AB0425 CLINICAL AND PATHOLOGICAL FEATURES OF BREAST CANCER IN PATIENTS WITH SYSTEMIC SCLEROSIS: PRELIMINARY DATA FROM THE SCLERO-BREAST STUDY

Background:Systemic Sclerosis (SSc) is a rare and life-threatening connective tissue disease characterized by vascular dysfunction, specific autoimmune abnormalities and fibrosis of the skin and internal organs. Previous studies have shown a 1.5-fold increase in cancer risk in SSc patients compared with the general population, including breast cancer (BC). The relationship between BC and SSc has long been discussed but past research has been contradictory and inconclusive on this topic.Objectives:The aim of our project was to analyze clinical and pathological characteristics of BC developed by SSc subjects and possible correlations with scleroderma features. Here we present the preliminary data from the Sclero-Breast study.Methods:Our observational retrospective multicenter study enrolled 33 SSc women with a personal history of BC identified at two Rheumatology/SSc Units in the north of Italy between January 2017 and December 2019 (lc/dcSSc 23/9, 1 unknown; mean age at SSc onset 57 years, range 32-73). All patients underwent general and instrumental assessment: smoking habits; presence of skin ulcers, calcinosis, teleangectasia; presence of gastro-intestinal and kidney involvement; interstitial lung disease (at HR-CT); pulmonary function tests; ECG abnormalities; echocardiographic assessment of pulmonary arterial hypertension (PAH); videocapillaroscopic pattern; autoantibody profile; exposure to immunosuppressive and vasoactive therapies; status at last follow-up evaluation and cause of death. Clinical and pathological characteristics of BC were also evaluated: age at diagnosis; menopausal status; histotype; hormone receptor status; MIB1, HER2 expression; clinical and pathological stage at diagnosis; metastatic sites; type of loco-regional treatment (surgery and radiotherapy); type of systemic treatment (neoadjuvant/adjuvant chemotherapy and endocrine treatment); other cancers and time from diagnosis of the first disorder to the second one.Results:A total of 54.5% of subjects developed BC before SSc (median interval of 5 years), whereas 45.5% of patients developed BC after SSc (median delay of 8 years). 54.5% of patients showed interstitial lung disease and the cause of death of the 6 deceased subjects was PAH. A significant association (p<0.05) was observed between the use of immunosuppressive therapy and diffuse skin extension, negative ACA, positive Anti-Scl-70 and interstitial lung disease, but not with BC status. 93.1% of patients were diagnosed with an early-stage tumor, 70.8% of invasive carcinomas with a low MIB-1, 8.3% with a tubular histotype, while 42.8% presented with a Luminal A-like tumor. 66.6% underwent breast conserving surgery and 55.5% RT after surgery. 40% of patients developed interstitial lung disease after RT and 20% dcSSc.Conclusion:According to our preliminary data, SSc patients developed BC at good prognosis, suggesting a de-escalation strategy of cancer therapies. On these grounds, a proper screening is mandatory in order to allow for early cancer detection in SSc patients. Further investigations on larger numbers of patients are needed. First of all, they would further clarify the intriguing relationship between BC and SSc. Secondly, they would help to explore the common biological and molecular pathways at the basis of these two disorders, with the aim to improve BC diagnosis and prognosis and to personalize oncological targeted treatments in this subset of fragile patients.Disclosure of Interests:None declared

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A study of respiratory system involvement in autoimmune connective tissue diseases: a marker of morbidity

International Journal of Research in Dermatology ◽

10.18203/issn.2455-4529.intjresdermatol20202660 ◽

2020 ◽

Vol 6 (4) ◽

pp. 532

Author(s):

Neha T. Solanki ◽

Sahana P. Raju ◽

Deepmala Budhrani ◽

Bharti K. Patel

Keyword(s):

