Lung cancer in older patients with granulomatosis with polyangiitis: a report of three cases.

BACKGROUND: Granulomatosis with polyangiitis (GPA) is characterized by necrotizing granulomatous inflammation with necrotizing vasculitis predominantly affecting small to medium vessels. The survival rates have drastically improved; however, GPA can be lethal, with older patients having a worse prognosis and higher mortality than younger patients. Moreover, the incidence of various cancers has been reported to increase in patients with GPA. We aimed to discuss possible associations between GPA and lung cancer and emphasize the associated diagnostic challenges. CASE PRESENTATION: We encountered three older patients with chronic GPA who developed lung cancer during long-term follow-up. Two of the patients had a smoking history, with one having silicosis and the other having chronic obstructive pulmonary disease. Furthermore, all of them had radiation exposure from repeated radiography/computed tomography. All the patients had confirmed GPA, and vasculitis relapse was first suspected when new lung lesions were noted during follow-up. However, they had no new clinical symptoms, and serum ANCA titer increased only in one patient. All the patients received standard immunosuppressive treatment but eventually died. CONCLUSIONS: Lung cancer is uncommon in patients with GPA; however, the similarity between the imaging findings of lung cancer and GPA may pose a diagnostic challenge. Clinicians should be particularly vigilant when treating older patients with an increased risk of cancer, as they are often asymptomatic or have poorly apparent clinical features.

Backlogs in formal interpretation of radiology examinations: a pilot global survey.

OBJECTIVE: Anecdotal reports from imaging facilities globally suggest growing radiology interpretation reporting delays. This pilot study's primary aim was to estimate the backlog of formal interpretation of imaging examinations. METHODS: An online survey was distributed to radiologists globally to gather practice-specific characteristics, imaging volumes, and reporting for 3 types of examinations (brain/head CT scans, chest CT scans, and chest radiographs) at 4 time points: 7, 30, 90 days, and 6 months. RESULTS: We received responses from 49 radiologists in 16 countries on six continents. Unreported examinations (backlog) were present in thirty of 44 (68%) facilities. Backlogs for brain/head CT, chest CT, and chest radiographs were present in, respectively, 48%, 50%, and 59% of facilities at 7 days and 20%, 23%, and 32% of facilities at 6 months. When present, the mean proportion of backlog (range) at 7 days was 17% (1 to 96) for brain/head CT, 18% (3 to 82) for chest CT, and 22% (1 to 99) for chest radiographs. CONCLUSIONS: Our findings from this pilot study show a widespread global backlog in reporting common imaging examinations, and further research is needed on the issue and contributing factors.

Molecular Identification of Extrapulmonary Vaccine Adverse Events after BCG in Paraffin-Embedded Specimens.

According to the World Health Organization (WHO), around 1 million children worldwide are diagnosed with tuberculosis each year. The Bacillus Calmette-Guérin (BCG) vaccine has been used around the world for over 100 years. The complications of the BCG vaccination can occur in about 0,06% of children and include local or systemic adverse reactions. Due to the close analogy between the vaccine strain and other species of the Mycobacterium tuberculosis complex (MTBC), molecular methods are recommended for differential diagnosis of Vaccine adverse events (VAE) after BCG. The ability to quickly and specifically identify BCG is important in view of different treatment regimens. The aim of the study was to assess the usefulness of genetic testing for Mycobacterium bovis BCG in the paraffin-embedded specimens' methods. We describe two cases of VAE in immune-compromised children presenting with osteoarticular changes that had been clinically suspected of tuberculosis and led to molecular identification through GeneXpert, GenoType MTBC, and Spoligotyping. Results: Mycobacterium bovis BCG was detected in osteoarticular changes embedded in paraffin block of two patients. Conclusion: Genetic tests using paraffin-embedded materials allow for quick identification and differential diagnosis of patients with Tuberculosis and VAE after BCG. This is an important issue, especially in cases where the tissue has only been submitted for histopathological examination without microbiological diagnostics for tuberculosis.

Sudden Vision Loss Due to Optic Neuritis-An Uncommon Presentation of Neurosarcoidosis.

Sarcoidosis is a systemic, granulomatous disease of unknown etiology, most often manifested by mediastinal and hilar lymph node enlargement and parenchymal nodules in the lungs. However, it may involve any other organ. Neuro-sarcoidosis, a condition that affects up to 20% of sarcoidosis patients, can be found in any part of the central or peripheral nervous system and has important ophthalmic and neuro-ophthalmic manifestations. We present two patients with sudden vision loss due to neurosarcoidosis. In both cases, biopsy of the mediastinal lymph node showed non-caseating granulomas consistent with sarcoidosis. Treatment involved high doses of methylprednisolone intravenously, followed by topical dexamethasone eye drops in the first case and a systemic steroid treatment in the second, resulting in symptom relief. Those two cases demonstrate that sarcoidosis should be considered as a differential diagnosis in cases of optic neuritis.

