Extrapulmonary Manifestations Research Articles

Different faces of Mycoplasma pneumoniae in children - cardiological and skin complications- case reports and literature review

Mycoplasma pneumoniae is one of the main pathogens responsible for atypical pneumonia, especially in children over the age of 5, where it accounts for about 40% of cases of extramedullary pneumonia. In addition to respiratory-related symptoms, it can lead to a variety of extrapulmonary manifestations associated with involvement of other organs. In this article, we present case reports of two pediatric patients with detected M. pneumoniae infection with associated cardiac and skin complications. The first case report describes an 11.5-year-old boy who was admitted to the Department of Pediatrics because of chest pain, fever and moist cough for several days, who was diagnosed with myocarditis during M. pneumoniae infection. The second case report describes a 17-year-old girl with a recent respiratory infection who was admitted to the hospital for pain, swelling, blistering of the lips and oral mucosa. Mycoplasma pneumoniae infection can lead to extrapulmonary complications in children, which can produce nonspecific symptoms, so clinical vigilance and early recognition are important to minimize the risk of complications.

Tuberculosis in infertility and in vitro fertilization-embryo transfer.

Tuberculosis (TB) is a prominent infectious disease globally that imposes a substantial health burden. Genital TB (GTB), an extrapulmonary manifestation, leads to complications such as tubal adhesions, blockage, and diminished ovarian function, culminating in infertility, and is recognized as a prevalent cause of infertility in nations with high-burden TB. In regions with low TB rates, infertility and active TB during pregnancy have been reported to be most common among female immigrants from countries with high-burden TB. In the context of TB, pregnant women often exhibit exacerbated symptoms after in vitro fertilization-embryo transfer (IVF-ET), heightening the risk of dissemination. Miliary pulmonary TB and tuberculous meningitis pose a serious threat to maternal and fetal health. This article integrates recent epidemiological data and clinical research findings, delineating the impact of TB on infertility and assisted reproduction and particularly focusing on the diagnosis and treatment of GTB, underscored by the imperative of TB screening before IVF-ET. Our objective is to increase awareness among respiratory and reproductive health professionals, promoting multidisciplinary management to enhance clinical vigilance. This approach seeks to provide patients with judicious reproductive plans and scientifically rigorous pregnancy management, thereby mitigating adverse pregnancy outcomes related to TB activity.

Effects and mechanisms of supramaximal high-intensity interval training on extrapulmonary manifestations in people with and without chronic obstructive pulmonary disease (COPD-HIIT): study protocol for a multi-centre, randomized controlled trial

BackgroundBeyond being a pulmonary disease, chronic obstructive pulmonary disease (COPD) presents with extrapulmonary manifestations including reduced cognitive, cardiovascular, and muscle function. While exercise training is the cornerstone in the non-pharmacological treatment of COPD, there is a need for new exercise training methods due to suboptimal adaptations when following traditional exercise guidelines, often applying moderate-intensity continuous training (MICT). In people with COPD, short-duration high-intensity interval training (HIIT) holds the potential to induce a more optimal stimulus for training adaptations while circumventing the ventilatory burden often associated with MICT in people with COPD. We aim to determine the effects of supramaximal HIIT and MICT on extrapulmonary manifestations in people with COPD compared to matched healthy controls.MethodsCOPD-HIIT is a prospective, multi-centre, randomized, controlled trial with blinded assessors and data analysts, employing a parallel-group designed trial. In phase 1, we will investigate the effects and mechanisms of a 12-week intervention of supramaximal HIIT compared to MICT in people with COPD (n = 92) and matched healthy controls (n = 70). Participants will perform watt-based cycling two to three times weekly. In phase 2, we will determine how exercise training and inflammation impact the trajectories of neurodegeneration, in people with COPD, over 24 months. In addition to the 92 participants with COPD performing HIIT or MICT, a usual care group (n = 46) is included in phase 2. In both phases, the primary outcomes are a change from baseline in cognitive function, cardiorespiratory fitness, and muscle power. Key secondary outcomes include change from baseline exercise tolerance, brain structure, and function measured by MRI, neuroinflammation measured by PET/CT, systemic inflammation, and intramuscular adaptations. Feasibility of the interventions will be comprehensively investigated.DiscussionThe COPD-HIIT trial will determine the effects of supramaximal HIIT compared to MICT in people with COPD and healthy controls. We will provide evidence for a novel exercise modality that might overcome the barriers associated with MICT in people with COPD. We will also shed light on the impact of exercise at different intensities to reduce neurodegeneration. The goal of the COPD-HIIT trial is to improve the treatment of extrapulmonary manifestations of the disease.Trial registrationClinicaltrials.gov: NCT06068322. Prospectively registered on 2023-09-28.