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Connective Tissue ◽

Pulmonary Function ◽

Lung Disease ◽

Pulmonary Function Tests ◽

Tertiary Care ◽

Connective Tissue Diseases ◽

Cutaneous Manifestations ◽

Respiratory Involvement

Background: The auto-immune connective tissue diseases (AICTD) are polygenic clinical disorders having heterogeneous overlapping clinical features. Certain features like autoimmunity, vascular abnormalities, arthritis/arthralgia and cutaneous manifestations are common to them. Lung involvement can present in AICTDs in form of: pleurisy, acute/ chronic pneumonitis, pulmonary artery hypertension (PAH), shrinking lung syndrome, diffuse alveolar damage, pulmonary embolism (PE), bronchiolitis obliterans organizing pneumonia, pulmonary infections, cardiogenic pulmonary edema, etc. High-resolution computed tomography (HRCT) plays an important role in identifying patients with respiratory involvement. Pulmonary function tests are a sensitive tool detecting interstitial lung disease.Methods: The present study is an observational study carried out on 170 patients of AICTD in department of Dermatology, Venereology and Leprosy at a tertiary care centre during a period of 2 years from October 2017 to August 2019. Detailed history, examination and relevant investigations like chest X-ray, pulmonary function test (PFT), HRCT thorax were done as indicated.Results: The overall incidence of respiratory involvement was 56.7% with maximum involvement in systemic sclerosis cases (82.8% of cases). 45.7% of patients of systemic lupus erythematosus had respiratory involvement, most common being pleural effusion in 11.5%. Impaired PFT’s were seen in 82.8% cases of systemic sclerosis (SSc) and all cases of UCTD. Interstitial lung disease was seen in 34.7% and 25% cases of SSc and DM respectively. PAH was found in 15.2% cases of SSc and 9.8% cases of mixed connective tissue diseases.Conclusions: AICTD are multisystem disorders in which pulmonary involvement can be an important cause of morbidity to the patient and early detection is necessary for prevention of long-term respiratory complications.

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Increased levels of HE4 (WFDC2) in systemic sclerosis: a novel biomarker reflecting interstitial lung disease severity?

Therapeutic Advances in Chronic Disease ◽

10.1177/2040622320956420 ◽

2020 ◽

Vol 11 ◽

pp. 204062232095642

Author(s):

Mingxia Zhang ◽

Liyun Zhang ◽

Linning E ◽

Ke Xu ◽

Xu Fei Wang ◽

...

Keyword(s):

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Pulmonary Function Tests ◽

Lavage Fluid ◽

Ct Scans ◽

Function Parameter ◽

Enzyme Linked Immunosorbent Assay ◽

Healthy Controls ◽

High Resolution Computed Tomography

Background: Human epididymis protein 4 (HE4, also known as WFDC-2) has been implicated in fibrotic disorders pathobiology. We tested the hypothesis that HE4 may be used as a candidate biomarker for systemic sclerosis (SSc)-related interstitial lung disease (SSc-ILD). Methods: A total of 169 consecutive SSc patients and 169 age-and sex-matched healthy controls were enrolled and blood samples were collected. Pulmonary function tests (PFTs) and paired lavage was performed on 169 patients and 37 healthy controls. All patients were classified as having SSc-no ILD or SSc-ILD, based on high-resolution computed tomography (CT) scans of the chest, and a semiquantitative grade of ILD extent was evaluated through CT scans (grade 1, 0–25%; grade 2, 26–50%; grade 3, 51–75%; grade 4, 76–100%). Serum and bronchoalveolar lavage fluid (BALF) HE4 levels were measured by enzyme-linked immunosorbent assay. Results: Serum HE4 levels were higher in SSc patients [median (interquartile range), 139.4 (85.9–181.8) pmol/l] compared with healthy controls [39.5 (24.3–54.2) pmol/l, p < 0.001] and were higher in patients with SSc-ILD [172.1 (94.8–263.3) pmol/l] than in those with SSc-no ILD [97.4 (85.5–156.5) pmol/l, p < 0.001]. This observation was replicated in the BALF samples. Corresponding values were 510.8 (144.6–1013.8) pmol/l for SSc cohort, 754.4 (299–1060) pmol/l for SSc-ILD, 555.1 (203.7–776.2) pmol/l for SSc-no ILD, and 238.7 (97.7–397.6) pmol/l for controls. The semiquantitative grade of ILD on CT scan was significantly proportional to the HE4 levels and the lung function parameter (i.e., FVC) had a negative correlation with the HE4 levels. Conclusion: This is the first study to demonstrate the potential clinical utility of blood and BALF HE4 as a biomarker for SSc-ILD. Future prospective validation studies are warranted.