Imaging Plays a Key Role in the Diagnosis and Control of the Treatment of Bone Sarcoidosis.

Sarcoidosis is a multisystem granulomatous disease of unknown origin. The most frequent localizations are thoracic lymph nodes and/or parenchymal lung disease, nevertheless any other organ may be involved. Musculoskeletal sarcoidosis, previously considered a rare manifestation of the disease, is presently recognized with increasing frequency, due to the development of modern imaging modalities. The classical X-ray sign of bone sarcoidosis is the image of lace in the phalanges of the hands. Most other locations present with atypical radiological images. Therefore, they may mimic metastatic neoplastic disease, especially when they are the first sign of sarcoidosis not previously recognized. On such occasions, none of the imaging methods will give the correct diagnosis, histopathological verification, monitoring of lesions or clinical data in a patient with confirmed sarcoidosis are indicated. The article summarizes the current status of knowledge concerning the recognition and therapy of bone sarcoidosis. In addition, an illustrative case of patient with bone and bone marrow sarcoidosis is presented.

The Presence of T Allele (rs35705950) of the MUC5B Gene Predicts Lower Baseline Forced Vital Capacity and Its Subsequent Decline in Patients with Hypersensitivity Pneumonitis.

Hypersensitivity pneumonitis (HP) is an exposure-related interstitial lung disease with two phenotypes-fibrotic and non-fibrotic. Genetic predisposition is an important factor in the disease pathogenesis and fibrosis development. Several genes are supposed to be associated with the fibrosing cascade in the lungs. One of the best-recognized and most prevalent is the common MUC5B gene promoter region polymorphism variant rs35705950. The aim of our study was to establish the frequency of the minor allele of the MUC5B gene in the population of patients with HP and to find the relationship between the MUC5B promoter region polymorphism and the development of lung fibrosis, the severity of the disease course, and the response to the treatment in patients with HP. Eighty-six consecutive patients with HP were tested for the genetic variant rs35705950 of the MUC-5B gene. Demographic, radiological, and functional parameters were collected. The relationship between the presence of the T allele and lung fibrosis, pulmonary function test parameters, and the treatment response were analyzed. The minor allele frequency in the study group was 17%, with the distribution of the genotypes GG in 69.8% of subjects and GT/TT in 30.2%. Patients with the GT/TT phenotype had significantly lower baseline forced vital capacity (FVC) and significantly more frequently had a decline in FVC with time. The prevalence of lung fibrosis in high-resolution computed tomography (HRCT) was not significantly increased in GT/TT variant carriers compared to GG ones. The patients with the T allele tended to respond worse to immunomodulatory treatment and more frequently received antifibrotic drugs. In conclusions: The frequency of MUC5B polymorphism in HP patients is high. The T allele may indicate a worse disease course, worse immunomodulatory treatment response, and earlier need for antifibrotic treatment.

Guidelines of the Polish Respiratory Society on the Diagnosis and Treatment of Progressive Fibrosing Interstitial Lung Diseases Other than Idiopathic Pulmonary Fibrosis.

The recommendations were developed as answers to previously formulated questions concerning everyday diagnostic and therapeutic challenges. They were developed based on a review of the current literature using the GRADE methodology. The experts suggest that PF-ILD be diagnosed based on a combination of different criteria, such as the aggravation of symptoms, progression of radiological lesions, and worsening of lung function test parameters. The experts recommend a precise diagnosis of an underlying disease, with serological testing for an autoimmune disease always being included. The final diagnosis should be worked out by a multidisciplinary team (MDT). Patients with an interstitial lung disease other than IPF who do not meet the criteria for the progressive fibrosis phenotype should be monitored for progression, and those with systemic autoimmune diseases should be regularly monitored for signs of interstitial lung disease. In managing patients with interstitial lung disease associated with autoimmune diseases, an opinion of an MDT should be considered. Nintedanib rather than pirfenidon should be introduced in the event of the ineffectiveness of the therapy recommended for the treatment of the underlying disease, but in some instances, it is possible to start antifibrotic treatment without earlier immunomodulatory therapy. It is also admissible to use immunomodulatory and antifibrotic drugs simultaneously. No recommendations were made for or against termination of anti-fibrotic therapy in the case of noted progression during treatment of a PF-ILD other than IPF. The experts recommend that the same principles of non-pharmacological and palliative treatment and eligibility for lung transplantation should be applied to patients with an interstitial lung disease other than IPF with progressive fibrosis as in patients with IPF.

