Challenges in the management of idiopathic pulmonary fibrosis from a low- and middle-income country.

Idiopathic pulmonary fibrosis (IPF) is the most common progressive form of interstitial lung disease (ILD) that leads to gradual deterioration of lung function and ultimately death. Data from low- and middle-income countries (LMIC) on IPF is scarce. In this communication, we report the challenges encountered in managing IPF from Pakistan's largest tertiary care centre. A total of 108 patients with IPF were evaluated at the Aga Khan University Hospital in Karachi, Pakistan from January 2017 to March 2020. A significant concern was that most patients with IPF presented late during their disease. A bigger challenge encountered in clinical practice was the cost and nonavailability of antifibrotic therapy in the country until mid-2020. Successfully addressing these limitations, it is anticipated that better care will be available for the patients suffering from IPF in this part of the world.

Pneumocystis pneumonia in HIV-positive and non-HIV patients: a retrospective comparative study from a lower-middle income country.

In this study, we compared the predisposing factors, key demographic and clinical characteristics, clinical outcomes, and factors associated with poor prognosis in pneumocystis pneumonia (PCP) infection among the human immunodeficiency virus (HIV)-positive and non-HIV patient populations. This retrospective analysis was conducted at the Aga Khan University Hospital, Karachi, via the collection and analysis of patient records with a diagnosis of "pneumocystosis" between January 2015 and October 2020. Additionally, the laboratory database was evaluated, and patients with a laboratory-confirmed diagnosis of PCP were included. During the study period, 52 laboratory-confirmed hospitalized PCP patients were identified. Of these, 23 and 29 patients were diagnosed using microscopy and polymerase chain reaction, respectively. 34.6% of our patients were HIV positive, with a median CD4 count of 20.5 cells/mm3 (range: 10.7-50.5). Other conditions identified were corticosteroid use, autoimmune diseases, malignancy, radiation, and chemotherapy. On chest imaging, consolidation was found in 30%, ground-glass opacities in 24%, and nodular infiltrates in 20% of the cases. HIV-positive patients had a lower hemoglobin level and a higher level of ß-D-glucan at the time of admission, whereas non-HIV patients were found to have more co-morbid conditions than HIV patients. We observed no difference in clinical outcomes between the two populations. Factors associated with a poor prognosis among our patients included concomitant infections at the time of diagnosis, the need for invasive mechanical ventilation, and a longer duration of stay in the hospital as well as the intensive care unit.

Allergic bronchopulmonary aspergillosis: radiological and microbiological profile of patients presented in an outpatient pulmonary clinic in a developing country.

There is limited data available about allergic bronchopulmonary aspergillosis (ABPA) in Pakistan. The aim of the study was to describe the radiological and microbiological profile of ABPA patients presenting to the outpatient pulmonary clinic of a tertiary care hospital in Karachi, Pakistan. A retrospective study was conducted on ABPA patients who presented to the pulmonary outpatient clinic at Aga Khan University Hospital, Karachi, Pakistan, from January 2017 to December 2019. Data was collected on microbiology and radiology features on predesigned proforma. A total of 7759 asthmatic patients presented at the outpatient pulmonology clinic during the study period. Of the 245 patients labeled as ABPA, 167 fulfilled the inclusion criteria, and 91 (54.5%) were female (mean age 41.9±13.0 years). A high resolution computed tomography scan of the chest was available for 126 patients. Of these, 104 (82.5%) patients had bronchiectasis. Central bronchiectasis was noted in 98 (94.2%), mucus plugging in 71 (56.3%), and hyperinflation was seen in 30 (23.4%) patients. Microbiological testing was available in 103/167 (61.7%) patients. The most common bacterial pathogen was Pseudomonas aeruginosa 32 (31.1%), followed by Hemophilus influenzae 16 (15.5%), and Moraxella catarrhalis 7 (9.7%). Aspergillus fumigatus 17 (23.6%) was the most common mold, followed by Aspergillus flavus 16 (22.2%) and Aspergillus niger 11 (15.3%). Co-infection (bacterial and fungal) was found in 18 (17.45%) patients. Bronchiectasis was frequently observed in our cohort of patients with ABPA. Pseudomonas aeruginosa was found to be common among bacterial pathogens. Isolation of fungus is not uncommon in these patients.