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Pulmonary function tests as outcomes for systemic sclerosis interstitial lung disease

European Respiratory Review ◽

10.1183/16000617.0102-2017 ◽

2018 ◽

Vol 27 (148) ◽

pp. 170102 ◽

Cited By ~ 15

Author(s):

Melissa Caron ◽

Sabrina Hoa ◽

Marie Hudson ◽

Kevin Schwartzman ◽

Russell Steele

Keyword(s):

Systematic Review ◽

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Pulmonary Function ◽

Lung Disease ◽

Validation Studies ◽

Pulmonary Function Tests ◽

Outcome Studies ◽

Surrogate Markers ◽

Function Tests

Interstitial lung disease (ILD) is the leading cause of morbidity and mortality in systemic sclerosis (SSc). We performed a systematic review to characterise the use and validation of pulmonary function tests (PFTs) as surrogate markers for systemic sclerosis-associated interstitial lung disease (SSc-ILD) progression.Five electronic databases were searched to identify all relevant studies. Included studies either used at least one PFT measure as a longitudinal outcome for SSc-ILD progression (i.e. outcome studies) and/or reported at least one classical measure of validity for the PFTs in SSc-ILD (i.e. validation studies).This systematic review included 169 outcome studies and 50 validation studies. Diffusing capacity of the lung for carbon monoxide (DLCO) was cumulatively the most commonly used outcome until 2010 when it was surpassed by forced vital capacity (FVC). FVC (% predicted) was the primary endpoint in 70.4% of studies, compared to 11.3% for % predicted DLCO. Only five studies specifically aimed to validate the PFTs: two concluded that DLCO was the best measure of SSc-ILD extent, while the others did not favour any PFT. These studies also showed respectable validity measures for total lung capacity (TLC).Despite the current preference for FVC, available evidence suggests that DLCO and TLC should not yet be discounted as potential surrogate markers for SSc-ILD progression.

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Effect of Bosentan on Systemic Sclerosis-associated Interstitial Lung Disease Ineligible for Cyclophosphamide Therapy: A Prospective Open-label Study

The Journal of Rheumatology ◽

10.3899/jrheum.110499 ◽

2011 ◽

Vol 38 (10) ◽

pp. 2186-2192 ◽

Cited By ~ 14

Author(s):

YOSHIAKI FURUYA ◽

MASATAKA KUWANA

Keyword(s):

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Pulmonary Function ◽

Lung Disease ◽

Pulmonary Function Tests ◽

High Dose ◽

Open Label ◽

High Resolution Computed Tomography ◽

Open Label Study ◽

Label Study

Objective.To evaluate the clinical benefits of the endothelin receptor antagonist bosentan on interstitial lung disease (ILD) in patients with systemic sclerosis (SSc) who are ineligible for cyclophosphamide (CYC) therapy.Methods.In this prospective open-label study, 9 patients with SSc and ILD received bosentan for 24 months. The main reasons for avoiding CYC included severely impaired lung function, long disease duration, and relapse after CYC treatment. Pulmonary function tests and Doppler echocardiograms were evaluated every 6 months, and high-resolution computed tomography (HRCT) was performed every 12 months. For an extended survival analysis, 17 historical controls who met the inclusion criteria at referral and had not used any immunosuppressive or antifibrotic agents thereafter were selected from the SSc database.Results.Two patients did not finish the study; one developed vasculitis requiring high-dose corticosteroids and another died of bacterial pneumonia. The remaining 7 patients tolerated bosentan and completed the study period. There were trends toward mildly reduced forced vital capacity, total lung capacity, and diffusing capacity for carbon monoxide over time. Two patients developed pulmonary hypertension during the 24-month period. HRCT scores for ground-glass opacity, pulmonary fibrosis, and honeycomb cysts gradually increased. In the extended study, there was no difference in cumulative survival rate between the bosentan-treated and historical control groups.Conclusion.The gradual worsening of pulmonary function and HRCT findings in patients treated with bosentan was consistent with the natural course of SSc-associated ILD. This study does not support the use of bosentan for SSc-associated ILD even when CYC treatment is inadvisable.

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Pulmonary Function Tests and 5-Year Survival in Patients Systemic Sclerosis and Interstitial Lung Disease (ILD)

CHEST Journal ◽

10.1378/chest.1386405 ◽

2012 ◽

Vol 142 (4) ◽

pp. 433A

Author(s):

Maria Otaola ◽

Silvia Quadrelli ◽

Gabriela Tabaj ◽

Raquel Aguirre ◽

Luciana Molinari ◽

...

Keyword(s):

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Pulmonary Function ◽

Lung Disease ◽

Pulmonary Function Tests ◽

Function Tests

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FRI0454 UNDER DETECTION OF INTERSTITIAL LUNG DISEASE IN JUVENILE SYSTEMIC SCLEROSIS (JSSC) UTILIZING PULMONARY FUNCTION TESTS. RESULTS FROM THE JUVENILE SCLERODERMA INCEPTION COHORT

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.1788 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 824.1-824

Author(s):

I. Foeldvari ◽

B. Hinrichs ◽

K. Torok ◽

M. J. Santos ◽

O. Kasapcopur ◽

...

