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   2021| December  | Volume 16 | Issue 5  
    Online since December 21, 2021

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Epidemiology and prevalence of connective tissue disorder–interstitial lung disease: World and Indian data
Rashmi Roongta, Arghya Chattopadhyay
December 2021, 16(5):3-9
Interstitial lung disease (ILD) is frequently seen in Connective Tissue Disorders (CTDs), most commonly in systemic sclerosis, rheumatoid arthritis, idiopathic inflammatory myositis, systemic lupus erythematosus, Sjogren's syndrome, and systemic vasculitides. Recognizing them early is of paramount importance for the timely initiation of treatment. Various modalities of detection of ILD are present of which high-resolution computed tomography is the most sensitive. This article focuses on the prevalence of ILD in various CTDs. It presents the data which have been published in different parts of the world and from India. The common subtypes of ILD found in each CTD and autoantibody association have been discussed. It briefly discusses various epidemiological factors associated with ILD and factors that predispose the patient to the development of ILD. Wherever available, morbidity and mortality data are also presented.
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Interstitial lung disease in rheumatic diseases
Sham Santhanam, Mohit Goyal
December 2021, 16(5):1-2
  1,269 262 -
Connective tissue disease-associated interstitial lung disease – A multicenter observational study from India
Sham Santhanam, Pravin Patil, Mohit Goyal, CB Mithun, Parthajit Das, Kavitha Mohanasundaram, Neeraj Jain, Vijaya Prasanna, Keerthi Talari, Himanshu Pathak, Parshant Aggarwal, Deepika Ponnuru, Vikram Raj Jain, Sharath Kumar
December 2021, 16(5):10-19
Objectives: The objectives of the study were to study the demographic, clinical, serological, and imaging characteristics of connective tissue disease-associated interstitial lung disease (CTD-ILD) patients seen at rheumatology centers across India. Methods: Adult outpatients with CTD-ILD and interstitial pneumonia with autoimmune features (IPAF), who were seen at the 12 participating rheumatology centers at least once in the preceding 6 months, were recruited and information was retrieved from their medical records. Information on demographics, duration of symptoms, diagnosis, comorbidities, autoantibodies, lung imaging, pulmonary function testing (PFT), echocardiography (ECHO), treatment, immunization, and admissions for exacerbations was obtained. Results: A total of 620 patients (505 women) were recruited. Rheumatoid arthritis (RA) was the most common CTD associated with ILD followed by systemic sclerosis and mixed connective tissue disorder. In our cohort, 372 (60%) patients had not received a diagnosis of their rheumatic disease or the ILD before seeing their rheumatologist. High-resolution computed tomography of the chest was available for 551 (88.9%) of the patients, and 327 (59.3%) had a nonspecific interstitial pneumonitis (NSIP) pattern. PFT and ECHO data were available for 437 (70.5%) and 453 (73.1%) patients, respectively. The mean forced vital capacity was 61.89% ± 14.8% of the predicted. Pulmonary hypertension (PH) was detected in 162 (35.8%) patients. The most common immunosuppressive used for lung disease was mycophenolate mofetil (54.0%) followed by azathioprine (19.5%). Medical records of these patients showed that after a diagnosis of ILD, in 79 patients, methotrexate (MTX) was changed to an alternative drug, whereas in 11 patients, MTX was added by the rheumatologist after diagnosing the CTD-ILD. There were 28 patients with IPAF. Mycophenolate and rituximab were the common drugs used in IPAF patients. Only 36 (5.8%) of the 620 patients were vaccinated with influenza as well as both the conjugate and the polysaccharide pneumococcal vaccines. Seventy-four (12.25%) patients had a history of one or more admissions for acute exacerbations or complications of ILD. Conclusion: In this study, RA-ILD was the most common CTD and NSIP was the predominant ILD pattern on high-resolution computerized tomography. More than a third of the patients had PH. In the real-life scenario, we noticed hesitancy among the treating doctors in using MTX in RA-ILD. The number of patients with complete influenza and pneumococcal vaccination was low and this issue needs to be specifically addressed. Larger prospective registry-based data are needed to understand CTD-ILD and the unmet needs in the Indian context.
