FRI0099 Pulmonary fibrosis and connective tissue diseases: Follow-up of lung involvement in rheumatoid arthritis and systemic sclerosis

Background/Aim. Systemic connective tissue diseases (SCTD) are chronic inflammatory autoimmune disorders of unknown cause that can involve different organs and systems. Their course and prognosis are different. All of them can, more or less, involve the respiratory system. The aim of this study was to find out the frequency of respiratory symptoms, lung function disorders, radiography and high-resolution computerized tomography (HRCT) abnormalities, and their correlation with the duration of the disease and the applied treatment. Methods. In 47 non-randomized consecutive patients standard chest radiography, HRCT, and lung function tests were done. Results. Hypoxemia was present in nine of the patients with respiratory symptoms (20%). In all of them chest radiography was normal. In five of these patients lung fibrosis was established using HRCT. Half of all the patients with SCTD had symptoms of lung involvement. Lung function tests disorders of various degrees were found in 40% of the patients. The outcome and the degree of lung function disorders were neither in correlation with the duration of SCTD nor with therapy used (p > 0.05 Spearmans Ro). Conclusion. Pulmonary fibrosis occurs in about 10% of the patients with SCTD, and possibly not due to the applied treatment regimens. Hypoxemia could be a sing of existing pulmonary fibrosis in the absence of disorders on standard chest radiography.

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Contribution of pulmonary function tests (PFTs) to the diagnosis and follow up of connective tissue diseases

Multidisciplinary Respiratory Medicine ◽

10.4081/mrm.2019.17 ◽

2019 ◽

Vol 14 ◽

Author(s):

Nicola Ciancio ◽

Mauro Pavone ◽

Sebastiano Emanuele Torrisi ◽

Ada Vancheri ◽

Domenico Sambataro ◽

...

Keyword(s):

Connective Tissue ◽

Pulmonary Function ◽

Lung Disease ◽

Pulmonary Function Tests ◽

Connective Tissue Diseases ◽

Respiratory Pattern ◽

Main Body ◽

Lung Involvement ◽

Function Tests

Introduction: Connective Tissue Diseases (CTDs) are systemic autoimmune conditions characterized by frequent lung involvement. This usually takes the form of Interstitial Lung Disease (ILD), but Obstructive Lung Disease (OLD) and Pulmonary Artery Hypertension (PAH) can also occur. Lung involvement is often severe, representing the first cause of death in CTD. The aim of this study is to highlight the role of Pulmonary Function Tests (PFTs) in the diagnosis and follow up of CTD patients. Main body: Rheumatoid Arthritis (RA) showed mainly an ILD with a Usual Interstitial Pneumonia (UIP) pattern in High-Resolution Chest Tomography (HRCT). PFTs are able to highlight a RA-ILD before its clinical onset and to drive follow up of patients with Forced Vital Capacity (FVC) and Carbon Monoxide Diffusing Capacity (DLCO). In the course of Scleroderma Spectrum Disorders (SSDs) and Idiopathic Inflammatory Myopathies (IIMs), DLCO appears to be more sensitive than FVC in highlighting an ILD, but it can be compromised by the presence of PAH. A restrictive respiratory pattern can be present in IIMs and Systemic Lupus Erythematosus due to the inflammatory involvement of respiratory muscles, the presence of fatigue or diaphragm distress. Conclusions: The lung should be carefully studied during CTDs. PFTs can represent an important prognostic tool for diagnosis and follow up of RA-ILD, but, on their own, lack sufficient specificity or sensitivity to describe lung involvement in SSDs and IIMs. Several composite indexes potentially able to describe the evolution of lung damage and response to treatment in SSDs are under investigation. Considering the potential severity of these conditions, an HRCT jointly with PFTs should be performed in all new diagnoses of SSDs and IIMs. Moreover, follow up PFTs should be interpreted in the light of the risk factor for respiratory disease related to each disease.