Keyword(s):

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Pulmonary Function ◽

Lung Disease ◽

Pulmonary Function Tests ◽

Disease Process ◽

Systemic Scleroderma ◽

Inception Cohort ◽

Research Support ◽

Juvenile Systemic Sclerosis

Background:Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence in around 3 in a million children. Pulmonary involvement in jSSc occurs in approximately 40 % in the inception cohort. Traditionally in jSSc, pulmonary function testing (PFT) with FVC and DLCO are used for screening and computed tomography (HRCT) was more reserved for those with abnormal PFTs. More recently, it has become apparent that PFTs might not be sensitive enough for detecting ILD in children.Objectives:Utilizing a prospective international juvenile systemic scleroderma cohort (JSScC) [2], to determine if pulmonary screening with FVC and DLCO is sufficient enough to assess the presence of interstitial lung disease in comparison to CT evaluation.Methods:The international juvenile systemic scleroderma cohort database was queried for available patients with recorded PFT parameters and HRCT performed to determine sensitivity of PFTs detecting disease process.Results:Of 129 patients in the jSScC, 67 patients had both CT imaging and an FVC reading from PFTs for direct comparison. DLCO readings were also captured but not in as many patients with tandem HRCT (n =55 DCLO and HRCT scan). Therefore, initial analyses focused on the sensitivity, specificity and accuracy of the FVC value from the PFTs to capture the diagnosis of interstitial lung disease as determined by HRCT.Overall, 49% of the patients had ILD determined by HRCT, with 60% of patients having normal FVC (>80%) with positive HRCT findings, and 24% of patients having normal DLCO (> 80%) with positive HRCT findings. Fourteen percent (n = 3/21) of patients with both FVC and DLCO values within the normal range had a positive HRCT finding.Conclusion:The sensitivity of the FVC in the JSScC cohort in detecting ILD was only 39%. Relying on PFTs alone for screening for ILD in juvenile systemic sclerosis would have missed the detection of ILD in almost 2/3 of the sample cohort, supporting the use of HRCT for detection of ILD in children with SSc. In addition, the cut off utilized, of less than 80% of predicted FVC or DLCO could be too low for pediatric patients to exclude beginning ILD. This pilot data needs confirmation in a larger patient population.Supported by the “Joachim Herz Stiftung”Disclosure of Interests:Ivan Foeldvari Consultant of: Novartis, Bernd Hinrichs: None declared, Kathryn Torok: None declared, Maria Jose Santos Speakers bureau: Novartis and Pfizer, Ozgur Kasapcopur: None declared, Amra Adrovic: None declared, Valda Stanevicha: None declared, Flávio R. Sztajnbok: None declared, Maria T. Terreri: None declared, Ana Paula Sakamoto: None declared, Ekaterina Alexeeva Grant/research support from: Roche, Pfizer, Centocor, Novartis, Speakers bureau: Roche, Novartis, Pfizer., Jordi Anton Grant/research support from: grants from Pfizer, abbvie, Novartis, Sobi. Gebro, Roche, Novimmune, Sanofi, Lilly, Amgen, Grant/research support from: Pfizer, abbvie, Novartis, Sobi. Gebro, Roche, Novimmune, Sanofi, Lilly, Amgen, Consultant of: Novartis, Sobi, Pfizer, abbvie, Consultant of: Novartis, Sobi, Pfizer, abbvie, Speakers bureau: abbvie, Pfizer, Roche, Novartis, Sobi, Gebro, Speakers bureau: abbvie, Pfizer, Roche, Novartis, Sobi, Gebro, Maria Katsikas: None declared, Vanessa Smith Grant/research support from: The affiliated company received grants from Research Foundation - Flanders (FWO), Belgian Fund for Scientific Research in Rheumatic diseases (FWRO), Boehringer Ingelheim Pharma GmbH & Co and Janssen-Cilag NV, Consultant of: Boehringer-Ingelheim Pharma GmbH & Co, Speakers bureau: Actelion Pharmaceuticals Ltd, Boehringer-Ingelheim Pharma GmbH & Co and UCB Biopharma Sprl, Rolando Cimaz: None declared, Mikhail Kostik: None declared, Simone Appenzeller: None declared, Mahesh Janarthanan: None declared, Monika Moll: None declared, Dana Nemcova: None declared, Dieneke Schonenberg: None declared, Cristina Battagliotti: None declared, Lillemor Berntson Consultant of: paid by Abbvie as a consultant, Speakers bureau: paid by Abbvie for giving speaches about JIA, Blanca Bica: None declared, Juergen Brunner Grant/research support from: Pfizer, Novartis, Consultant of: Pfizer, Novartis, Abbvie, Roche, BMS, Speakers bureau: Pfizer, Novartis, Abbvie, Roche, BMS, Patricia Costa Reis: None declared, Despina Eleftheriou: None declared, Liora Harel: None declared, Gerd Horneff Grant/research support from: AbbVie, Chugai, Merck Sharp & Dohme, Novartis, Pfizer, Roche, Speakers bureau: AbbVie, Bayer, Chugai, Merck Sharp & Dohme, Novartis, Pfizer, Roche, Dragana Lazarevic: None declared, Kirsten Minden Consultant of: GlaxoSmithKline, Sanofi, Speakers bureau: Roche, Susan Nielsen: None declared, Farzana Nuruzzaman: None declared, Anjali Patwardhan: None declared, Yosef Uziel: None declared, Nicola Helmus: None declared