Body compositions phenotypes of older adults with COPD.

Changes in nutritional status are important extrapulmonary manifestations of the chronic obstructive pulmonary disease (COPD). The study aimed to assess the prevalence of different body composition phenotypes in older patients with COPD and to investigate the relationship between these phenotypes and the severity of the disease, as well as physical performance of the subjects. The study included 124 subjects aged ≥60 with COPD. In all of them body composition analysis and muscle strength measurement were performed. Additionally, data from patients' medical records were analyzed. Study sample was divided into four groups based on the phenotypic body composition: normal phenotype (N), sarcopenia, obesity and sarcopenic obesity (SO). Incidence of sarcopenia was significantly higher in patients with severe or very severe COPD based on GOLD in comparison with subjects with mild or moderate obstruction (p = 0.043). Participants with sarcopenia, obesity and SO had lower results of the 6-min walk test than subjects with N (225.77 m, 275.33 m, 350.67 m, 403.56 m, respectively). Moreover, sarcopenia and SO had lower results than obesity (p = 0.001, p = 0.041, respectively). Sarcopenia is common in patients with advanced COPD. Sarcopenia and SO are associated with poorer physical performance. All older people with COPD should routinely have their body composition assessed, instead of simply measuring of body weight or body mass index (BMI).

12268 Hypercalcemia Alert: Exploring The Link To Isolated Bone Marrow Sarcoidosis

Abstract Disclosure: M. Renzu: None. C.M. Hubers: None. V. mehta: None. A. Qazi: None. D.K. Yerasuri: None. Introduction: Sarcoidosis, a complex disorder marked by granuloma formation in diverse organs, often affects the lungs, but can involve various systems as well. Rare cases of bone marrow involvement may mimic conditions like multiple myeloma, emphasizing the need to consider sarcoidosis in patients with bone marrow abnormalities, especially those without typical myeloma features. Case Presentation: A 79-year-old white male with a medical history of hypertension, hyperlipidemia, deep venous thrombosis, prostate cancer in remission, chronic knee pain, and basal cell carcinoma presented for pre-operative evaluation, as he was scheduled for an elective orthopedic procedure. On routine lab work, he was found to have acute hypercalcemia and normocytic anemia. This finding raised suspicion for an underlying systemic pathology. He reported that he did not take any calcium or vitamin D supplements at home. Subsequent endocrine assessment, including evaluation of parathyroid hormone and vitamin D levels, revealed a non-PTH-mediated hypercalcemia. The persistence of hypercalcemia prompted further investigations, including assessments for multiple myeloma by oncology. The workup was negative for multiple myeloma but significant for granulomas on bone marrow biopsy, a finding consistent with sarcoidosis. Additionally, angiotensin converting enzyme levels were elevated at the time of diagnosis. Interventions led to the resolution of hypercalcemia following steroid therapy of prednisone 20 mg daily and close follow up with endocrinology. As part of his follow up, ACE levels are being regularly monitored, with the patient going for lab work every other month. Discussion: Bone marrow biopsies rarely reveal granulomas and sarcoidosis accounts for only a very small percentage of these findings. The standard workup for sarcoidosis typically does not include bone marrow biopsies. By integrating biopsies into the standard diagnostic protocol researchers may gain insights into the etiology and mechanisms driving this manifestation of sarcoidosis, ultimately improving diagnostic accuracy and patient care. Distinguishing sarcoidosis from multiple myeloma also poses diagnostic challenges, necessitating thorough evaluation in patients with bone marrow abnormalities. Despite its rarity, clinical suspicion for sarcoidosis should be considered in patients with underlying hematologic disorders or malignancies. Additionally, sarcoidosis exhibits geographical variation, being less common in Japanese men and more prevalent in African American women. This highlights the importance of considering demographic factors when evaluating patients for sarcoidosis and its extrapulmonary manifestations. Long-term outcomes in isolated bone marrow sarcoidosis remain unclear, warranting surveillance for progression to multiple myeloma or other lymphoproliferative disorders. Presentation: 6/2/2024

Tuberculous calcific constrictive pericarditis in an adult female with a past history of abdominal tuberculosis.

