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 Table of Contents  
Year : 2021  |  Volume : 16  |  Issue : 5  |  Page : 3-9

Epidemiology and prevalence of connective tissue disorder–interstitial lung disease: World and Indian data

Department of Clinical Immunology and Rheumatology, Institute of Postgraduate Medical Education and Research, Kolkata, West Bengal, India

Date of Submission03-Aug-2021
Date of Acceptance11-Nov-2021
Date of Web Publication21-Dec-2021

Correspondence Address:
Dr. Arghya Chattopadhyay
Department of Clinical Immunology and Rheumatology, Institute of Postgraduate Medical Education and Research, 244, AJC Bose Road, Kolkata - 700 020, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-3698.332973

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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.

Keywords: Association, connective tissue diseases, frequency, interstitial lung diseases

How to cite this article:
Roongta R, Chattopadhyay A. Epidemiology and prevalence of connective tissue disorder–interstitial lung disease: World and Indian data. Indian J Rheumatol 2021;16, Suppl S1:3-9

How to cite this URL:
Roongta R, Chattopadhyay A. Epidemiology and prevalence of connective tissue disorder–interstitial lung disease: World and Indian data. Indian J Rheumatol [serial online] 2021 [cited 2022 May 28];16, Suppl S1:3-9. Available from:

  Connective tissue disease-related interstitial lung disease Top

Pulmonary manifestations of connective tissue disorders (CTDs) are varied, however, Interstitial lung disease (ILD) is the most challenging in terms of symptoms, management, and morbidity. ILDs are diffuse parenchymal lung disorders having specific radiological and histopathological features. ILD can be idiopathic, environmental/occupational exposure related, medication related, or CTD related. Histologically, CTD-ILD can be of several types – usual interstitial pneumonia (UIP), nonspecific IP (NSIP), respiratory bronchiolitis–ILD, desquamative interstitial pneumonia (DIP), organizing pneumonia (OP), diffuse alveolar damage (DAD), and lymphoid interstitial pneumonia (LIP).[1]

ILD may be the only or the presenting symptom of a CTD, and around 15% of patients diagnosed with NSIP are found to have an underlying CTD.[2] Around 40% of patients with CTDs have underlying ILD.[3] It is important to diagnose an underlying CTD because treatment options differ, and the prognosis of CTD-ILD is better than idiopathic pulmonary fibrosis.[4] Occasionally, there may be clinical or serological evidence of a CTD without fulfilling the CTD criteria. This entity has been termed as interstitial pneumonia with autoimmune features.[5]

The CTDs where ILDs are seen are systemic sclerosis (SSc), rheumatoid arthritis (RA), idiopathic inflammatory myositis (IIM), mixed connective tissue disease (MCTD), systemic lupus erythematosus (SLE), vasculitides, and Sjogren's syndrome (SjS). In this article, we will be discussing the epidemiology and prevalence of CTD-ILD and the world and Indian data.

  Systemic Sclerosis Top

SSc is a chronic autoimmune disease that manifests with fibrosis of the skin and internal organs and small-vessel vasculopathy. Among all the CTDs, the highest case-based mortality is that of SSc. Recently, ILD and pulmonary artery hypertension (PAH) have surpassed the scleroderma renal crisis as the leading cause of morbidity and mortality in SSc.[6]

ILD is the most common direct pulmonary manifestation of diffuse cutaneous SSc. It occurs early in the disease course. ILD usually develops in the first 5 years of disease onset, and the risk decreases after 15 years of diagnosis.[7] High-resolution computed tomography (HRCT) changes of ILD are present in up to 80-90% of patients with SSc, autopsy changes in 90-100%,[8] and 40-75% will have abnormalities in pulmonary function tests (PFTs),[9] however, only about 30%–40% develop clinically significant ILD. These symptomatic patients have a 10-year mortality rate of 40%. Patients with ILD have a 3-fold mortality rate than those without ILD.