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Pulmonary sarcoidosis
Deepak Talwar, Dhruv Talwar, Hariharan Iyer
December 2021, 16(5):47-57
Pulmonary sarcoidosis is an enigma due to its similarities with many other diseases in terms of presentation and hence the disease may require repeated reconsideration and revision of diagnosis. It is largely a diagnosis of exclusion. In India, exclusion of tuberculosis (TB) in every new suspected case of pulmonary sarcoidosis is a colossal task due to overlapping features and the high burden of TB. However, pulmonary sarcoidosis may be associated with extrapulmonary organ involvement, and in such cases, diagnosis can be made with greater certainty. Once diagnosed, whether to treat or not, and with what agent, and for how long are questions unsettled despite 150 years of the disease being known to mankind. This is due to a lack of randomized controlled trials as well as a dissimilar natural course of the disease among patients with a similar clinicoradiological presentations. This makes a generalization about treatment impossible and hence, individualized decisions need to be made utilizing predictors of future worsening by multidisciplinary experts. Oral steroids form the core of treatment but longterm side effects of oral steroids ultimately dictate the use of steroidsparing agents and discontinuation of steroids once the disease stabilizes and the goals of treatment are achieved. Some patients with a benign course of sarcoidosis may not require any treatment. There remains a gap in our knowledge of sarcoidosis, especially in the context of India, as it features as a common cause of interstitial lung disease and a close differential of TB. We need to intervene before the condition worsens, as this may reduce the survival significantly. However, this is not always possible and more research including longitudinal studies are needed to define and understand the course of the disease.
  1,277 178 -
Spectrum of interstitial lung disease in rheumatic diseases
Gouri Koduri, Damodar Makkuni, Ameen Jubber, Arumugam Moorthy
December 2021, 16(5):20-28
Among the diverse forms of lung involvement, interstitial lung disease (ILD) and pulmonary arterial hypertension are two important conditions in patients with rheumatic diseases that are associated with significant morbidity and mortality. The management of ILD is challenging and these challenges are associated with several mechanisms, particularly the inflammatory processes of immune cells and also fibrotic processes, which play a crucial role in disease progression, making immunosuppressive therapy less effective. However, recent advances in pharmacologic interventions have been shown to delay disease progression of these lung conditions and improve patient survival. Disease stratification and early identification of patients who are more likely to progress, and thus in need of more aggressive treatment, is important in the field of ILD. Early diagnosis, monitoring, and multidisciplinary team input are very important to improve overall outcomes. This review focuses on ILD in patients with various rheumatic diseases (rheumatoid arthritis, myositis, Sjogren' syndrome, and systemic sclerosis) and the challenges.
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Assessment and follow-up of interstitial lung disease
Devarasetti Phani Kumar
December 2021, 16(5):69-78
Patients with suspected interstitial lung disease (ILD) clinically presenting with cough and breathlessness are initially investigated with a chest radiograph and spirometry. The finding of a normal or increased forced expiratory volume in 1 second (FEV1)/forced vital capacity (FVC) ratio in the presence of reduced FVC on spirometry should alert for the presence of a restrictive defect and the need to order for complete lung function tests, including lung volumes and diffusion capacity of the lungs for carbon monoxide (DLCO) and 6-minute walk test (6MWT). High-resolution computed tomography (HRCT) is required to identify the specific patterns. To determine a more specific diagnosis, serology for connective tissue diseases, bronchoalveolar lavage, and a lung biopsy are needed. A multidisciplinary approach involving the pathologist, rheumatologist, pulmonologist, and radiologist allows the correlation of the clinical, radiologic, and pathologic findings to arrive at the right diagnosis. Screening guidelines based on expert opinion are available for idiopathic pulmonary fibrosis (IPF) and non-IPF ILDs (sarcoidosis, hypersensitivity pneumonitis, and systemic sclerosis). Follow-up monitoring for progression based on risk factors is mandatory for the early identification and management. Symptom assessment, a decline in pulmonary function tests (forced vital capacity [FVC], DLCO, and 6MWT) and worsening fibrosis on HRCT give clues for progression. The monitoring schedule in progressive fibrosing ILD for repeat pulmonary function tests is between 3 and 6 months and for HRCT is 12–18 months in the appropriate clinical context. An individualized approach to repeat pulmonary function tests and HRCT in stable ILD is required.
  1,092 153 -
Multi-disciplinary team approach in the management of connective tissue disease-interstitial lung disease: The way forward
Ben Rowlands, Puja Mehta, Harsha Gunawardena
December 2021, 16(5):116-120
Interstitial lung disease (ILD) is a heterogeneous group of diffuse parenchymal lung disorders and is a common manifestation of autoimmune connective tissue disease (CTD). In some patients, ILD is associated with a clinical spectrum, whereas in some cases it is the presenting or dominant feature. A prompt and correct diagnosis of CTD-ILD is paramount, as early recognition and intervention are essential to deliver an optimal patient pathway and clinical outcomes. This requires detailed clinical assessment of pulmonary and extra-pulmonary disease, robust autoimmune serological testing, review of radiological patterns, and appropriate treatment pathways. We describe the key components required for multidisciplinary evaluation of patients with CTD-ILD, highlighting the need for collaborative working between specialist teams to provide high-quality care.