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AB0605 CLINICAL PROFILE AND CHEST HIGH-RESOLUTION COMPUTED TOMOGRAPHY (HRCT) FINDINGS IN PATIENTS WITH CONNECTIVE TISSUE DISEASES AND INTERSTITIAL LUNG DISEASE: EXPERIENCE OF A SINGLE REFERENCE RHEUMATOLOGY CENTER

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.3758 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1598.2-1599

Author(s):

I. Rusu ◽

L. Muntean ◽

M. M. Tamas ◽

I. Felea ◽

L. Damian ◽

...

Keyword(s):

Computed Tomography ◽

Systemic Sclerosis ◽

High Resolution ◽

Interstitial Lung Disease ◽

Connective Tissue ◽

Lung Disease ◽

Interstitial Pneumonia ◽

Connective Tissue Diseases ◽

High Resolution Computed Tomography ◽

Hrct Patterns

Background:Interstitial lung disease (ILD) is a common manifestation of connective tissue diseases (CTDs), and is associated with significant morbidity and mortality. Chest high-resolution computed tomography (HRCT) play an important role in the diagnosis of ILD and may provide prognostic information.Objectives:We aimed to characterize the clinical profile and chest HRCT abnormalities and patterns of patients diagnosed with CTDs and ILD.Methods:In this retrospective, observational study we included 80 consecutive patients with CTDs and ILD referred to a tertiary rheumatology center between 2015 and 2019. From hospital charts we collected clinical data, immunologic profile, chest HRCT findings. HRCT patterns were defined according to new international recommendations.Results:Out of 80 patients, 64 (80%) were women, with a mean age of 55 years old. The most common CTD associated with ILD was systemic sclerosis (38.8%), followed by polymyositis (22.5%) and rheumatoid arthritis (18.8%). The majority of patients had dyspnea on exertion (71.3%), bibasilar inspiratory crackles were present in 56.3% patients and 10% had clubbing fingers. Antinuclear antibodies (ANA) were present in 78.8% patients, and the most frequently detected autoantibodies against extractable nuclear antigen were anti-Scl 70 (28.8%), followed by anti-SSA (anti-Ro, 17.5%), anti-Ro52 (11.3%) and anti-Jo (7.5%). Intravenous cyclophosphamide therapy for 6-12 months was used in 35% of patients, while 5% of patients were treated with mycophenolate mofetil.The most frequent HRCT abnormalities were reticular abnormalities and ground glass opacity. Non-specific interstitial pneumonia (NSIP) was identified in 46.3% CTDs patients. A pattern suggestive of usual interstitial pneumonia (UIP) was present in 32.5% patients, mainly in patients with systemic sclerosis. In 21.3% patients the HRCT showed reticulo-nodular pattern, micronodules and other abnormalities, not diagnostic for UIP or NSIP pattern.Conclusion:Nonspecific interstitial pneumonia (NSIP) is the most common HRCT pattern associated with CTDs. Further prospective longitudinal studies are needed in order to determine the clinical and prognostic significance of various HRCT patterns encountered in CTD-associated ILD and for better patient management.References:[1]Ohno Y, Koyama H, Yoshikaua T, Seki S. State-of-the-Art Imaging of the Lung for Connective Tissue Disease (CTD). Curr Rheumatol Rep. 2015;17(12):69.[2]Walsh SLF, Devaraj A, Enghelmeyer JI, Kishi K, Silva RS, Patel N, et al. Role of imaging in progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150)Disclosure of Interests:None declared

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Limited cutaneous systemic sclerosis: Total rehabilitation with fixed prosthesis on dental implants

Journal of Scleroderma and Related Disorders ◽

10.1177/23971983211004362 ◽

2021 ◽

pp. 239719832110043

Author(s):

Miguel Angel Garcés Villalá ◽

Carolina Zorrilla Albert

Keyword(s):

