scholarly journals Clinical and serological characteristics of systemic sclerosis: Experience of a tertiary care center in Pakistan

2021 ◽  
Vol 36 (4) ◽  
pp. 587-594
Author(s):  
Sadia Asif ◽  
Asadullah Khan ◽  
Muhammad Faiq ◽  
Zia Ud Din ◽  
Sarmad Zahoor ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Function Tests ◽  
Care Center ◽  
Tertiary Care ◽  
Disease Onset ◽  
Clinical Feature ◽  
Digital Ulcers ◽  
Limited Disease ◽  
Raynaud Phenomenon ◽  
Diffuse Disease

Objectives: This study aims to evaluate the clinical and serological characteristics of systemic sclerosis (SSc) in Pakistani population. Patients and methods: This prospective, cross-sectional study included a total of 38 patients (6 males, 32 females; mean age: 34.5±1.5 years; range, 16 to 60 years) with SSc who were admitted to our rheumatology clinic between November 2019 and January 2020. We evaluated the clinical, serological, and radiological features of SSc patients. Results: Thirty-four (89.5%) patients developed Raynaud phenomenon at the time of disease onset, while sclerodactyly was found in 34 (89.5%), digital ulcers in 25 (65.8%), and tendon friction rub in 12 (31.6%) patients. Interstitial lung disease was present in 30 (78.9%) patients with a higher prevalence in diffuse scleroderma (100%) than in limited scleroderma (70%) (p=0.01). Pulmonary hypertension was present in 18 patients with a significantly higher prevalence in diffuse disease (57.1%) than limited disease (11.8%) (p<0.01). Thirty (78.9%) patients had impaired pulmonary function tests. Fibromyalgia was present in seven (18.4%) patients, and depression was present in 10 (26.3%) patients. Antinuclear antibody (ANA) was positive in 30 (78.9%) patients. Anti-Scl-70 antibodies were present in 24 (63.2%) patients with a significant association with diffuse disease (85% vs. 35.3%, respectively; p<0.01). The anti-centromere antibodies (ACA) were present in 20 (52.6%) patients with a significantly higher rate in limited disease (94.2% vs. 19.0%, respectively; p<0.01). Conclusion: Scleroderma has a female preponderance. Raynaud phenomenon is the most initial clinical feature followed by other manifestations of a variable course and disease severity.

scholarly journals AB0093 CLINICAL AND SEROLOGICAL CHARACTERISTICS OF SYSTEMIC SCLEROSIS: EXPERIENCE FROM A TERTIARY CARE CENTER IN PAKISTAN

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1075.2-1076
Author(s):  
S. Asif ◽  
M. Haroon ◽  
A. Khan ◽  
M. Faiq
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Memorial Hospital ◽  
Limited Disease ◽  
Raynaud Phenomenon ◽  
Female Ratio ◽  
Diffuse Disease ◽  
Gi Symptoms

Background:Systemic sclerosis (SS) is less studied Connective tissue disease in our population. It is characterized by different manifestations which if left undiagnosed and untreated lead to serious complications. The hallmark of this disease is fibrosis of various organs including skin and also involving pulmonary, gastrointestinal and cardiovascular system. We aim to evaluate the clinical and serological characteristics of SS in our population. In addition, we evaluated the prevalence of Fibromyalgia and Depression in patients with SS.Objectives:We aimed to determine Clinical and Serological Characteristics of Systemic Sclerosis visiting our department of Rheumatology, Fatima Memorial hospital, Lahore.Methods:It is a cross-sectional study of 38 patients visiting outdoor and indoor in Rheumatology department of Fatima Memorial Hospital from September 2019 to January 2020. All systemic sclerosis patients with age 16 years or above were included in this study. After taking informed consent, we filled the performas to record all the required information. We evaluated the clinical, serological and radiological features of Systemic Sclerosis.Results:A total of 38 patients were included in the study with mean age was 34.47 ± 1.53 years. Female gender comprised of 32(84.2%), with male to female ratio of 1:5.3. Thirty-four (89.5%) had developed Raynaud phenomenon and sclerodactyly was found in 34(89.5%), digital ulcers in 25 (65.8%), and tendon friction rub in 12 (31.6%) patients. Skin tightness proximal to elbow was present in 9 (23.7%). Microstomia (decreased mouth opening) was present in 32(84.2%) patients. Twenty-eight (73.7%) patients had arthritis.Respiratory symptoms comprised of shortness of breath in 36(94.7%), palpitation in 24 (63.2%) and chest pain in 7(18.4%). Gastrointestinal (GI) symptoms comprised of regurgitation in 31 (81.5%) and dysphagia in 14 (36.8%). Interstitial lung disease was present in 30 (78.9%) patients, with higher prevalence in diffuse scleroderma (100%) than in limited scleroderma (70%) (p=0.01). Pulmonary hypertension was present in 18 patients, with significantly higher prevalence in diffuse disease (57.1%), that was secondary to interstitial lung disease and in limited disease it was found in (11.8%) (p<0.01). Thirty (78.9%) patients were found to have restricted disease on pulmonary function tests.Obstetric history showed a higher prevalence of primary infertility in at least 6 (15.8%) patients, with significantly higher prevalence in limited systemic sclerosis disease as compared to diffuse disease (23.5% vs 9.5%, and p=0.05). Fibromyalgia diagnosed as per ACR criteria was present in 7 (18.4%) patients, and depression assessed by Hospital Anxiety and depression (HADS) score was present in 10 (26.3%) patients.Anti nucleic acid antibody (ANA) was found positive in 30 (78.9%) patients. Anti Scl-70 antibodies were in 24 (63.2%) patients, with significant association with diffuse disease (85% vs 35.3% and p<0.01), while anti centromere antibodies were present in 20 (52.6%) patients; significantly higher in limited disease (94.2% vs 19.0%, and p<0.01).Conclusion:Scleroderma is a very important, autoimmune multisystem disease. It has female preponderence. Raynaud phenomenon is the most initial clinical feature followed by other manifestations of variable course and disease severity. Interstitial lung disease and pulmonary hypertension were the most important complication found in our patients which has poor prognosis. So, It is imperative to early diagnose and treat the disease manifesations to prevent future complications.Keywords:Scleroderma, Systemic sclerosis.Disclosure of interest:Sadia Asif: None declared, Muhammad Haroon: None declared, Dr Asadullah Khan: None declared, Dr. Muhammad Faiq: None declareDOI: 10.1136/annrheumdis-2021-eular.1635