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Clinical and Pathological Features of Breast Cancer in Systemic Sclerosis: Results from the Sclero-Breast Study

Journal of Personalized Medicine ◽

10.3390/jpm11060580 ◽

2021 ◽

Vol 11 (6) ◽

pp. 580

Author(s):

Angela Toss ◽

Amelia Spinella ◽

Chrystel Isca ◽

Caterina Vacchi ◽

Guido Ficarra ◽

...

Keyword(s):

Breast Cancer ◽

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Early Stage ◽

Fold Increase ◽

Breast Conserving Surgery ◽

Pathological Features ◽

Luminal A ◽

Clinical And Pathological Features

Systemic Sclerosis (SSc) is a chronic disease associated with a 1.5-fold increase in cancer risk, including lung cancer, hematological malignancies, and breast cancer (BC). This is a retrospective study aiming to explore the clinical and pathological features of BC developed by SSc patients. A total of 54.5% of patients developed BC before SSc (median interval: 5 years), whereas 45.5% of patients developed BC after SSc (median delay: 8 years). A total of 93.1% of patients were diagnosed with an early stage tumor. Among invasive carcinomas, 70.8% presented with a low Mib1, 8.3% with a tubular histotype, and 42.8% with a Luminal A-like phenotype. A total of 66.6% of patients underwent breast-conserving surgery and 55.5% RT. A total of 40% of patients developed interstitial lung disease after RT and 20% diffuse cutaneous SSc. The cause of death of the six deceased patients was PAH. A significant association was observed between the use of immunosuppressive therapy and diffuse skin extension, negative ACA, positive Anti-Scl-70, and interstitial lung disease, but not BC status. SSc patients developed BC at a good prognosis, suggesting a de-escalation strategy of cancer therapies. In particular, ionizing radiation and chemotherapeuticals should be limited to higher-risk cases. Finally, proper screening is mandatory in order to allow for early cancer detection in SSc patients.

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AB0592 NAILFOLD CAPILLARY ABNORMALITIES PREDICT INTERSTITIAL LUNG DISEASE (ILD) COMPLICATION IN SYSTEMIC SCLEROSIS PATIENTS

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.5623 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1592.2-1593

Author(s):

T. Miyagi ◽

T. Kameda ◽

S. Nakashima ◽

H. Shimada ◽

R. Wakiya ◽

...

Keyword(s):

