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| ORIGINAL ARTICLE |
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| Year : 2023 | Volume
: 22
| Issue : 3 | Page : 246-251 |
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Spectrum of high-resolution computed tomography pattern in lungs in patients with connective tissue disorders
Maheswar Chaudhury, E Hari Kishore, Likhitha Lingam, Adya Kinkar Panda
Department of Radiodiagnosis, IMS and SUM Hospital, SOA Deemed to be University, Bhubaneswar, Odisha, India
| Date of Submission | 28-Jul-2021 |
| Date of Acceptance | 14-Nov-2022 |
| Date of Web Publication | 16-May-2023 |
Correspondence Address:
Maheswar Chaudhury
Staff Quarter – 8 CET Campus Kalinga Nagar, Ghatikia, Bhubaneswar - 751 029, Odisha
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/aam.aam_157_21
 Abstract | | |
Background: Connective tissue disease associated with interstitial lung disease, or CT-ILD, is a lung condition that affects a large number of patients with a connective tissue disease. Objective: Our aim in this study is to correlation between images of high-resolution computed tomography (HRCT) of different connective tissue diseases associated interstitial lung diseases (CTD-ILDs). Methods: We shall be aiming to investigate the feasibility of HRCT imaging and thereby avoid lung biopsy in such patients. Results: Rheumatoid arthritis predominantly presented with usual interstitial pneumonia (UIP) (47.8%), followed by nonspecific interstitial pneumonia (NSIP) (30.4%). Mixed connective tissue disorder predominantly presented with NSIP and UIP (42.8%), followed by organizing pneumonia (OP) (14.2%). Systemic lupus erythematosus predominantly presented with UIP (38.8%), followed by NSIP (27.7%). Sjogren's syndrome predominantly presented with lymphocytic interstitial pneumonia (40%), followed by UIP (26.6%). Scleroderma predominantly presented with UIP (45.4%), followed by NSIP (36.4%). Sarcoidosis predominantly presented with UIP (75%), followed by NSIP (25%). Dermatomyositis predominantly presented with NSIP (50%), followed by UIP and OP each (25%). Conclusion: Both clinicians and radiologists should be aware of the expected evolution of HRCT changes in a variety of CT-ILDs.
Résumé
Contexte: La maladie du tissu conjonctif associée à la maladie pulmonaire interstitielle, ou CT ILD, est une affection pulmonaire qui affecte un grand nombre de patients atteints d'une maladie du tissu conjonctif. Objectif: Notre objectif dans cette étude est de mettre en corrélation des images de tomodensitométrie à haute résolution (HRCT) de différentes maladies du tissu conjonctif associées à des maladies pulmonaires interstitielles (CTD ILDs). Méthodes: Notre objectif sera d'étudier la faisabilité de l'imagerie HRCT et d'éviter ainsi la biopsie pulmonaire chez ces patients. Résultats: La polyarthrite rhumatoïde se présentait principalement avec une pneumonie interstitielle habituelle (PUI) (47,8 %), suivie d'une pneumonie interstitielle non spécifique (NSIP) (30,4 %). Trouble mixte du tissu conjonctif présenté principalement avec NSIP et UIP (42,8 %), suivi d'une pneumonie organisée (OP) (14,2 %). Le lupus érythémateux disséminé présentait principalement une UIP (38,8 %), suivie d'une NSIP (27,7 %). Le syndrome de Sjogren présentait principalement une pneumonie interstitielle lymphocytaire (40 %), suivie d'une PUI (26,6 %). La sclérodermie se présentait principalement avec l'UIP (45,4 %), suivi du NSIP (36,4 %). La sarcoïdose se présentait principalement avec l'UIP (75 %), suivi du NSIP (25 %). La dermatomyosite se présentait principalement avec NSIP (50 %), suivi par UIP et OP chacun (25 %). Conclusion: Les cliniciens et les radiologues doivent être conscients de l'évolution attendue des changements HRCT dans une variété d'ILD CT.