An Easy-To-Use Prehospital Severity Scoring Tool To Triage COVID-19 Positive Adults In A Resource-Limited Setting.

Objectives: To develop an easy-to-use severity scoring tool for prehospital triage of patients infected by the coronavirus disease-2019 in resource-limited settings. METHODS: The cohort study was conducted at a tertiary care hospital in Karachi, from August to September 2020, and comprised adult patients of either gender who tested positive for coronavirus disease-2019 on real-time polymerase chain reaction. The scoring system and categorisation were based on validated scales for the detection of pneumonia and opinions from pulmonologists. Data was analysed using SPSS 19. RESULTS: Of the 206 participants, 100(48.5%) were in-patients and 106(51.5%) were out-patients. There were 144(69.9%) males and 62(30.1%) females with an overall mean age of 48.4±16.2 years. After categorisation based on severity, significantly higher number of in-patients were found to be in categories III and IV (p<0.05). CONCLUSIONS: The severity scoring tool could effectively help classify coronavirus disease-2019 patients into mild, moderate and severe cases.

Risk Factors Of Idiopathic Pulmonary Fibrosis In Pakistani Population: A Matched Case-Control Studyy.

OBJECTIVE: To evaluate the factors associated with idiopathic pulmonary fibrosisrisk. Methods: The case-controlstudywas conductedfromJanuary 5, 2017,toSeptember 4, 2018, attheprivate-sectorAga Khan University Hospital and the public-sector Jinnah Postgraduate Medical Centre, two large tertiary care centres in Karachi, andcomprisedadultpatientsof eithergenderwithdiagnosedidiopathicpulmonary fibrosis, asdefinedby the IndianChest Registry. Subjects without idiopathic pulmonary fibrosis but registered with the department of pulmonology of the two hospitalswere enrolledas controls.Datawas collectedusinga structuredquestionnaire, andanthropometricmeasurements were noted for each subject. Gastroesophageal reflux disease was assessed using GerdQ. This wasfollowed by serological evaluations and spirometry. Data was analysed using SPSS 21. RESULTS: Of the 459 subjects, 154(33.6%)were cases and305(66.4%)were controls.Amongthe cases, 81(52.6%)were females and 73(47.4%) were males with mean age 66.1±10.9 years. Among the controls, 162(53.1%) were females and 143(46.9%) were males with mean age 64.6±11.1 years(p>0.05.)The most common ethnicity wasUrdu-speaking; 89(58%) among the cases and 150(49%) among the controls (p<0.05). Ethnicity, number of persons in the household per room, and type of house were significantly associated with the risk of developing idiopathic pulmonary fibrosis(p<0.05). CONCLUSIONS: Ethnicity,type of house and the number of personsin a household perroom were found to be the significant risk factorsfor idiopathic pulmonary fibrosisIPF.

A global systematic review and meta-analysis on the emerging evidence on risk factors, clinical characteristics, and prognosis of multisystem inflammatory syndrome in adults (MIS-A).

Background: The multisystem inflammatory syndrome in adults (MIS-A) has emerged, similar to those in children associated with SARS-CoV-2 (severe acute respiratory syndrome coronavirus 2) [multisystem inflammatory syndrome in children (MIS-C)]. This review aimed to analyze the risk factors, clinical course, and prognosis of MIS-A. Methods: A comprehensive literature search was conducted using several databases for cases reporting MIS-A from 1 December 2019 till 9 September 2021. The case definitions used to identify potential cases were those recommended by the World Health Organization, Center for Disease Control, and individual country/physician classification. The meta-analysis was performed using Comprehensive Meta-Analysis (CMA) 2.2.027 and Review Manager (RevMan) 5.4.1, employing 95% confidence intervals (CI). Results: Seventy studies were assessed for full-text eligibility, out of which 37 were included. The mean age of the study population was 32.52±10.29 years. The most common symptoms were fever (89.8%, 95% CI: 77.7-95.7%) and diarrhea (49%, 95% CI: 35.4-62.7%). Ventricular tachycardia (57.1%, 95% CI: 43.1-70.1%) was the most common electro-cardiac abnormality. The most common inflammatory marker was elevated C-reactive protein (89.8%, 95% CI: 77.7-95.7%). Abnormal echocardiogram was the most common imaging test result (commonly, ventricular dysfunction and arrhythmias), while steroids were the most administered treatment. Severe cases had a higher need for vasopressor and inotropic support and antibiotic therapy compared to the non-severe cases. One death was reported due to cardiovascular failure. Conclusion: Our collated findings will help clinicians identify the typical presenting symptoms and optimal management of MIS-A. Further research is required to understand the long-term prognosis and the correlation between coronavirus disease 2019 (COVID-19) and MIS-A to understand its pathogenesis and clinical spectrum.