  1,064 106 -
Imaging in connective tissue disease-associated interstitial lung disease
Praveen P G. Rao, Kushal Joshi, Sidath Liyanage, Daniel Dalili, Gouri Koduri
December 2021, 16(5):58-68
Interstitial lung disease (ILD) in rheumatic or connective tissue disease (CTD) is well recognized and presents both diagnostic and management challenges to rheumatologists. Imaging plays a central role in diagnosing and assessing treatment response. Chest radiography is easily available and is usually the first imaging modality but is neither sensitive nor specific. Lung ultrasound (LUS) is an emerging modality to detect the presence and assess the evolution of ILD. There are established correlations of B-lines and subpleural interlobular septal thickening in ILD. LUS could be a useful tool in the early screening of younger patients at risk of ILD and in minimizing cumulative radiation exposure. High-resolution computed tomography (HRCT) remains the modality of choice in characterization, assessment of disease severity/progression, and response to therapy. Interpretation of HRCT in ILD is often challenging, but an emphasis on pattern recognition in the clinical context should lead to prompt diagnosis and management. The most common ILD pattern in rheumatic disease is nonspecific interstitial pneumonitis followed by usual interstitial pneumonitis. Other less common but well-recognized patterns are organizing pneumonia, lymphocytic interstitial pneumonitis, diffuse alveolar damage, and disease-modifying antirheumatic drug-related pneumonitis. A systematic approach with a focus on the dominant pattern, zonal distribution, and additional specific features such as necrobiotic nodules (rheumatoid arthritis) and dilated esophagus (systemic sclerosis) should, in a majority of the cases, lead to the right diagnosis. Comparison with any prior imaging and follow-up imaging aids diagnostic confidence and can prognosticate disease outcome. This article aims at describing the established CTD-ILD patterns and diseases with a pictorial review and emphasizes on emerging LUS technique.
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Genetics and pathogenesis of connective tissue disease-associated interstitial lung disease
Sharath Kumar
December 2021, 16(5):29-38
Connective tissue diseases (CTD) are a broad category of diseases with autoimmune etiology and multi-system involvement. The lung seems to be involved in some form in most CTDs. Interstitial lung disease (ILD) is the prototypic lung manifestation and is seen in 2%–50% of patients with CTDs.[1] It leads to significant morbidity and mortality. Unfortunately, the ubiquitous involvement across CTDs, along with the possible benefit of treating this manifestation, has not translated into a good understanding of its pathogenesis or treatment. We must aim for a better understanding of the genetic determinants of this manifestation (to help identify those who are at risk) as well as its pathogenesis (to help identify therapeutic targets). This review aims to provide a summary of the key genetic determinants of CTD-ILD as well as the key players of the pathogenic pathway of this manifestation.
  824 119 -
Interstitial pneumonia with autoimmune features
Uma Karjigi, Balebail G Dharmanand
December 2021, 16(5):39-46
Interstitial pneumonia (IP) or interstitial lung disease (ILD) affect lung parenchyma, and if not treated will lead to irreparable lung damage. Classification of this group of diseases based on the etiology helps in diagnosing and managing these conditions. IP with autoimmune features (IPAF) is the term used for ILD with subtle signs of autoimmune disease but where there is insufficient evidence for it to be categorised as connective tissue disease. This review aims to look at the evidence available so far with regard to the concept of IPAF. It discusses the advantages and limitations of the current criteria, the epidemiology, clinical, radiological, serological features, management aspects, and prognosis of patients who fit into this particular entity.
  758 139 -
Management of connective tissue disease-associated interstitial lung disease
Keerthi Talari
December 2021, 16(5):87-91
The management of connective tissue diseases (CTD)-associated interstitial lung disease (ILD) is challenging considering its myriad presentations and variable course. Evidence to guide treatment is insufficient with very few randomized trials reported till date. This review discusses the current evidence guiding management of CTD-ILD with a specific focus on corticosteroids, cyclophosphamide, mycophenolate, azathioprine, calcineurin inhibitors, and pirfenidone. Corticosteroids (except in systemic sclerosis [SSc]-ILD) in combination with immunosuppressants form the mainstay of therapy in CTD-ILD. Cyclophosphamide and mycophenolate mofetil are backed by randomised trials-Scleroderma lung study I and II respectively for SSc-ILD, while calcineurin inhibitors with or without cyclophosphamide have emerged as a promising initial therapy in myositis associated ILD. Rituximab is probably beneficial for all forms of CTD-ILD even as randomized trial data are awaited. General measures such as lifestyle modification, pulmonary rehabilitation, vaccination, prophylaxis, supplemental oxygen, palliative care are also of paramount importance. Future research should focus on long-term efficacy, safety and head-to-head comparison of drugs, role of upfront combination therapies, supportive and palliative care.