Systemic Sclerosis ◽

Bone Loss ◽

Dental Implants ◽

Social Life ◽

Connective Tissue Diseases ◽

The Body ◽

Calcinosis Cutis ◽

Complex Disorders ◽

Standardized Parameters

Introduction: Limited cutaneous systemic sclerosis with special manifestations (calcinosis cutis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) is part of the group of connective tissue diseases, these rare autoimmune systemic pathologies cause thickening and hardening of tissues in different parts of the body and can lead to complex disorders. Oral manifestations of systemic sclerosis may include limited ability to open the mouth, xerostomia, periodontal disease, enlarged periodontal ligament, and bone resorption of the jaw. Case Description: A 54-year-old Caucasian patient presented with oral pain, swallowing, phonation and chewing difficulties associated with dental instability, hygiene/handling difficulties and her main problem with microstomia, which prevented her from removing the skeletal prosthesis for 4 years, depriving her of social life. Gradual treatment with dental implants was diagnosed and planned to support a fixed total denture adapted to the ridge with self-cleaning characteristics. After implant insertion, panoramic radiographs with standardized parameters were taken to compare crestal bone levels at the time of prosthesis placement and with 10 years of follow-up. Conclusion: The average crestal bone loss of the 12 implants after the 10 years of follow-up was 1.26 mm for the maxilla and 1.17 mm for the mandible. The survival of the 12 support implants of two total fixed prostheses in a clinical/radiographic follow-up of 10 years was 100%. After 10 years of follow-up, the 12 implants inserted had a bone loss similar to that of healthy patients and no pathologies were registered, recovering function, aesthetics, and self-esteem. This therapy must be implemented before the interincisal distance decreases to 30 mm to allow intraoral surgical/prosthetic access. Implant-supported total fixed rehabilitation is a viable, predictable, and recommended therapy in patients with limited cutaneous systemic sclerosis.

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Factors Associated with Erosive Arthritis in Rheumatoid Arthritis and Other Connective Tissue Diseases

JCR Journal of Clinical Rheumatology ◽

10.1097/rhu.0000000000000341 ◽

2016 ◽

Vol 22 (1) ◽

pp. 22-29

Author(s):

Shiwen Yuan ◽

Dongying Chen ◽

Youjun Xiao ◽

Minxi Lao ◽

Qian Qiu ◽

...

Keyword(s):

Rheumatoid Arthritis ◽

Connective Tissue ◽

Connective Tissue Diseases ◽

Factors Associated ◽

Erosive Arthritis

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Knee myopericytoma-case report and literature review

MOJ Orthopedics & Rheumatology ◽

10.15406/mojor.2020.12.00534 ◽

2020 ◽

Vol 12 (6) ◽

pp. 127-128

Author(s):

Susana Rodrigues ◽

Catrine Ferreira ◽

Tiago Coelho ◽

Diogo Gaspar ◽

Jean Fallah ◽

...

Keyword(s):

Rheumatoid Arthritis ◽

Case Report ◽

Systemic Sclerosis ◽

Middle Age ◽

Subcutaneous Tissue ◽

Connective Tissue Diseases ◽

Systemic Lupus Erythematous ◽

Systemic Lupus ◽

Calcinosis Cutis ◽

Substantial Morbidity

Calcinosis cutis is a rare disorder characterized by of deposition of insoluble calcium salts in the skin and subcutaneous tissue. Five subtypes of calcinosis cutis are described: dystrophic, metastatic, idiopathic, iatrogenic, and calciphylaxis.1 Of these subtypes, dystrophic calcinosis (DC) is the most common, and it is the most frequently seen in association with underlying autoimmune connective tissue diseases.2 Dermatomyositis, systemic sclerosis and less commonly systemic lupus erythematous were described to be complicated by DC. However, DC associated with rheumatoid arthritis (RA) is extremely rare.2 The condition causes substantial morbidity and is associated with pain and limitation of movement when the process involves areas close to joints or when ulceration occurs.2 We report a middle age Sudanese woman with good controlled RA who developed dystrophic calcinosis cutis.

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