Guidelines of the Polish Respiratory Society on the Diagnosis and Treatment of Progressive Fibrosing Interstitial Lung Diseases Other than Idiopathic Pulmonary Fibrosis

The recommendations were developed as answers to previously formulated questions concerning everyday diagnostic and therapeutic challenges. They were developed based on a review of the current literature using the GRADE methodology. The experts suggest that PF-ILD be diagnosed based on a combination of different criteria, such as the aggravation of symptoms, progression of radiological lesions, and worsening of lung function test parameters. The experts recommend a precise diagnosis of an underlying disease, with serological testing for an autoimmune disease always being included. The final diagnosis should be worked out by a multidisciplinary team (MDT). Patients with an interstitial lung disease other than IPF who do not meet the criteria for the progressive fibrosis phenotype should be monitored for progression, and those with systemic autoimmune diseases should be regularly monitored for signs of interstitial lung disease. In managing patients with interstitial lung disease associated with autoimmune diseases, an opinion of an MDT should be considered. Nintedanib rather than pirfenidon should be introduced in the event of the ineffectiveness of the therapy recommended for the treatment of the underlying disease, but in some instances, it is possible to start antifibrotic treatment without earlier immunomodulatory therapy. It is also admissible to use immunomodulatory and antifibrotic drugs simultaneously. No recommendations were made for or against termination of anti-fibrotic therapy in the case of noted progression during treatment of a PF-ILD other than IPF. The experts recommend that the same principles of non-pharmacological and palliative treatment and eligibility for lung transplantation should be applied to patients with an interstitial lung disease other than IPF with progressive fibrosis as in patients with IPF.

Large Pericardial Effusion-Diagnostic and Therapeutic Options, with a Special Attention to the Role of Prolonged Pericardial Fluid Drainage.

BACKGROUND: Large pericardial effusion (LPE) is associated with high mortality. In patients with cardiac tamponade or with suspected bacterial etiology of pericardial effusion, urgent pericardial decompression is necessary. AIM: The aim of the present retrospective study was to assess the short-term results of pericardial decompression combined with prolonged drainage in LPE. MATERIAL: This study included consecutive patients with LPE who had been treated with pericardial fluid drainage between 2007 and 2017 in the National Tuberculosis and Lung Diseases Research Institute. METHODS: Echocardiographic examination was used to confirm LPE and the signs of cardiac tamponade. Pericardiocentesis or surgical decompression were combined with pericardial fluid (PF) drainage. Short-term effectiveness of therapy was defined as less than 5 mm of fluid behind the left ventricular posterior wall in echocardiography. RESULTS: The analysis included 74 patients treated with pericardial fluid drainage (33 female and 41 male), mean age 58 years, who underwent pericardial decompression. Out of 74 patients, 26 presented with cardiac tamponade symptoms. Pericardiocentesis was performed in 18 patients and pericardiotomy in 56 patients. Median PF drainage duration was 13 days. In 17 out of 25 patients with neoplastic PF, intrapericardial cisplatin therapy was implemented. In 4 out of 49 patients with non-malignant PF, purulent pericarditis was recognized and intrapericardial fibrinolysis was used. Short-term effectiveness of the therapy was obtained in all of patients. Non-infective complications were noted in 16% of patients and infective ones in 10%. CONCLUSION: Pericardial decompression combined with prolonged PF drainage was safe and efficient method of LPE treatment.

Tuberculous Pericarditis-Own Experiences and Recent Recommendations.

Tuberculous pericarditis (TBP) accounts for 1% of all forms of tuberculosis and for 1-2% of extrapulmonary tuberculosis. In endemic regions, TBP accounts for 50-90% of effusive pericarditis; in non-endemic, it only accounts for 4%. In the absence of prompt and effective treatment, TBP can lead to very serious sequelae, such as cardiac tamponade, constrictive pericarditis, and death. Early diagnosis of TBP is a cornerstone of effective treatment. The present article summarises the authors' own experiences and highlights the current status of knowledge concerning the diagnostic and therapeutic algorithm of TBP. Special attention is drawn to new, emerging molecular methods used for confirmation of M. tuberculosis infection as a cause of pericarditis.

Pulmonary amyloidosis mimicking interstitial lung disease and malignancy - A case series with a review of a pulmonary patterns.