Tuberculous pericarditis, one of the rarer extrapulmonary manifestations of tuberculosis, remains a challenge in endemic countries such as India. As the clinical presentation of tuberculous pericarditis is highly variable, the diagnosis is often missed, delayed, or incorrectly diagnosed. Constrictive pericarditis (CP) is one of tuberculous pericarditis's most serious and advanced sequelae. Several invasive and non-invasive diagnostic modalities are crucial to address the challenges of confirming the diagnosis of CP in suspected cases. This study reports the case of an Indian adult female with a past history of previously treated abdominal tuberculosis diagnosed anew with tuberculous calcific constrictive pericarditis.

S4203 COVID-19 and Extra-Pulmonary Manifestations: A Case Study of Marked Severe Hepatic Enzyme Elevation in the Absence of Respiratory Symptoms

S4203 COVID-19 and Extra-Pulmonary Manifestations: A Case Study of Marked Severe Hepatic Enzyme Elevation in the Absence of Respiratory Symptoms

Anal tuberculosis presented as chronic perianal abscess in an immunocompetent adult: A rare case report

ABSTRACT There are reported 7.5 million cases of extra pulmonary tuberculosis (TB) (EPTB) worldwide and 19% in the South-East Asia region. Anal TB represents an exceedingly uncommon extra pulmonary manifestation of TB), occurring in fewer than 1% of individuals who acquire TB. The occurrence of perianal illness is extremely rare, accounting for 1% of the gastrointestinal (GI) tract, while GI TB makes up less than 1% of all TB cases. The most prevalent manifestation is the development of a fistula in the anus, while the presentation as a perianal abscess is uncommon. We present a case of isolated perianal TB with no associated GI or pulmonary dissemination in the immunocompetent.

Characteristics of Severe Asthma Clinic Patients with Eosinophilic Granulomatosis with Polyangiitis

BackgroundEosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis associated with varying clinical presentations and overlapping multiorgan involvement. Asthma is a predominant feature of EGPA, typically in its prodromal phase, often severe and precedes vasculitic complications. However, there is paucity of studies describing the prevalence and characteristics of EGPA in the asthma population. ObjectiveWe aim to describe the clinical and serological characteristics and longitudinal therapeutic outcomes of patients with EGPA in the severe asthma (SA) cohort. MethodsRetrospective study of patients with EGPA attending the multidisciplinary SA clinic in a tertiary hospital from 2011 to 2023 was conducted. Baseline demographics, organ manifestations, biological markers, lung function and therapeutic outcomes were assessed. Results23 out of 596 patients in the SA registry were identified to have EGPA. Median time interval between asthma and EGPA diagnosis was 10 years (range 2.5 to 32 years). Almost all patients (95.7%) had peak blood eosinophil count of >1.0 x 109/L (range 0.47 – 14.08 x 109/L). Upper airway involvement was the most detected manifestation in addition to asthma, followed by neuropathy and renal involvement. Patients who were treated with biologic therapy were significantly younger, had more upper airway, renal, pulmonary involvement and lower five factor score (FFS). ConclusionThe prevalence of EGPA in the SA population was 3.9% in our cohort. Its diagnosis requires high clinical suspicion in patients with SA and blood eosinophilia, prompting stringent evaluation for extrapulmonary manifestations and multidisciplinary involvement.

Extra-pulmonary manifestations of COVID-19: A comprehensive review

The COVID-19 pandemic, caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has predominantly been recognized for its respiratory impact. However, as the pandemic has progressed, it has become evident that SARS-CoV-2 affects multiple organ systems beyond the lungs, resulting in a wide array of extra-pulmonary manifestations. These manifestations encompass cardiovascular, neurological, gastrointestinal, hepatic, renal, dermatological, hematological, endocrine, musculoskeletal, ocular, immunological, and psychiatric complications. This review aims to provide a comprehensive understanding of these extra-pulmonary manifestations, highlighting their clinical significance, underlying mechanisms, and implications for patient management. Recognizing these manifestations is critical for improving patient outcomes and informing ongoing research and therapeutic strategies.