Development and progression of ILD in SSc are associated with diffuse cutaneous SSc, African-American ethnicity,[10] older age at onset, shorter disease duration, anti-Scl-70, and anti-topoisomerase I antibodies. The presence of anti-centromere antibodies is protective.[11] HLADRB1*11 and DRB1*301 confer risk of ILD in patients positive for anti-topoisomerase 1 antibodies.[12] The most common pattern of ILD seen in SSc is NSIP.[13]

The incidence and prevalence of ILD in SSc have been poorly studied, and the data derived are mostly from cohort studies that describe the proportion of ILD in SSc. In a meta-analysis of SSc and SSC-ILD, the proportion of ILD in SSc was 35% based on 3 studies and the reported prevalence was 1.7–4.2 per 100,000 individuals in Europe depending on the method of ILD assessment. The proportion of SSc patients with ILD was reported to be between 30% and 52.3%. The incidence of SSc-ILD ranged from 0.1 to 0.4 per 100,000 individuals in Europe, and incidence-specific data for North America were not identified in this review. The mean age of SSc-ILD diagnosis was 46–61.8 years and 52.5–54.5 years in Europe and North America, respectively. Survival and mortality data in these patients were limited.[14]

Concerning Indian data, a cohort study was conducted in Western India on 110 scleroderma patients, and 75 patients had evidence of ILD on clinical presentation.[15] Chest radiographic abnormalities of ground-glass opacities, reticular reticular shadows* and honeycombing were found in 65.3% (n = 100) and 26.8% (n = 87) patients of SSc in two studies conducted in North India.[16],[17] In a study of 78 patients of SSc in South India, 21.8% had chest radiographic abnormalities.[18] The largest Indian cohort is that of a single-center retrospective study with 327 patients of scleroderma. In this cohort, 193/327 (59%) patients presented with breathlessness and 90% of those patients' breathlessness was due to ILD. PFT data for 141 patients with ILD were available that showed 20% had normal (>80%) FVC, 14.2% had mild (70%–79%) restriction, 37.6% had moderate (50%–69%), and 28.4% had severe (<50%) restriction. ILD was present in 288/327 (88.1%); in 280, it was diagnosed by HRCT and the rest by chest radiograph or clinically. Out of these 280, 47.9% had NSIP, 14.7% had UIP, and 14.1% had no specified pattern. A significant association was seen between Scl-70 positivity and the presence of ILD (P = 0.000), and a strong association of CENP antibody positivity with the absence of ILD (P = 0.000) was seen.[19]

Another study done in South India studied the frequency of pulmonary involvement in 100 SSc patients. Forty percent of subjects had reticulonodular shadows and 60% of patients had no abnormality on chest radiographs. Eighty-seven patients underwent HRCT and 85 (97.7%) had findings suggestive of ILD. Among 90 subjects with ILD, 29 were asymptomatic for respiratory complaints, in whom 12 subjects had FVC <70% and 17 subjects had desaturation >4%. Duration of disease was significantly associated with ILD and abnormal echo, but 6-min walk distance, anti-Scl-70 antibody, and anterior chest wall thickening were not. Borg score, Rodnan score, cough, and dyspnea were associated with severe pulmonary involvement (FVC <50%).[20]

Hence, the prevalence of ILD in Indian patients varied with the method of detection, ranging from 20% to 65% where chest radiographs were used and up to 97% when HRCT was used, with NSIP being the most common pattern.

  Rheumatoid Arthritis-Related Interstitial Lung Disease Top

ILD is one of the most common extra-articular manifestations of RA and a cause of increased morbidity and mortality. A variable proportion of patients (30%–76%) of RA have ILD detected by imaging modalities, but only about 5%–10% are clinically significant.[21] Although RA is more common in females, RA-ILD has a preponderance in males with a male-to-female ratio of 2:1.[22] Smoking and seropositivity have been proposed to be significant risk factors for the development of ILD. For patients with a history of smoking of more than 25 pack-years, the odds ratio for developing ILD is 3.8.[23] Unlike other CTD-ILDs, the most common pattern of ILD seen in RA is UIP (seen in up to 60%) followed by NSIP.[24] Other types of ILD include OP or bronchiolitis obliterans, acute interstitial pneumonia with DAD, DIP, and LIP.