  764 131 -
Efficacy and safety of conventional synthetic, biologic, and targeted synthetic disease-modifying antirheumatic drugs in rheumatoid arthritis-interstitial lung disease: A narrative review
Richard Conway, Luke Corcoran, Elena Nikiphorou
December 2021, 16(5):92-100
The range of therapeutic options available for rheumatoid arthritis (RA) is becoming increasingly diverse. Conventional synthetic disease-modifying antirheumatic drugs (DMARDs), biologic DMARDs, and targeted synthetic DMARDs offer the rheumatologist a far greater breadth of treatment options than before. When choosing a treatment in the individual patient, several important factors need to be considered, one of which is the safety in RA interstitial lung disease (RA-ILD). Rheumatologists frequently encounter RA patients with RA-ILD or other co-existing lung diseases. The pulmonary safety profile of our medications is difficult to ascertain and as they are generally infrequent, adverse events are rarely identified in the initial treatment trials. The concern for the safety of these treatments has largely emerged from real-world observational data and has often been based on small numbers of case studies or retrospective analyses of observational studies. The evidence has been controversial with many agents implicated both in the context of predisposing or worsening the risk of ILD and at the same time as potentially beneficial treatments in delaying the onset or progression of ILD. As a result, clear guidance on the treatment of RA-ILD is generally lacking. The purpose of this article, therefore, is to detail our current knowledge of the safety of DMARDs in RA-ILD.
  752 116 -
Lung transplantation in connective tissue disease associated interstitial lung disease
Srinivas Rajagopala, Apar Jindal
December 2021, 16(5):101-108
The science and practice of organ transplant have evolved, and a lung transplant is now a viable option that can add years and quality to life. The connective tissue diseases (CTDs) at present form a very small percentage of the indications for which lung transplants are done today. Patients with interstitial lung disease or those with respiratory failure as a result of pulmonary vascular disease may benefit from transplants if done in the right setting. The various nonpulmonary manifestations of CTDs influence the odds of the success of a lung transplant and need to be taken into account beforehand. Evaluation of the individual candidate, ensuring disease remission at the time of the transplant, anticipating the surgical and anesthetic challenges and the postsurgical intensive care-related challenges is important. Careful patient selection and meticulous preprocedure evaluation are likely to result in good outcomes.
  733 94 -
Updates in the management of connective tissue disease-associated interstitial lung disease
Chengappa Kavadichanda, G S R S N K Naidu
December 2021, 16(5):109-115
The occurrence of interstitial lung disease (ILD) in a setting of a well-defined autoimmune disease or in the presence of autoantibodies is being increasingly recognized. The traditional approach to the treatment of connective tissue disease-associated ILD (CTD-ILD) was a combination of glucocorticoids and cyclophosphamide or mycophenolate mofetil. Most of the evidence in treating these cases is limited to a few trials in scleroderma and idiopathic pulmonary fibrosis along with several observational studies in other CTDs. Insights into the pathology of these diseases and the associated lung involvement have resulted in the emergence of several immunosuppressive, antifibrotic, and small molecules as alternatives in CTD-ILD. In this review, we have attempted to summarize several emerging therapeutic options for CTD-ILD.
  696 121 -
Role of interventions in diagnosis and management of lung disorders in rheumatic diseases
Venkata Nagarjuna Maturu, Chetan Rao Vaddepally, V Pratibh Prasad
December 2021, 16(5):79-86
Pulmonary manifestations of rheumatic diseases are common and lead to significant morbidity. Rheumatic diseases can affect the lung parenchyma, the interstitium, airways, pleura as well as the mediastinum. The pulmonary manifestation can be a part of the active disease, or be because of infectious complications, drug-induced changes, or pulmonary manifestations of other organ failure. It is hence important to diagnose early and appropriately, and treat aggressively. Interventional Pulmonology, a subspecialty of pulmonary medicine helps in diagnosis of these pulmonary manifestations. Interventional pulmonology procedures also help to treat central airway obstruction. Various modalities like bronchoscopy, endobronchial ultrasonography and thoracoscopy are now available. This article focuses on these modalities, the procedures which can be performed (bronchoalveolar lavage, endobronchial biopsy, forceps and cryo transbronchial lung biopsies, transbronchial needle aspiration) and the indications for the same.
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