Elevated serum angiotensin converting enzyme correlates with specific HLA-DRB1 alleles and extrapulmonary manifestations in sarcoidosis

BackgroundGenetics influence the clinical picture in sarcoidosis, a granulomatous heterogeneous disease often accompanied by elevated serum angiotensin converting enzyme (s-ACE). We aimed to investigate if certain HLA-DRB1 alleles correlate with the levels of s-ACE, known as a marker of the granuloma burden. MethodsMedical journals of patients with sarcoidosis from a Swedish clinical registry were retrospectively examined to extract the highest recorded s-ACE value and analysed in relation to patient characteristics including phenotype [Löfgren syndrome (LS)/ non-LS], chest X-ray staging according to Scadding, treatment with immunosuppressants, presence of extrapulmonary manifestations (EPM), HLA-DRB1 alleles and prognosis (resolving vs. non-resolving disease within 2 years). Data were analysed with Fisher's exact test and Bonferroni correction was applied for HLA analyses. ResultsOf 1204 patients included, 40% had s-ACE levels above reference value. In comparison with patients with normal s-ACE, those with elevated levels were more often classified into non-LS (78% vs 59%, p < 0.001), and Scadding stage II (50% vs 38%, p < 0.001) but less often Scadding stage I (33% vs 46%, p < 0.001) and had more often EPM (45% vs 23%, p < 0.001). The patients with HLA-DRB1×04 had more often elevated s-ACE (p < 0.01) while those with HLA-DRB1×03 commonly had normal levels (p < 0.001). ConclusionsIn this retrospective study, HLA alleles associated with s-ACE levels in sarcoidosis patients, which in turn correlated with occurrence of EPM. These findings shed some new light on possible mechanisms behind differences in s-ACE levels.

No Increase in Severity of Mycoplasma pneumoniae: Insights From the Postpandemic Epidemic.

Following near-absence during the COVID-19 pandemic, Mycoplasma pneumoniae infection has recently reemerged in children, much later than other respiratory pathogens. We describe the resurgence that we have observed starting in late 2023. Compared with prepandemic, disease severity and extrapulmonary manifestations did not change, while median age at presentation increased.

Mendelian randomization provides causal association between COVID-19 and thyroid cancer: insights from a multi-cancer analysis.

Since the onset of the COVID-19 pandemic, the SARS-CoV-2 virus has caused over 600 million confirmed infections and more than 6.8 million deaths worldwide, with ongoing implications for human health. COVID-19 has been extensively documented to have extrapulmonary manifestations due to the widespread expression of necessary ACE2 receptors in the human body. Nevertheless, the association between COVID-19 and cancer risk remains inadequately explored. This study employs Mendelian randomization (MR) methods to examine the causal relationship between genetic variations associated with COVID-19 and the risk of developing cancer. The findings indicate that COVID-19 has negligible impact on most cancer risks. Interestingly, a higher COVID-19 impact is associated with a decreased risk of thyroid cancer. In summary, our findings demonstrate a genetic correlation between COVID-19 and thyroid cancer, contributing to our understanding of the interplay between COVID-19 and cancer risk.

Is YouTube a sufficient source of information on Sarcoidosis?

BackgroundThe internet is a common source of health information for patients and caregivers. To date, content and information quality of YouTube videos on sarcoidosis has not been studied. The aim of our study was to investigate the content and quality of information on sarcoidosis provided by YouTube videos.MethodsOf the first 200 results under the search term “sarcoidosis,” all English-language videos with content directed at patients were included. Two independent investigators assessed the content of the videos based on 25 predefined key features (content score with 0–25 points), as well as reliability and quality (HONCode score with 0–8 points, DISCERN score with 1–5 points). Misinformation contained in the videos was described qualitatively.ResultsThe majority of the 85 included videos were from an academic or governmental source (n = 63, 74%), and median time since upload was 33 months (IQR 10–55). Median video duration was 8 min (IQR 3–13) and had a median of 2,044 views (IQR 504 − 13,203). Quality assessment suggested partially sufficient information: mean HONCode score was 4.4 (SD 0.9) with 91% of videos having a medium quality HONCode evaluation. Mean DISCERN score was 2.3 (SD 0.5). Video content was generally poor with a mean of 10.5 points (SD 0.6). Frequently absent key features included information on the course of disease (6%), presence of substantial geographical variation (7%), and importance of screening for extrapulmonary manifestations (11%). HONCode scores were higher in videos from academic or governmental sources (p = 0.003), particularly regarding “transparency of sponsorship” (p < 0.001). DISCERN and content scores did not differ by video category.ConclusionsMost YouTube videos present incomplete information reflected in a poor content score, especially regarding screening for extrapulmonary manifestations. Quality was partially sufficient with higher scores in videos from academic or governmental sources, but often missing references and citing specific evidence. Improving patient access to trustworthy and up to date information is needed.