In the Early RA Study, 1460 patients with early RA were studied and it was found that ILD was present in 52 patients (only symptomatic patients or those with clinical features were investigated for ILD), leading to an annualized incidence rate of 4.1/1000 and a 15-year cumulative incidence of 62.9/1000. ILD was present at the time of diagnosis in 25% of patients and developed in another 25% within 3 years of diagnosis. Three years was the median survival after diagnosis of ILD.[25] In another prospective study of 150 RA patients evaluated by HRCT, 19% of patients had evidence of ILD. The only clinical findings significantly associated with ILD were the presence of bibasilar crackles, FEV1/FVC, and DLCO.[26] In a retrospective look at a cohort in the US, the incidence of RA-ILD ranged from 2.7 to 3.8 cases per 100,000 people and the prevalence from 3.2 to 6.0 cases per 100,000 people across 10 years.[27]

In a retrospective Chinese cohort of 1121 RA patients who underwent HRCT, 101 (9%) patients had onset of ILD before RA diagnosis. Excluding these patients, during follow-up, 278/923 (30%) patients developed ILD. Fifty-nine percent had ILD within 5 years and 75% within 10 years of onset of RA. UIP and NSIP patterns accounted for 33% and 28% of ILD, respectively. Limited, moderate, and extensive involvement was seen in 52%, 25%, and 22% of patients. The average FVC% was 85.69%. The 3- and 5-year survival rates were 89.21% and 83.45%, respectively, for RA-ILD patients. Older patients, older age of onset of RA, males, smokers, shorter duration of RA, not being on medications, higher ESR, CRP, LDH, and RF were associated with the development of ILD. Among patients with follow-up CT scans, 83 had progressive ILD and 102 patients had stable disease.[28]

A study was done in India on 100 RA patients, all of whom underwent HRCT and spirometry, which showed abnormal HRCT and PFT in 59.3% and 51% of patients, respectively. The most common HRCT abnormality was ILD suggestive of findings in 31% of patients. Chest radiographs were abnormal in 22% of patients. Risk factors for pulmonary involvement were increasing age and the presence of rheumatoid factor. No association was found with gender, duration of disease, and the severity of the disease.[29]

In summary, clinical ILD is present in about 10% of patients with RA and subclinical in about 30%, with the most common pattern being UIP.

  Myositis-Associated Interstitial Lung Disease Top

The lung is the most common extramuscular organ involved in myositis, and the involvement can range from mild, asymptomatic ILD to fatal ILD without any muscle weakness.[30] The prevalence of ILD in myositis is reported between 20% and 78% depending on the modality of imaging used. It is associated with increased morbidity and mortality.[30],[31],[32] Black race, older age, and arthritis at onset and anti-Jo-1 antibodies are associated with ILD.[32] In 13%–38% of patients, ILD can precede muscle weakness, and there is a poor correlation between the severity of ILD and myositis activity.[30],[32],[33] A subset of rapidly progressive ILD (RP-ILD) has been described in myositis, especially those with anti-MDA5 antibodies.[34] HLADRB1*07-DQA1*02-DQB1*02 confers a reduced risk of ILD.[35] Anti-synthetase antibodies confer a higher risk of developing ILD – up to 86% of patients with anti-Jo1 antibodies can have ILD.[36],[37] Anti PL-7 and PL-12 patients have more severe ILD than anti-Jo-1 patients.[36] NSIP is the most common type seen – almost 60%–80%. Organizing pneumonia can be seen in up to 20% of biopsy-proven cases and is responsive to treatment. DAD is rare rapidly progressive and with the worst outcome with diagnosis usually made postmortem.[33],[38]