Mots-clés: Tissu conjonctif, CT ILDs, tomodensitométrie haute résolution, poumon interstitiel
Keywords: Connective tissue, CT-ILDs, high-resolution computed tomography, interstitial lung
How to cite this article:
Chaudhury M, Kishore E H, Lingam L, Panda AK. Spectrum of high-resolution computed tomography pattern in lungs in patients with connective tissue disorders. Ann Afr Med 2023;22:246-51 |
How to cite this URL:
Chaudhury M, Kishore E H, Lingam L, Panda AK. Spectrum of high-resolution computed tomography pattern in lungs in patients with connective tissue disorders. Ann Afr Med [serial online] 2023 [cited 2023 Sep 27];22:246-51. Available from: https://www.annalsafrmed.org/text.asp?2023/22/3/246/377178 |
| Introduction | |  |
Connective tissue disease refers to a group of disorders involving the protein-rich tissue that supports organs and other parts of the body. Examples of connective tissue are fat, bone, and cartilage. These disorders often involve the joints, muscles, and skin, but they can also involve other organ systems, including the eyes, heart, lungs, kidneys, gastrointestinal tract, and blood vessels. Connective tissue disease associated with interstitial lung disease, or CT-ILD, is a lung condition that affects a large number of patients with a connective tissue disease.[1] Examples of connective tissue diseases-also known as rheumatologic, collagen vascular, or autoimmune diseases-include scleroderma, sarcoidosis, rheumatoid arthritis (RAs), Sjogren's syndrome, systemic lupus erythematosus (SLE), polymyositis, dermatomyositis, and mixed connective tissue disease.[2] Interstitial lung disease (ILD) is a group of diffuse parenchymal lung diseases affecting the pulmonary interstitium.[3]
It comprises three subdivisions as follows:
- Axial (bronchovascular): Situated in peribronchovascular location running from hila to secondary pulmonary lobules
- Parenchymal (acinar): Situated in alveolar walls
- Subpleural: Situated between the pleura and lung parenchyma, continuous with the perivenous interstitial space and interlobular septa.
High-resolution computed tomography (HRCT) is the most accurate, noninvasive, cross-section imaging modality for the diagnosis and follow-up monitoring of ILD. Study shall be done to check the basic HRCT patterns associated with ILD and correlation of HRCT patterns with clinical data in the differential diagnosis of ILD.[4] The current guidelines recommend lung biopsy as gold standard for identification of connective tissue associated ILDs. However, lung biopsy is associated with certain limitations.[5],[6],[7],[8] Our aim here is to analysis and to find correlation between images of HRCT of different connective tissue diseases associated interstitial lung diseases (CTD-ILDs), we shall be aiming to investigate the feasibility of HRCT imaging and thereby avoid lung biopsy in such patients [Table 1].[9] | Table 1: Classification of interstitial lung diseases and their classical patterns
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| Methods | | |
A total 107 number of patients with diagnosed connective tissue disorders with pulmonary symptoms, attending pulmonary medicine ward and outpatient department of tertiary care teaching hospital, between the December 2018 and May 2020. The diagnosis of ILD pattern was made with combined clinical and radiological findings were selected. Seven patients were excluded from the study due to unstable hemodynamic state. A full clinical examination was conducted on all the patients included in the study. Clinical history and physical examination findings were recorded with particular attention to present or previous history of cough, fever, shortness of breath (dyspnea), initially while exercising and eventually while at rest, fatigue, dizziness or fainting spells (syncope), chest pressure or pain, swelling (edema) in ankles, legs and eventually in abdomen (ascites), bluish color to lips and skin (cyanosis), heart palpitations, history of smoking, alcohol, ATT medication, and drug abuse.
| Results | | |
Out of 100 suitable patients for study 59 were male 41 were female, out of which 23 were diagnosed RAs (14 ‒ male), (9 ‒ female); 21 were diagnosed with mixed connective tissue disorder (MCTD) (10 ‒ male), (11 ‒ female); 18 were diagnosed with SLE (16 ‒ male), (2 ‒ female); 15 were diagnosed with Sjogren's (10 ‒ male), (5 ‒ female); 11 were diagnosed with scleroderma (4 ‒ male), (7 ‒ female); eight were diagnosed with sarcoidosis (2 ‒ male), (6 ‒ female); 23 were diagnosed with dermatomyositis (3 ‒ male), (1 ‒ female) [Figure 1]. Out of total patients, 30 patients are in the age group of 30–50 years, whereas 70 patients are in the age group of 50–70 years age group. While taking history and performing clinical examination, out of 100 sampled patients 60% presented with exertional dyspnea, 70% patients had cough with expectoration, 50% patients had cough without expectoration, 20% patients presented with fever, 10% with hemoptysis; systemic manifestations like cutaneous manifestations were presented in 80% patients, 80% patients with arthropathy, 75% were hypertensive, and 50% patients had clubbing. However, there is no discrete correlation between clinical features and HRCT findings of these connective tissue disorders. | Figure 1: Demographics of connective tissue disorders. MCTD = Mixed connective tissue disorder, SLE = Systemic lupus erythematosus