Cardiovascular Complications in Respiratory Viral Infections with a Focus on COVID-19

Patients with respiratory viral infections have altered immune responses, which may predispose them to cardiovascular complications. In the face of the pandemic of a new kind of severe acute respiratory syndrome (SARS), coronavirus disease 2019 (COVID-19), there is a resurgence of interest in the early diagnosis, prevention, and treatment of patients who are at risk. COVID-19 often manifests as viral pneumonia, although extrapulmonary manifestations are also common. Acute cardiac damage associated with elevated highsensitivity troponin levels crucially contributes to mortality in severe COVID-19. The present review clinically compares cardiovascular complications between COVID-19 and other respiratory infections caused by singlestranded RNA viruses, namely influenza, SARS, and Middle East respiratory syndrome (MERS). Estimating the death rate from RVIs has been a subject of intense research, but the mortality from cardiovascular complications in these infections is less understood and calls for further research.

Болезнь легионеров с неврологическими проявлениями: клинический случай

INTRODUCTION. Legionnaires' disease may be manifested by neurological symptoms along with lung damage. Cerebellar ataxia and dysarthria are rare extrapulmonary manifestations of legionellosis. These symptoms significantly reduce the quality of life of patients. AIM. To analyze the literature data on neurological manifestations of legionellosis and to present a clinical case of Legionnaires' disease manifestation with neurological symptoms. MATERIAL AND METHODS. We performed an analysis of the available literature. Twenty-four Russian and international literature sources (PubMed databases) were evaluated, describing the characteristics and prevalence of neurological manifestations in Legionnaires' disease. We also described the main theories of the pathogenesis of these clinical manifestations. Our clinical case is presented. RESULTS AND DISCUSSION. As of today, few cases of Legionnaires' disease with cerebellar symptoms have been described. Dysarthria and ataxia are the most frequent manifestations of cerebellar dysfunction in legionellosis. Cases where neurologic symptoms persisted after the resolution of pneumonia have been described previously. There are also reports of manifestation of Legionnaires' disease with cerebellar ataxia. The pathophysiology of cerebellar involvement is unknown. There are hypotheses of toxin-mediated and immune-mediated mechanisms. Recent studies have shown that L. pneumophila can activate receptors responsible for enacting the inflammasome pathway. This mechanism promotes cell death that occurs to remove the intracellular pathogen, which may be responsible for central nervous system dysfunction. CASE PRESENTATION. A 48-year-old woman was hospitalized with a suspected stroke. She works in diamond production and is in contact with aerosolized liquids. Four days before hospitalization she complained of a headache, and in the evening before she lost consciousness. Slurred speech, unsteadiness, and inability to walk were noted. At hospitalization, no instrumental evidence of stroke was obtained. Inflammatory markers were elevated. Chest computer tomography showed infiltrative changes. The rapid test for antigenuria (L. pneumophila) was positive, and community-acquired legionellosis was confirmed. Levofloxacin was administrated. After discharge, dysarthria, dysphagia, and instability in the Romberg pose persisted. CONCLUSION. The example of this clinical observation demonstrates that in rare cases Legionnaires' disease can manifest as other diseases, which requires a certain vigilance of doctors of different specialities in order to timely diagnose the disease. KEYWORDS: Legionnaires' disease, legionellosis, community-acquired pneumonia, central nervous system, infectious disease

Pneumomediastinum and pneumoretroperitoneum after COVID-19: concealed intestinal perforation