In a 10-year study conducted in South India, 87 patients with myositis were identified. ILD was the most common respiratory problem, identified in 9.2% of patients.[39] In another study, 134 patients with IIM- or CTD-associated myositis were identified and the clinicoserological associations with myositis autoantibodies were studied. ILD was present in 28 (22.6%) patients. Among patients with anti-synthetase syndrome (n = 29), 55% had ILD, and only those with anti-Jo1 antibodies (Ab) had a significant correlation with ILD (P < 0.0001). None of the 26 patients with anti-Mi2 Ab had ILD (P = 0.002). Patients with anti-Ro52 Ab (n = 45) had ILD more commonly than those without (46.6% vs. 8.8%, P = 0.0001) independent of the presence of other autoantibodies.[40] In a multicentric study from India (MyoIn cohort), ILD was significantly associated with anti-Jo1, PL-7, and Ro-52 antibodies.[41]

In summary, ILD is the most common extra-muscular manifestation of IIM following skin involvement and can be present in up to 78% of the cases. It can either precede or follow muscle involvement and is associated with higher mortality.

  Vasculitis Top

ILD in vasculitis has increasingly been recognized in the last few years first reported by Nada et al. in 2 patients with MPO-AAV.[42] Subsequently, Arimura et al. found a 43% prevalence of ILD in 46 MPO-AAV patients.[43] The prevalence of ILD is higher in MPA patients as compared to GPA.[44],[45] A slight male preponderance of 60% has been shown in those with ILD.[46] ILD has been documented to be more prevalent in Japan, but this is because of the predominance of MPO-AAV over PR3-AAV cases in Japan.[47] MPA patients with ILD present about a decade later than those without ILD (66 vs. 55 years). ILD is often present at diagnosis, and UIP is the predominant subtype (50%) followed by NSIP in less than a third of cases and DIP in 15%.[45],[47] ILD is a poor prognostic factor and a predominant cause for mortality in AAV.[44],[47] Anti-MPO antibody is more often associated with ILD (46-71%) as compared to PR3 (0-29%).[44],[48] Fibrosis is very rare in EGPA.

In a retrospective Indian study of 92 patients with AAV, a CT scan of the thorax was performed in 69 patients. Ground-glass opacities (GGOs) were seen in 24 (34.8%) patients – 15 out of 51 (29.4%) GPA patients, 1 out of 8 (12.5%) EGPA and 8 out of 10 (80%) MPA patients. However, the authors mention that they did not find any evidence of pulmonary fibrosis in their patients although pulmonary involvement was common.[49] Lung involvement was noted in 68% of AAV patients in a cohort of 105 patients with GPA, however, ILD was not specified. Thirteen patients underwent lung biopsy, of which two showed OP.[50] In another study of 31 AAV patients, 23 underwent CT scans and GGOs were found in 4 patients (all GPA).[51]

Hence, among the vasculitides, MPO-AAV has been recently shown to have ILD as a prominent lung manifestation and is usually of the UIP pattern. Further studies are required for the prevalence of ILD in MPO-AAV, especially in India.

Sjogren's syndrome

ILD is the most common pulmonary manifestation with SjS with a prevalence of around 16%–20%.[52],[53],[54] The prevalence of ILD increases with disease duration –10% in the 1st year of diagnosis, 20% after 5 years, and 47% after 15 years of diagnosis.[55] About 10%–51% of patients can have ILD diagnosed before the diagnosis of Sjogren's.[53],[54],[55] The mean age of 55–61 years is reported from the largest cohorts of SjS-ILD with a 2:8 male:female ratio. Twenty percent are smokers.[53],[54],[55],[56],[57],[58] Cough and dyspnea are reported in 40%–60% of patients and can be due to ILD, xerotrachea, or obstructive lung disease.[53],[58] Risk factors for ILD in pSS patients are found to be the presence of digital clubbing, weight loss, dry cough, older age, male gender, smoking, and a focus score ≥4 in minor salivary gland biopsy.[56],[57],[58] A higher incidence of Raynaud's phenomenonW and gastroesophageal reflux (50%) has been seen in pSS patients with ILD than those without.[53] The most common pattern is NSIP (up to 45%), followed by UIP (10%), organizing pneumonia and LIP in around 4%.[57],[58] LIP is closely associated with SjS, and LIP with nodules on HRCT should prompt a search for lymphoma.[59] Acute exacerbation of ILD is common with the UIP phenotype and has a high mortality rate of 80%.[60]