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Odds Ratio ◽

Pulmonary Function Tests ◽

Classification Criteria ◽

Digital Ulcers ◽

Number Of Patients ◽

Multicenter Prospective Cohort Study ◽

The Relationship

Background:Systemic sclerosis (SSc) have various organ involvements including pulmonary hypertension (PH), digital ulcers (DU), and interstitial lung disease (ILD). On the other hand, Nailfold capillary (NFC) abnormalities (enlarged/giant capillaries, fresh or old hemorrhages, avascular areas, ramified/bushy capillaries) detected by capillaroscopy are included in ACR/EULAR classification criteria for SSc as one of important findings. In addition, many studies have reported the relationship between NFC abnormalities and organ involvements (DU, PH) [1][2]. However, there are a few reports about the relationship between NFC abnormalities and ILD.Objectives:We clarify the association with NFC abnormalities and ILD in SSc patients.Methods:We enrolled SSc patients without PH from January 2016 to December 2019 in our institution. SSc patients were diagnosed according to EULAR classification criteria in 2013. ILD was detected by chest CT scans. We assessed severity of ILD with pulmonary function tests (PFT). Abnormal PFT was defined as vital capacity (%VC) or diffusion capacity (DLCO) < 70%. NFC abnormalities were detected with “OptiPiX capillaroscopy Clinic 1.7.x” and the number of capillaries was measured per 1mm in 2nd to 5th fingers of both hand. We defined enlarged and giant capillaries as >30 µm and >50 µm, respectively.Results:We enrolled 59 SSc patients (54 females, 5 males). Mean age is 65.0 ± 8.0 years. Thirty-one patients (52.5%) were complicated with ILD. Mean capillary counts are 6.6/mm. The number of patients with each NFC abnormalities (enlarged capillaries, giant capillaries, microhemorrhages, ramified, avascular areas) are 42, 32, 48, 38, and 33 cases, respectively. Two cases did not have NFC abnormalities. SSc patients with giant capillaries had fewer ILD complications (p <0.05, odds ratio 0.183 [0.059 – 0.57]). Other NFC abnormalities were not associated with ILD in SSc patients. We inspected %VC of 23 patients and DLCO of 20 patients with ILD. Eleven patients had abnormal PFT (5 patients had abnormal %VC and 9 patients had abnormal DLCO). Most of them had not enlarged capillaries than patient with normal PFT (odds ratio 0.11 [0.016 – 0.81]). Other NFC abnormalities including giant capillaries were not associated with abnormal PFT.Conclusion:We investigated the relationship between NFC abnormalities and ILD conplications in SSc patients. NFC abnormalities are associated with ILD complicacion and severity of ILD. It was suggested that no giant capillary in SSc patients may predict ILD complication. Moreover, no enlarged capillary may predict the severe ILD.References:[1]Valeria Riccieri et al. Systemic sclerosis patients with and without pulmonary arterial hypertension: a nailfold capillaroscopy study. Rheumatology, Volume 52, Issue 8, 1 August 2013, Pages 1525–1528[2]Maurizio Cutolo et al. Nailfold Videocapillaroscopic Features and Other Clinical Risk Factors for Digital Ulcers in Systemic Sclerosis: A Multicenter, Prospective Cohort Study. Arthritis Rheumatol. 2016 Oct; 68(10): 2527–2539.Disclosure of Interests:None declared

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Interstitial lung disease in systemic sclerosis: where do we stand?

European Respiratory Review ◽

10.1183/16000617.00002915 ◽

2015 ◽

Vol 24 (137) ◽

pp. 411-419 ◽

Cited By ~ 50

Author(s):

Susanna Cappelli ◽

Silvia Bellando Randone ◽

Gianna Camiciottoli ◽

Amato De Paulis ◽

Serena Guiducci ◽

...

Keyword(s):

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Pulmonary Function Tests ◽

Treatment Strategies ◽

Haematopoietic Stem Cell ◽

High Resolution Computed Tomography ◽

Cellular Targets ◽

Need For Treatment ◽

Risk Of Progression

Interstitial lung disease (ILD) is common in systemic sclerosis (SSc) patients and despite recent advances in the treatment is, at present, the major cause of death. Today, an early diagnosis of ILD is possible, and is mandatory to improve the prognosis of the disease.Pulmonary function tests and high-resolution computed tomography remain the mainstay for the diagnosis of SSc-ILD, but there is a growing interest in lung ultrasound. Recently, the correlation between severity of fibrosis and some peripheral blood biomarkers has been described.Nonselective immunosuppressors are still the main treatment for ILD, with cyclophosphamide (CYC) most widely used to obtain remission. Novel therapies towards specific molecular and cellular targets have been suggested; in particular, rituximab (RTX) has shown promising results, but further research is needed. It is of paramount importance to define the severity of the disease and the risk of progression in order to define the need for treatment and the treatment intensity. We propose the division of the treatment strategies at our disposal to induce remission into three categories: high intensity (haematopoietic stem cell transplantation), medium intensity (CYC and RTX) and low intensity (azathioprine (AZA) and mycophenolate mofetil (MMF)). After obtaining remission, maintenance treatment with AZA or MMF should be started.In this review we explore new advances in the pathogenesis, diagnosis and treatment of SSc-ILD.

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