BackgroundWith the prevalence of coronavirus disease 2019 (COVID-19), many severe cases have been discovered worldwide. Here, a case of concurrent pneumomediastinum, pneumoretroperitoneum, and intestinal perforation was reported. This case was the first report on COVID-19-induced related complications.Case presentationA 74-year-old female patient was hospitalized for COVID-19. Air leakage was unexpectedly found during imaging reexamination. Considering the unobvious subjective feeling of the patient, a conservative treatment was given at the early stage, and finally, sigmoid colon perforation was surgically confirmed. The family gave up the treatment at last, because the patient could not be taken off the ventilator. Coincidentally, the patient also had abnormal renal anatomical position. This situation led to an abnormal air leakage direction and the atypical manifestations of peritonitis. It was also one of the important reasons for the delayed diagnosis and treatment of the disease.ConclusionsClinicians should be vigilant for spontaneous gastrointestinal perforation in patients with COVID-19, particularly those undergoing treatment with glucocorticoids and tocilizumab. The case is shared to highlight this rare and fatal extrapulmonary manifestation of COVID-19 and further assist clinicians to raise their awareness and timely implement imaging investigation and multidisciplinary intervention so as to facilitate early discovery, diagnosis and treatment and reduce the mortality.

The correlation between the level of cellular classification in bronchoalveolar lavage fluid of children with severe Mycoplasma pneumoniae and clinical characteristics.

The BALF of children with SMPP is predominantly neutrophilic. A lower percentage of lymphocytes is related to a higher incidence of pleural effusion, extrapulmonary manifestations and progression to RMPP, as well as a longer length of hospitalisation. • Mycoplasma pneumonia in children is relatively common in clinical practice. Bronchoalveolar lavage (BAL) is a routine clinical procedure. However, there are relatively few studies focusing on the cytomorphological analysis of cells in BAL fluid.

Elexacaftor-tezacaftor-ivacaftor use after solid organ transplant.

In 2019, the United States Food and Drug Administration approved a breakthrough therapeutic for cystic fibrosis, elexacaftor-tezacaftor-ivacaftor (ETI), because of its profound effect on lung function in large phase III clinical trials. ETI acts directly on the dysfunctional protein that causes the systemic manifestations of cystic fibrosis and also leads to improvement in nonpulmonary symptoms of cystic fibrosis. Transplant recipients were excluded from the pivotal clinical trials of ETI but may stand to benefit from correction of the underlying protein defect. Drug interactions between the three drugs in ETI and immunosuppression medications are one of the primary concerns about using ETI after transplant. No rigorous studies exist to assess the safety of ETI in transplant recipients. Multiple recent publications describe the use of ETI after solid organ transplantation, including lung and nonlung transplants, and the real-world evidence for drug interactions between ETI and immunosuppression medications. In nonlung transplant recipients, the pulmonary benefits of ETI have been confirmed, but adverse events occur and may have implications for their transplanted organ (e.g. liver biopsy in the setting of elevated transaminases). Lung transplant recipients may have higher rates of ETI discontinuation than nontransplant recipients given a lack of direct pulmonary benefit and frequency of side effects. Drug interactions have not been difficult to manage, with most studies reporting variable rates of mild to moderate increased tacrolimus levels after initiation of ETI. Limited data exist to support the use of ETI after solid organ transplantation and further research is warranted. Given the unknown risks and benefits, case by case consideration of ETI use is indicated when extra-pulmonary manifestations are present in lung transplant recipients with cystic fibrosis. Given the proven benefit in cystic fibrosis lung disease, benefits likely outweigh the risks of ETI for nonlung solid organ transplant recipients.

Sarcoidosis versus Granulomatous and Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency: A Comparative Review.

Sarcoidosis and Granulomatous and Lymphocytic Interstitial Lung Diseases (GLILD) are two rare entities primarily characterised by the development of Interstitial Lung Disease (ILD) in the context of systemic immune dysregulation. These two conditions partially share the immunological background and pathologic findings, with granuloma as the main common feature. In this narrative review, we performed a careful comparison between sarcoidosis and GLILD, with an overview of their main similarities and differences, starting from a clinical perspective and ending with a deeper look at the immunopathogenesis and possible target therapies. Sarcoidosis occurs in immunocompetent individuals, whereas GLILD occurs in patients affected by common variable immunodeficiency (CVID). Moreover, peculiar extrapulmonary manifestations and radiological and histological features may help distinguish the two diseases. Despite that, common pathogenetic pathways have been suggested and both these disorders can cause progressive impairment of lung function and variable systemic granulomatous and non-granulomatous complications, leading to significant morbidity, reduced quality of life, and survival. Due to the rarity of these conditions and the extreme clinical variability, there are still many open questions concerning their pathogenesis, natural history, and optimal management. However, if studied in parallel, these two entities might benefit from each other, leading to a better understanding of their pathogenesis and to more tailored treatment approaches.