Macrolides for better resolution of community-acquired pneumonia: A global meta-analysis of clinical outcomes with focus on microbial aetiology.

OBJECTIVES: This meta-analysis examined the effect of macrolides on resolution of community-acquired pneumonia (CAP) and interpretation of clinical benefit according to microbiology; emphasis is given to data under-reported countries (URCs). METHODS: This meta-analysis included 47 publications published between 1994 and 2022. Publications were analysed for 30-d mortality (58 759 patients) and resolution of CAP (6465 patients). A separate meta-analysis was done for the prevalence of respiratory pathogens in URCs. RESULTS: Mortality after 30 d was reduced by the addition of macrolides (odds ratio [OR] 0.65, 95% confidence interval [CI] 0.51-0.82). The OR for CAP resolution when macrolides were added to the treatment regimen was 1.23 (95% CI 1.00-1.52). In the CAP resolution analysis, the most prevalent pathogen was Streptococcus pneumoniae (12.68%; 95% CI 9.36-16.95%). Analysis of the pathogen epidemiology from the URCs included 12 publications. The most prevalent pathogens were S. pneumoniae (24.91%) and Klebsiella pneumoniae (12.90%). CONCLUSION: The addition of macrolides to the treatment regimen led to 35% relative decrease of 30-d mortality and to 23% relative increase in resolution of CAP.

GRADE-ADOLOPMENT of clinical practice guidelines and creation of clinical pathways for the primary care management of chronic respiratory conditions in Pakistan.

INTRODUCTION: In Pakistan, chronic respiratory conditions contribute a large burden of morbidity and mortality. A major reason for this is the lack of availability of local evidence-based clinical practice guidelines (EBCPGs) in Pakistan, particularly at the primary care level. Thus, we developed EBCPGs and created clinical diagnosis and referral pathways for the primary care management of chronic respiratory conditions in Pakistan. METHODS: The source guidelines were selected by two local expert pulmonologists after a thorough literature review on PubMed and Google Scholar from 2010 to December 2021. The source guidelines covered idiopathic pulmonary fibrosis, asthma, chronic obstructive pulmonary disorders, and bronchiectasis. The GRADE-ADOLOPMENT process consists of three key elements: adoption (using recommendations as is or with minor changes), adaptation (effective context-specific changes to recommendations) or additions (including new recommendations to fill a gap in the EBCPG). We employed the GRADE-ADOLOPMENT process to adopt, adapt, adopt with minor changes, or exclude recommendations from a source guideline. Additional recommendations were added to the clinical pathways based on a best-evidence review process. RESULTS: 46 recommendations were excluded mainly due to the unavailability of recommended management in Pakistan and scope beyond the practice of general physicians. Clinical diagnosis and referral pathways were designed for the four chronic respiratory conditions, explicitly delineating the role of primary care practitioners in the diagnosis, basic management, and timely referral of patients. Across the four conditions, 18 recommendations were added (seven for IPF, three for bronchiectasis, four for COPD, and four for asthma). CONCLUSION: The widespread use of the newly created EBCPGs and clinical pathways in the primary healthcare system of Pakistan can help alleviate the morbidity and mortality related to chronic respiratory conditions disease in the country.

Post-COVID-19 sequelae of the respiratory system. A single-centre experience reporting the compromise of the airway, alveolar and vascular components.