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Among 23 patients diagnosed with RAs, 74%, i.e., 17 patients had reticular opacities; 95.5%, i.e., 22 patients had nodular opacities; 100%, i.e., 23 patients had ground glass opacities; 22%, i.e., five patients had honey combing; 95.5%, i.e., 22 patients had pleural effusion/thickening; 100%, i.e., 23 patients had bronchiectasis; 78%, i.e., 18 patients had fibrosis;
After assessing the HRCT findings their pattern, distribution and extension following spectrum of ILD pattern were diagnosed, 30.4%, i.e., seven patients were diagnosed with nonspecific interstitial pneumonia (NSIP), 47.8%, i.e., 11 patients were diagnosed with usual interstitial pneumonia (UIP), 8.6%, i.e., two patients were diagnosed with organizing pneumonia (OP), 30.4%, i.e., seven patients were diagnosed with lymphocytic interstitial pneumonia (LIP), and 4.3%, i.e., one patient were diagnosed with diffuse alveolar damage (DAD) [Table 2] and [Figure 2]. | Table 2: Spectrum of high-resolution computed tomography interstitial lung disease patterns in respective connective tissue disorders
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 | Figure 2: Spectrum of HRCT ILD patterns in respective connective tissue disorders. HRCT = High-resolution computed tomography, ILD = Interstitial lung disease, NSIP = Nonspecific interstitial pneumonia, UIP = Usual interstitial pneumonia, OP = Organizing pneumonia, LIP = Lymphocytic interstitial pneumonia, DAD = Diffuse alveolar damage, MCTD = Mixed connective tissue disorder, SLE = Systemic lupus erythematosus
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Among 21 patients diagnosed with MCTD, 43%, i.e., nine patients had reticular opacities; 71.5%, i.e., 15 patients had nodular opacities; 48%, i.e., 10 patients had ground-glass opacities; 38%, i.e., eight patients had honey combing; 5%, i.e., one patient had cystic changes; 86%, i.e., 18 patients had pleural effusion/thickening; 38%, i.e., eight patients had mosaic pattern, 71.5%, i.e., 15 patients had consolidation; 48%, i.e., 10 patients had bronchiectasis; 86%, i.e., 18 patients had fibrosis; 96%, i.e., 20 patients had lymphadenopathy [Table 3].
After assessing the HRCT findings their pattern, distribution and extension following spectrum of ILD pattern were diagnosed, 42.8%, i.e., nine patients were diagnosed with NSIP, 42.8%, i.e., nine patients were diagnosed with UIP, 14.2%, i.e., three patients were diagnosed with OP [Table 3] and [Figure 3]. | Figure 3: Incidence of HRCT imaging findings in different connective disease
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Among 18 patients diagnosed with SLE, 55.5%, i.e., 10 patients had reticular opacities; 53.5%, i.e., eight patients had nodular opacities; 55.5%, i.e., 10 patients had ground-glass opacities; 50%, i.e., nine patients had honey combing; 100%, i.e., 18 patients had pleural effusion/thickening; 23%, i.e., five patients had mosaic pattern, 55.5%, i.e., 10 patients had consolidation; 94.5%, i.e., 17 patients had bronchiectasis; 94.5%, i.e., 17 patients had fibrosis.
After assessing the HRCT findings their pattern, distribution and extension following spectrum of ILD pattern were diagnosed, 27.7%, i.e., five patients were diagnosed with NSIP, 38.8%, i.e., seven patients were diagnosed with UIP, 16.6%, i.e., three patients were diagnosed with OP, and 16.6%, i.e., three patients were diagnosed with DAD [Table 3] and [Figure 3]. Among 15 patients diagnosed with Sjogren's syndrome, 100%, i.e., 15 patients had nodular opacities; 93.5%, i.e., 14 patients had ground-glass opacities; 100%, i.e., eight patients had mosaic pattern, 53.5%, i.e., eight patients had consolidation; 100%, i.e., 15 patients had bronchiectasis; 93.5%, i.e., 14 patients had lymphadenopathy.
After assessing the HRCT findings their pattern, distribution and extension following spectrum of ILD pattern were diagnosed, 26.6%, i.e., four patients were diagnosed with NSIP, 13.3%, i.e., two patients were diagnosed with UIP, 6.6%, i.e., one patient were diagnosed with OP, 40%, i.e., six patients were diagnosed with LIP, and 13.3%, i.e., two patients were diagnosed with DAD [Table 3] and [Figure 3].
Among 11 patients diagnosed with scleroderma, 27.3%, i.e., three patients had reticular opacities; 9%, i.e., one patient had nodular opacities; 67%, i.e., seven patients had ground-glass opacities; 67%, i.e., seven patients had honey combing; 67%, i.e., seven patient had cystic changes; 27.5%, i.e., three patients had consolidation; 45.5%, i.e., five patients had bronchiectasis; 36.5%, i.e., four patients had fibrosis; 100%, i.e., 11 patients had lymphadenopathy. After assessing the HRCT findings their pattern, distribution and extension following spectrum of ILD pattern were diagnosed, 36.4%, i.e., four patients were diagnosed with NSIP, 45.4%, i.e., five patients were diagnosed with UIP, 9.1%, i.e., one patient were diagnosed with OP, and 9.1%, i.e., one patient were diagnosed with DAD [Table 3] and [Figure 3].