BACKGROUND: Amyloidosis is an uncommon condition, which results from accumulation of misfolded extracellular insoluble protein in tissues and organs of the body, causing its damage and dysfunction. Histologically, after staining with Congo red, the amyloid deposits show an apple-green birefringence under polarized light microscope. Amyloidosis can affect all organ systems and is classified into hereditary or acquired, localized or systemic. Respiratory involvement occurs in 50% of the patients with amyloidosis and it may take tracheobronchial, nodular parenchymal, diffuse alveolar septal and lymphatic forms. METHODS: We report four cases of pulmonary amyloidosis. A female patient with localized form of tracheobronchial and nodular parenchymal pulmonary amyloidosis, which was initially misdiagnosed as sarcoidosis. A male patient who was referred to our department for further evaluation of multiple tumors in lungs accompanied by mediastinal lymphadenopathy, liver and peritoneal tumors. A male patient with suspect of lung malignancy. A male patient with diagnosed idiopathic pulmonary fibrosis and the possibility of malignancy. RESULTS: All the diagnoses were established by demonstration of amyloid protein in tissue specimens obtained in transbronchial or open lung biopsies. CONCLUSIONS: Due to its nonspecific clinical and radiological findings, amyloidosis can often mimic other diseases and should be considered as one of the differential diagnoses. In order to confirm the diagnosis, proving the presence of amyloid deposition with positive Congo red staining in respiratory specimen is mandatory.

A thymoma or not a thymoma-that is the question: a case report.

Thymomas are malignant, epithelial tumors of the thymus of diverse morphology that may metastasize or relapse after resection. The WHO histological classification includes five main subtypes A, AB, B1, B2 and B3. Types A and AB usually harbour a specific GTF2I gene mutation. Thymolipomas are very rare, benign tumors composed of thymic parenchyma and adipose tissue. We present the case of a 37-year-old male with an incidentally found mediastinal tumor that shared morphological features of a thymoma of unknown histological type and a thymolipoma-like tumor. Microscopically the tumor contained three components: (I) a highly organoid component that reproduced the thymic parenchyma with numerous Hassall corpuscles; (II) a lymphocyte-poor, epithelial component; (III) mature adipose tissue. A wide panel of immunohistochemical tests was used, but the results were not decisive for differential diagnosis. Genetic analysis of GTF2I, BRAF and NRAS genes revealed no mutations. The tumor was completely resected. The patient did not receive adjuvant radiotherapy. A 1.5 years after resection there was no evidence of tumor recurrence. Based on our case we carefully analyse and compare the microscopic features of thymoma vs. thymolipoma. The differentiation between these tumors is crucial due to their distinct clinical course and required therapeutic approach.

SARS-CoV-2 lung disease in a patient with pulmonary sarcoidosis - case report.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is the recently identified cause of the current pandemic. In patients with chronic respiratory lung diseases, SARS-CoV-2 may result in significant morbidity and increased mortality. We present a case of a 69-year-old male with stage II pulmonary sarcoidosis who had been under observation for 30 months without immunosuppressive treatment. He then developed severe SARS-CoV-2 disease with typical radiological and laboratory findings. Therapy with oxygen, antibiotics, low-molecular-weight heparin in a prophylactic dose, and dexamethasone resulted in marked clinical improvement. We will discuss the rationale for corticosteroid use in both SARS-CoV-2 disease and in SARS-CoV-2 disease that is complicating comorbid sarcoidosis.

Familial pneumothoraces - Birt-Hogg-Dubé syndrome. Differentiation with other cystic lung diseases.

Birt-Hogg-Dubé syndrome (BHDS) is a rare, genetic, autosomal dominant disease caused by mutation in a folliculin gene. This syndrome is characterised by three main symptoms: benign lesions originating from hair follicles, variously shaped cysts in the lungs, and various types of benign and malignant kidney neoplasms. In our article we are going to present cases of two sisters with BHDS. In the case of the first sister skin lesions were accompanied by lung abnormalities. The second sister, however, presented with recurrent pneumothoraces associated with variously shaped lung cysts located mainly below the tracheal carina. In both instance diagnosis was confirmed by genetic test.

[The bleeding into the emphysematosus bulla imitating lung tumor]. / Krwawienie do pecherza rozedmowego imitujace guz pluca.

Bleeding into the lung parenchyma is a rare phenomenon that usually occurs as a result of chest trauma, other causes are anticoagulant therapy, and infections. The following case presents a patient admitted to the hospital due to haemoptysis, which was a symptom of bleeding into the emphysematosus bulla caused by anticoagulation therapy. The decisive diagnostic examination was chest magnetic resonance. This imaging method allows the precise differentiation of tissues. Using modern imaging techniques can often dispense with invasive diagnostic methods.