsy was consistent with the unusual pattern of acute fibrinous and organizing pneumonia. Clinical and radiological improvement was noted after corticosteroid therapy, but the patient died from septic complications. CONCLUSION: Flecainide-induced lung injury has rarely been reported in the literature and remains a diagnosis of exclusion. The histological pattern of acute fibrinous and organizing pneumonia has been previously observed with amiodarone. There are no firm guidelines for the treatment of acute fibrinous and organizing pneumonia, but some patients may positively respond to corticosteroids.

Scoping review: The state of research on cryptogenic organizing pneumonia therapeutics.

Cryptogenic organizing pneumonia is a diffuse interstitial lung disease that starts in the alveolar wall and subsequently expands to the alveolar ducts and respiratory bronchioles. Randomized controlled trials are lacking to guide the treatment of cryptogenic organizing pneumonia, so treatment decisions and practice guidelines are often based upon observations from case series or expert clinical opinions. The backbone of treatment involves immunosuppression via corticosteroids. In refractory cases, cytotoxic therapy is considered. The evidence that supports the use of these regimens are limited. The goal of this scoping review is to conduct a systematic search of the literature to determine what regimens have been utilized to treat steroid refractory organizing pneumonia and to characterize the evidence supporting their use.

Case of organising pneumonia in HIV infection.

A man in his 50s presented to his doctor with a fever, sore throat, cough, dysgeusia and dyspnoea of several days' duration. Tests for HIV antigen, HIV antibody and HIV PCR were positive. He was referred to our hospital for initiation of antiretroviral therapy and bronchoscopy to clarify the cause of an abnormal lung shadow on chest CT. He was diagnosed with organising pneumonia, with concurrent HIV infection. His pulmonary lesions were remitted spontaneously, and he was administered a fixed-dose combination of tenofovir (50 mg), emtricitabine (200 mg) and bictegravir (25 mg) for HIV. This is a rare report of organising pneumonia with HIV infection. Physicians need to consider organising pneumonia when lung opacity is observed in a patient with HIV infection.

Organizing Pneumonia: A Clinical Challenge in a Child With Previous Rhabdomyosarcoma.

Organizing pneumonia is a pulmonary disease of undefined etiology, with few reported cases in children. It may be secondary to chemotherapy, radiation therapy, infectious agents, or hematopoietic cell transplantation. We present a case of an 18-year-old boy who presented to a follow-up consult with respiratory symptoms at the age of 11 years, 8 years after finishing treatment for a prostatic relapse of a pelvic rhabdomyosarcoma. Chest radiography revealed nodular opacities in the left lung, the one in the left lower lobe with silhouette sign with the left hemidiaphragm. Chest computerized tomography showed 2 nodular lesions in the left upper lobe, one of them cavitated, and another nodular lesion in the left lower lobe; 2 of these nodules had surrounding ground-glass opacities. Microbiological work-up, including tuberculosis screening, was negative. Biopsy revealed findings suggestive of organizing pneumonia. He presented spontaneous resolution. This case presented a diagnostic challenge due to rarity of this condition and its indetermined association with the patient's history of rhabdomyosarcoma. With this case, the authors alert that organizing pneumonia must be considered in patients presenting with pulmonary lesions with a history of previous hematopoietic stem cell transplants, lung irradiation, or immunosuppression. Pulmonary metastases and secondary tumors must be considered as a differential diagnosis in patients with a heavily treated relapsed rhabdomyosarcoma.

Effectiveness of Steroid Treatment for SARS-CoV-2 Pneumonia With Cryptogenic Organizing Pneumonia-Like Reaction: A Case Report.

Several studies on the treatment of coronavirus disease 2019 (COVID-19) are being conducted, and various drugs are being tried; however, the results have not been uniform. Steroids have been widely used in the treatment of COVID-19, but their effects are controversial. As immunosuppressive and anti-inflammatory agents, steroids are considered to reduce lung damage by regulating various inflammatory responses. We report a case of severe acute respiratory syndrome coronavirus-2 pneumonia manifesting as a cryptogenic organizing pneumonia-like reaction and discuss its treatment, clinical course, and favorable outcomes after steroid administration.

Secondary organizing pneumonia after recovery of mild COVID-19 infection.