The long-term sequelae of COVID-19 have now become more common and appreciable. The SARS-CoV-2 virus can cause a variety of infectious and non-infectious pulmonary complications. The purpose of this study is to raise awareness about post-COVID-19 pulmonary sequelae, both infectious and non-infectious, in this geographical area. A retrospective study was conducted from July 1st 2020 to December 20th 2020. A total of 1200 patients were evaluated, with 83 suffering from post-COVID-19 pulmonary complications. The patients' mean age was 62 years (IQR 55-69), with 63 (75.9%) being male. The most common co-morbid illnesses were hypertension (49, 59%) and diabetes (45, 54.2%). The majority of them (37, 44.6%) had severe COVID-19, followed by critical COVID-19 (33, 39.8%). There was no statistically significant difference in recurrence of respiratory symptoms or duration of current illness between non-severe, severe, and critical COVID-19 patients. Non-infectious complications were observed in the majority of patients (n=76, 91.5%), including organizing pneumonia/ground glass opacities in 71 (88%) patients, fibrosis in 44 (55%), pulmonary embolism in 10 (12.5%), pneumomediastinum in 6 (7.4%) and pneumothorax in 7 (8.6%). Infective complications (25, 30.1%) included aspergillus infection in 10 (12.0%) and bacterial infection in 5 (8.47%), with more gram-negative infections and one patient developing Mycobacterium tuberculosis. Post COVID-19 mortality was 11 (13.3%). The long-term pulmonary sequelae of COVID-19 are not rare. Cryptogenic organizing pneumonia, ground glass opacities, and fibrosis were common post-COVID-19 sequelae in our patients. This necessitates frequent close monitoring of these patients in order to initiate early appropriate management and prevent further morbidity and eventual mortality.

Sarcoidosis: An experience from a tertiary care center in Karachi Pakistan.

OBJECTIVE: To assess the demographic and clinical characteristics and treatment of patients with sarcoidosis in tertiary care settings. METHODS: The descriptive retrospective study was conducted at the Aga Khan University Hospital, Karachi, and comprised data from 2017 to 2019 of patients with established diagnosis of sarcoidosis. Data was collected on a predesigned proforma and it was analysed using SPSS 23. RESULTS: Of the 974 patients evaluated, sarcoidosis was established in 108(11.1%); 58(53.7%) of them being females. The overall mean age was 50.9±12.9 years. Hypertension 49(45.4%) and diabetes mellitus 37(34.3%) were the most frequent co-morbidities. Extra-pulmonary manifestations were found in 27(25%) patients; ophthalmic 9(33.3%), musculoskeletal 8(29.6%) and skin 7(25.9%). Prednisolone was the mainstay of treatment in 93(86.1%) patients. CONCLUSIONS: Sarcoidosis was not found to be rare in Pakistan.

Residual symptoms and the quality of life in individuals recovered from COVID-19 infection: A survey from Pakistan.

BACKGROUND: There remains scarcity of literature regarding the patient's health status post-COVID-19 infection. This study analyzes the prevalence of residual symptoms and quality of life (QoL) after COVID-19. METHODS: An anonymous online survey was administrated in Pakistan from November 2020 to April 2021 in COVID-19 survivors. The questionnaire used the 12-Item Short Form Health Survey (SF-12) to assess mental and physical QoL. Multivariate linear regression was used to explore factors associated with mental and physical QoL scores. RESULTS: A total of 331 COVID-19 survivors participated in our survey. Around 42.0% of the cohort reported within 1-3 months of diagnosis of COVID-19. The common residual symptoms were body aches (39.9%), low mood (32.6%), and cough (30.2%). Better physical QoL was associated with being male (adjusted beta: 3.328) and having no residual symptoms (6.955). However, suffering from nausea/vomiting during initial COVID-19 infection (-4.026), being admitted to the ICU during COVID-19 infection (-9.164), and suffering from residual body aches (-5.209) and low mood (-2.959) was associated with poorer QoL. Better mental QoL was associated with being asymptomatic during initial COVID-19 infection (6.149) and post-COVID (6.685), while experiencing low mood post-COVID was associated with poorer mental QoL (-8.253 [-10.914, -5.592]). CONCLUSION: Despite presumed "recovery" from COVID-19, patients still face a wide range of residual symptoms months after initial infection, which contributes towards poorer QoL. Healthcare professionals must remain alert to the long-lasting effects of COVID-19 infection and aim to address them appropriately to improve patients' QoL.

The effect of ivermectin on non-severe and severe COVID-19 disease and gender-based difference of its effectiveness.