Among eight patients diagnosed with sarcoidosis, 25%, i.e., two patients had reticular opacities; 87.5%, i.e., seven patients had nodular opacities; 25%, i.e., two patients had ground-glass opacities; 37.5%, i.e., three patients had honey combing; 12.5%, i.e., one patient had cystic changes; 25%, i.e., two patients had pleural effusion/thickening; 12.5%, i.e., one patients had consolidation; 50%, i.e., four patients had bronchiectasis; 50%, i.e., four patients had fibrosis; 100%, i.e., eight patients had lymphadenopathy. After assessing the HRCT findings their pattern, distribution and extension following spectrum of ILD pattern were diagnosed, 75%, i.e., six patients were diagnosed with UIP, and 25%, i.e., two patients were diagnosed with LIP [Table 3] and [Figure 3].
Among four patients diagnosed with dermatomyositis, 75%, i.e., three patients had reticular opacities; 25%, i.e., one patient had nodular opacities; 25%, i.e., one patient had honey combing; 25%, i.e., one patient had mosaic pattern, 75%, i.e., three patients had consolidation; 25%, i.e., one patient had bronchiectasis.
After assessing the HRCT findings their pattern, distribution and extension following spectrum of ILD pattern were diagnosed, 50%, i.e., two patients were diagnosed with NSIP, 25%, i.e., one patient were diagnosed with UIP, 25%, i.e., one patient were diagnosed with OP [Table 3] and [Figure 3].
| Discussion | | |
Despite the global distribution of ILDs with a progressive-fibrosing phenotype, their incidence and prevalence are not well defined. This may be related to a number of reasons, potentially to the heterogeneous nature of the etiology, the complexity of diagnosis (and subsequent recording of cases), the low numbers of patients diagnosed, and the methods employed to retrospectively analyses patient databases. ILDs that may present a progressive-fibrosing phenotype appear to be more common in older adults and are associated with a complex network of environmental and genetic factors. Few of the available epidemiological data are from low- or middle-income countries; this may be attributable to factors such as the availability of HRCT and access to health-care professionals with the expertise needed to differentiate between ILDs. Given the negatively associated prognosis, further epidemiological studies are warranted to help identify ILD patients who may develop a progressive-fibrosing phenotype and enable effective clinical management. Using hospital statistics from a national secondary care dataset in the UK from 1997 to 2008, they estimated in-hospital mortality following surgical lung biopsy to be 1.7%, 30-day mortality to be 2.4% and 90-day mortality to be 3.9%. Advanced age, rapid deterioration of underlying lung disease, requirement for mechanical ventilation, and immunosuppressed conditions were reported as risk factors of surgical lung biopsy.[10] Complications of surgical lung biopsy were defined as documented postoperative infections such as pneumonia, empyema, or other nosocomial infection; prolonged air leakage (5 days or more); pleurodesis or reinsertion of a chest tube; respiratory failure requiring mechanical ventilation more than 72 h or documented acute exacerbation (AE); hospital readmission due to surgical complication after hospital discharge.[11],[12],[13],[14] AE was defined by the criteria of an increase in dyspnea within 30 days, newly developing opacities on chest radiography or HRCT, decrease in PaO2 of more than 10 mmHg under similar conditions, and absence of apparent infectious agents and heart disease.[15] HRCT lung is gaining attention in the precise diagnosis of CT-ILD, without the above complications and it is simple, quick, reproducible, and cost-effective.[16] Studies pulmonary manifestations in HRCT in connective tissue disorders with various CTDs having respiratory symptoms were evaluated for respiratory system involvement.[17] ILD was the most common (38.5%) presentation of CTDs.[18] It was observed in nearly three-fourths of the cases with scleroderma, followed by RA cases (44.5%).[19] HRCT revealed reticulonodular lesions, pleural effusion (mainly in patients with RA and SLE), honeycomb appearance (mainly in patients with RA and scleroderma), and bronchiectasis (mainly in patients with RA).[20]
| Conclusion | | |
HRCT of the lung is a key component of the multidisciplinary approach to diagnosis in connective tissue disorder-associated interstitial lung disease (CT-ILD). HRCT also plays an important role in the follow-up of patients with established CT-ILD. In this respect, serial HRCT examinations may provide valuable information that cannot be determined from clinical history and other diagnostic tests, such as pulmonary function tests. Important roles of HRCT in this context include assisting in determining prognosis, monitoring for the efficacy of treatment, detecting the progression of disease or complications, and evaluating patients with worsening or acute symptoms. Both clinicians and radiologists should be aware of the expected evolution of HRCT changes in a variety of CT-ILDs.
Financial support and sponsorship
This study was financially supported by the Department of Radiodiagnosis, IMS and SUM Hospital, Bhubaneswar, Odisha, India.
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]
[Table 1], [Table 2], [Table 3]
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