In a large retrospective cohort study of SjS in South India, out of 332 patients, 40 underwent HRCT, of which 21 were found to have ILD.[61] In a prospective observational study of 82 SjS patients in South India, 15 patients had ILD with NSIP in 9 patients, UIP in 4, and BOOP in 2.[62]

Systemic lupus erythematosus

ILD affects 1%–15% of lupus patients.[63] Trivial interstitial involvement may be seen in a third of cases. Long-standing disease (>10 years), older age, male gender, Raynaud's phenomenon, anti-U1 RNP antibodies, sclerodactyly, and abnormal nail fold capillary loops are risk factors for the development of ILD.[64],[65],[66] Men have a relative risk of 1.6 as compared to women for the development of ILD.[66] NSIP is the most commonly found subtype.[63] UIP, LIP, follicular bronchitis, and nodular lymphoid hyperplasia have also been reported. The disease course is less severe, and the prognosis of SLE-related ILD is better than other CTD-ILDs. Acute lupus pneumonitis is thought to be a precursor of ILD in one-third of the patients who survive the episode.[67],[68]

In a large Indian study of lupus reported from various centers, the incidence of ILD was 2%. However, this study was published in 1997 and is not representative of the current scenario.[69] In another cohort from Maharashtra, 3 out of 87 (3.5%) patients were found to have ILD.[70] Two out of 60 patients were found to have ILD in a study from Western India.[71]

None of the studies reported the demographic characteristics of patients with ILD or the ILD subtype. Multiple other epidemiological studies from India were found, but none reported the prevalence of ILD probably because of its rarity or low numbers of HRCT scans done in lupus patients.

ILD in lupus is probably not as uncommon as once thought, and newer studies are required to establish the same.

Mixed connective tissue diseases

MCTD is the prototype overlap CTD, and the lungs are involved in 20%–80% of the cases, although most are asymptomatic.[72] About 90% of patients with ILD show a restrictive pattern on PFT. Lung involvement is associated with increased mortality. In a Norwegian cohort of 104 patients, 36 patients (36%) had ILD. In a Hungarian cohort of 280 MCTD patients, with a mean follow-up of 13.1 years, 2 patients (0.7%) had ILD at diagnosis and 132 (47.1%) developed ILD during the disease. ILD was recurrent in 24 cases. Lung biopsy was done in 16 patients and all revealed NSIP patterns.[73] Up to 20% of patients can have lung fibrosis and 12%–100% have ground-glass opacities.[74] PAH occurs in 45% of patients. A study that compared the clinical characteristics of MCTD patients with and without ILD found that the presence of anti-Smith antibody was a significant factor for the development of ILD. On removing anti-Sm Ab from the multivariate analysis, age, dysphagia, and Raynaud's phenomenon correlated positively with the presence of ILD, whereas disease duration, the presence of arthritis, and RF were inversely associated with ILD.[75]

In an Indian cohort of 111 MCTD patients from Vellore, 43 (38.7%) had ILD with predominantly lower zone involvement. Women constituted 92.8% of the cohort, and the mean age at presentation was 39.3 years.[76] In a study from North India on 41 MCTD patients, ILD and PAH were present in 20 (57%) and 15 (44%) patients, respectively.[77] In another retrospective study, 9 out of 16 patients with MCTD had ILD.[78]

Hence, ILD in MCTD is common and usually of NSIP pattern.

  Conclusion Top

ILD has a heterogeneous presentation and is most commonly seen in SSc and RA among the CTDs. Risk factors, the pattern of ILD, and prognostic factors vary with different CTDs. The frequency of ILD detected is dependent on the patient selected and detection methods such as clinical signs, radiography, CT, or PFT. ILD may precede the onset of other CTD symptoms. Prompt detection is important for initiating therapy. However, the true incidence and prevalence studies of ILD in CTD are lacking. There is a paucity of Indian epidemiological studies as evidenced by the review. Many of the older studies have used radiography for the detection of ILD; only a few have used CT. Hence, the actual proportion of patients with ILD might be higher than proposed. This calls for epidemiological studies from different parts of the country for a better estimation of the disease burden.

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