The COVID-19 pandemic has led to mortality and morbidity since December 2019. Many possible treatment options have been advised till date. The role of ivermectin in the treatment of COVID-19 disease remains controversial. The aim of our study was to evaluate the effect of ivermectin in hospitalized patients with non-severe and severe COVID-19 disease. We conducted a retrospective cohort study that compared outcomes in 2 groups of COVID-19 patients hospitalized at the largest tertiary care center of Pakistan. The study group was given ivermectin along with standard treatment of covid-19 disease; the comparison group was not. Data on mortality, inflammatory markers such as C-reactive protein (CRP) and ferritin, length of hospital stay and baseline characteristics were collected from Aga Khan University's database from October 2020 till February 2021. Statistical analysis was done to determine the effectiveness of ivermectin in non-severe and severe COVID-19. Comparison of effectiveness of Ivermectin in both the genders was also conducted. The cohort included 188 patients out of which 90 were treated with ivermectin. Mortality and length of hospitalization was not found to be significantly different in the study group compared with the control group (5.6% vs 5.1%; p=0.87 and 5 days vs 4 days; p=0.27). Analysis of secondary outcomes did not yield statistically significant results, apart from ferritin levels which were significantly less in patients treated with ivermectin (547.1 vs 756.7; p=0.03). The ferritin and CRP levels in affected males were higher than in females on admission and discharge. Our findings suggest ivermectin does not significantly affect all-cause mortality, length of hospitalization and CRP levels in hospitalized COVID-19 patients. Large scale randomized controlled trials (RCTs) are required to further evaluate the role of ivermectin in covid-19 disease.

Persistence of post-COVID lung parenchymal abnormalities during the three-month follow-up.

INTRODUCTION: COVID-19-associated pulmonary sequalae have been increasingly reported after recovery from acute infection. Therefore, we aim to explore the charactersitics of persistent lung parenchymal abnormalities in patients with COVID-19. MATERIAL AND METHODS: An observational study was conducted in patients with post-COVID lung parenchymal abnormalities from April till September 2020. Patients ≥18 years of age with COVID-19 who were diagnosed as post-COVID lung parenchymal abnormality based on respiratory symptoms and HRCT chest imaging after the recovery of acute infection. Data was recorded on a structured pro forma, and descriptive analysis was performed using Stata version 12.1. RESULTS: A total of 30 patients with post-COVID lung parenchymal abnormalities were identified. The mean age of patients was 59.1 (SD 12.6), and 27 (90.0%) were males. Four HRCT patterns of lung parenchymal abnormalities were seen; organizing pneumonia in 10 (33.3%), nonspecific interstitial pneumonitis in 17 (56.7%), usual interstitial pneumonitis in 12 (40.0%) and probable usual interstitial pneumonitis in 14 (46.7%). Diffuse involvement was found in 15 (50.0%) patients, while peripheral predominance in 15 (50.0%), and other significant findings were seen in 8 (26.7%) patients. All individuals were treated with corticosteroids. The case fatality rate was 16.7%. Amongst the survivors, 32.0% recovered completely, 36.0% improved, while 32.0% of the patients had static or progressive disease. CONCLUSION: This is the first study from Southeast Asia that identified post-COVID lung parenchymal abnormalities in patients who had no pre-existing lung disease highlighting the importance of timely recognition and treatment of this entity that might lead to fatal outcome.

Role of ivermectin in patients hospitalized with COVID-19: a systematic review of literature.

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has affected almost every country in the world since De-cember 2019. Despite the efforts of the human race to combat the virus, we are still looking for an evidence-based permanent cure for the disease. Ivermectin has recently emerged as one of the therapies having a beneficial effect on COVID-19. Ivermectin, owing to its properties, continues to be a possible treatment against the COVID-19 disease. Already being a mainstream drug with minimal adverse effects, it garners valid consideration. It's use in hospitalized patients, randomized controlled trials, and observational studies has also supported its implementation. In this article, we have reviewed recent studies and explored the effectiveness of ivermectin in hospitalized COVID-19 patients.

Aspirated Denture: A Rare Cause of Empyema in an Asian Male.

Here, we report the case of an undiagnosed foreign body aspiration (FBA) in a 50-year-old male who presented with fever, productive cough, and shortness of breath suggestive of pneumonia. The patient reported a history of empyema for which he underwent left-sided video-assisted thoracoscopic surgery and decortication at another facility. Careful evaluation of prior chest imaging revealed a radio-opaque linear density projecting along the left of his spine suspicious for a foreign body in the airway which was missed on radiographic evaluation at the time of empyema. On flexible fiberoptic bronchoscopy, an irretrievable foreign body was visualized in the patient's left mainstem bronchus, which was removed via rigid bronchoscopy. This is a rare case of an aspirated denture manifesting as empyema and subsequently as pneumonia. We suggest that in patients with a recurrent chest infection, the possibility of FBA must be ruled out by detailed history and careful evaluation of imaging.