A 36-year-old male with diffuse large B-cell lymphoma on maintenance rituximab therapy presented to the emergency department with high fever and fatigue. A chest X-ray showed a lobar infiltrate, 40 days before admission the patient suffered from a mild coronavirus disease 2019 (COVID-19) infection and fully recovered. PCR nasopharyngeal swab was negative for COVID-19. Comprehensive biochemical, radiological, and pathological evaluation including 18-fluorodeoxyglucose positron emission tomography with computed tomography and transbronchial lung biopsy found no pathogen or lymphoma recurrence. Treatment for pneumonia with antibiotic and antifungal agents was nonbeneficial. A diagnosis of secondary organizing pneumonia (OP) was made after pneumonia migration and a rapid response to corticosteroids. OP secondary to a viral respiratory infection has been well described. Raising awareness for post-COVID-19 OP has therapeutic and prognostic importance because those patients benefit from steroid therapy. We believe the condition described here is underdiagnosed and undertreated by doctors worldwide. Because of the ongoing global pandemic we are now encountering a new kind of patient, patients that have recovered from COVID-19. We hope that this case may contribute to gaining more knowledge about this growing patient population.

Acute fibrinous and organizing pfneumonia: two case reports and literature review.

BACKGROUND: Acute fibrinous and organizing pneumonia (AFOP) is a rare histologic interstitial pneumonia pattern characterized by the intra-alveolar fibrin deposition and organizing pneumonia. Its clinical characteristics are still not well known and there is no consensus on treatment yet. CASE PRESENTATION: We report two female cases in their fifties diagnosed with AFOP confirmed by a second lung biopsy. Case 1 was idiopathic AFOP with manifestation of 6-week fever, dyspnea, and cough, while case 2 was secondary to systemic lupus erythematosus and fever was the major symptom. Their chest CT scans revealed bilateral multiple consolidations, predominantly in the lower lobes. Both cases were initially diagnosed with pneumonia, but did not improve after treatment with broad-spectrum antibiotics. In both cases, transbronchial biopsy and bronchoalveolar lavage fluid examination were inconclusive and the pathological diagnosis was confirmed by percutaneous lung biopsy. Both patients had a good clinical response to prednisone. CONCLUSIONS: We report two rare AFOP cases to highlight the importance of awareness of this disease. We further perform the most comprehensive review to date in AFOP, including 150 patients since 2002. Consolidation was the most common imaging pattern, followed by ground-glass opacity and nodules. A lung biopsy is required for a definitive diagnosis. Corticosteroids is recommended as the most effective therapy, but treatment options should depend on the etiology and disease severity.

Organising pneumonia manifesting as a late-phase complication of COVID-19.

Acute COVID-19 usually lasts 4 weeks from the onset of symptoms. We report two cases of COVID-19-associated organising pneumonia (OP) occurring beyond 4 weeks from the acute onset of symptoms. Both tested positive for SARS reverse transcription-PCR 2 months before presentation with a resolution of respiratory symptoms. The first case presented with residual fatigue and worsening exertional dyspnoea. Chest CT revealed an OP pattern. The second case presented with worsening cough and new-onset pleuritic chest pain with persistent radiological consolidation. A transbronchial lung biopsy confirmed OP. Both patients responded well to 12 weeks of steroid therapy. This case illustrates the rare presentation of OP as a late sequela of COVID-19 and the good response to steroid therapy.

Clinical features of acute fibrinous and organizing pneumonia: An early histologic pattern of various acute inflammatory lung diseases.

BACKGROUND: Acute fibrinous and organizing pneumonia (AFOP) is a rare histologic pattern of acute lung involvement with intra-alveolar fibrin deposition. However, the clinical significance of the pathological findings of AFOP remains unclear. This study aimed to explore the clinical significance of AFOP through a comprehensive clinical examination. METHODS: The medical records of patients with lung diseases accompanied by the pathological finding of intra-alveolar organization between January 2010 and December 2019 were retrospectively reviewed. The clinical and radiological findings were compared between the groups with and without the histologic pattern of AFOP. RESULTS: We identified 34 patients with AFOP (AFOP group) and 143 without AFOP (non-AFOP group). The underlying diseases of the AFOP group were as follows: 19 patients had cryptogenic organizing pneumonia (OP), 5 had connective tissue diseases, 3 had radiation pneumonitis, 3 had chronic eosinophilic pneumonia, 2 had myelodysplastic syndromes, and 2 had drug-induced pneumonia. Fever was more common, the time from symptom onset to biopsy was shorter, and the serum C-reactive protein level was higher in the AFOP group than in the non-AFOP group. On high-resolution computed tomography, 85% of patients had OP pattern, and halo sign was more common in the AFOP group. Corticosteroids were effective in 94% of the patients in the AFOP group; however, recurrences were more frequent, and a higher corticosteroid dose was needed during recurrence. CONCLUSIONS: AFOP might be an early phase of a histologic pattern associated with known etiologies. In addition, it could be a marker indicating intense inflammatory diseases with a tendency of recurrence.