Acceptance of COVID-19 Vaccine in Pakistan: A Nationwide Cross-Sectional Study.

Introduction The coronavirus disease 2019 (COVID-19) vaccine is available across various countries worldwide, with public-private partnerships ensuring all individuals are vaccinated through a phased approach. Irrespective of the geographical spread, several myths pertaining to the COVID-19 vaccine have stemmed, ultimately limiting the national administration of vaccines and rollouts. This study assessed the acceptance of the COVID-19 vaccine among the general public in Pakistan. Methods A pre-validated questionnaire was administered from January 2021 to February 2021 to assess the public attitude and acceptance of the COVID-19 vaccine. Logistic regression analyses were run to identify factors associated with the acceptance among the population. Results A total of 936 responses were elicited, where 15% perceived their risk of being infected at 20-30% with an overall 70% agreeing to be vaccinated if recommended. Multivariate analysis identified higher acceptance in the male gender, healthcare workers, and students. Of all, 66% respondents chose healthcare workers and public officials, whereas 15.6% chose scientific literature, and 12.9% chose social media as the most reliable source of COVID-19 information. Conclusion Given the relatively greater trust in healthcare providers for information regarding COVID-19, healthcare workers ought to be on the frontline for vaccine campaigns and public outreach efforts, with governmental efforts in addition to the promotion of scientific materials for population-level understanding.

National Registry of Interstitial Lung Disease from Pakistan.

Introduction Interstitial lung disease (ILD) is a heterogeneous group of over 200 parenchymal lung diseases with a myriad of etiologies. Interstitial lung disease registries from around the world show varying prevalence and incidence of these diseases. The aim of this study was to determine the epidemiology and characteristics of ILD in Pakistan. Methods This web-based registry, which is the first multicenter registry of ILD from Pakistan, recruited patients from 10 centers of five major cities between January 2016 and March 2019. Results A total of 744 patients were enrolled in the registry. The five most frequent ILDs were idiopathic pulmonary fibrosis (IPF) 34.4%, hypersensitivity pneumonitis (HP) - 17.7%, idiopathic nonspecific interstitial pneumonitis (iNSIP) - 16.8%, connective tissue disease-associated ILD (CTD-ILD) - 16.3%, and sarcoidosis - 9.1%. Conclusion Idiopathic pulmonary fibrosis is the most prevalent ILD in Pakistan, followed by HP and iNSIP. An ongoing prospective registry with longitudinal follow-up will help us further elaborate on the clinical characteristics, treatment, and survival outcome of patients with ILD.

Eosinophilic lung disease as a sequela of MSSA pneumonia.

Eosinophilic lung diseases are a rare group of lung disorders with multiple known and unknown aetiologies and the diagnosis is often challenging. We present a case of a young man who was admitted with pneumonia due to methicillin-sensitive Staphylococcus aureus and was discharged on antibiotics. He presented to the emergency department approximately 2 weeks after discharge with high-grade fever, cough and shortness of breath associated with serum and bronchoalveolar lavage eosinophilia. He was then treated with steroids with complete resolution of disease process.

Sarcoidosis presenting as vocal cord palsy: an unusual presentation and literature review.

We report the case of a 60-year-old woman, presenting with left vocal cord paralysis (VCP). Diagnostic evaluation revealed sarcoidosis to be the underlying pathology. Computed tomography (CT) scans exhibited enlarged subcarinal, pretracheal, and prevascular lymph nodes as the possible mechanism of left VCP through compression of the left recurrent laryngeal nerve. Initial treatment with oral prednisolone and azathioprine led to remission of symptoms; however, tapering of dosage led to relapse of cough without any signs of VCP. The dosage of prednisolone was further boosted, leading to complete remission. A review of literature reveals 20 cases have been reported to date, with all but one involving the left vocal cord. This is the first reported case, with a relapse of the disease without a relapse of VCP, indicating the rarity of sarcoidosis-associated VCP.