Clinical Correlates of CENP-A and CENP-B Antibodies in a Large Cohort of Patients with Systemic Sclerosis

2012 ◽  
Vol 39 (4) ◽  
pp. 787-794 ◽  
Author(s):  
MARIE HUDSON ◽  
MICHAEL MAHLER ◽  
JANET POPE ◽  
DANIEL YOU ◽  
SOLENE TATIBOUET ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Indirect Immunofluorescence ◽  
Disease Onset ◽  
Scleroderma Renal Crisis ◽  
Limited Disease ◽  
Anticentromere Antibody ◽  
Multicenter Cohort Study ◽  
Inflammatory Myositis ◽  
Diffuse Disease ◽  
Multicenter Cohort

Objective.To study the clinical phenotypes of centromeric proteins (CENP)-A- and CENP-B-positive patients with systemic sclerosis (SSc) and to compare them to anticentromere antibody (ACA)-positive and negative SSc patients.Methods.Sera samples were collected from 802 patients with SSc enrolled in a multicenter cohort study. Antibodies to CENP-A and B were detected by ELISA, and ACA by indirect immunofluorescence. Associations with clinical and other serological manifestations of SSc were investigated.Results.CENP-A antibodies were detected in 276 (34%), CENP-B in 286 (36%), and ACA in 279 (35%) patients. Patients having ACA, CENP-A, and/or CENP-B resembled each other and differed from the remainder of the cohort in the following respects: older chronologically and at disease onset; more commonly women; more likely to have limited disease and lower skin scores; less likely to have finger ulcers, digital tuft resorption, or finger contractures; more likely to have pulmonary hypertension; less likely to have interstitial lung disease, scleroderma renal crisis, inflammatory arthritis, and inflammatory myositis; and having lower overall disease severity. CENP-A and/or B status was predictive of the extent of skin involvement over time. Patients with limited disease who were CENP-A-negative at baseline were more likely to progress to diffuse disease compared to CENP-A-positive patients (OR 2.55, 95% CI 1.37, 4.85, p = 0.004).Conclusion.Clinical immunology laboratories are increasingly using high-throughput ELISA tests for CENP antibodies, with or without ACA detected by indirect immunofluorescence. The phenotype of CENP-A and/or B-positive patients is generally similar to that associated with ACA.

The Association Between Disease Activity and Duration in Systemic Sclerosis

2010 ◽  
Vol 37 (11) ◽  
pp. 2299-2306 ◽  
Author(s):  
JENNIFER G. WALKER ◽  
RUSSELL J. STEELE ◽  
MIREILLE SCHNITZER ◽  
SUZANNE TAILLEFER ◽  
MURRAY BARON ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Disease Activity ◽  
Disease Duration ◽  
Activity Index ◽  
Disease Onset ◽  
Depression Scale ◽  
Disease Activity Index ◽  
Cross Sectional ◽  
Diffuse Disease ◽  
Patient Reported

Objective.The absence of a standardized disease activity index has been an important barrier in systemic sclerosis (SSc) research. We applied the newly derived Valentini Scleroderma Disease Activity Index (SDAI) among our cohort of patients with SSc to document changes in disease activity over time and to assess possible differences in activity between limited and diffuse disease.Methods.Cross-sectional study of a national cohort of patients enrolled in the Canadian Scleroderma Research Group Registry. Disease activity was measured using the SDAI. Depression scores were measured using the Centre for Epidemiologic Studies Depression Scale (CES-D).Results.A total of 326 out of 639 patients had complete datasets at the time of this analysis; 87% were female, of mean age 55.6 years, with mean disease duration 14.1 years. SDAI declined steeply in the first 5 years after disease onset and patients with diffuse disease had 42% higher SDAI scores than patients with limited disease with the same disease duration and depression scores (standardized relative risk 1.42, 95% CI 1.21, 1.65). Patients with higher CES-D scores had higher SDAI scores relative to patients with the same disease duration and disease subset (standardized RR 1.22, 95% CI 1.14, 1.31). Among the 10 components that make up the SDAI, only skin score (standardized OR 0.59, 95% CI 0.43, 0.82) and patient-reported change in skin (standardized OR 0.64, 95% CI 0.45, 0.92) decreased with increasing disease duration. High skin scores (standardized OR 32.2, 95% CI 15.8, 72.0) were more likely and scleredema (standardized OR 0.58, 95% CI 0.37, 0.92) was less likely to be present in patients with diffuse disease. High depression scores were associated with positive responses for patient-reported changes in skin and cardiopulmonary function.Conclusion.Disease activity declined with time and patients with diffuse disease had consistently higher SDAI scores. Depression was found to be associated with higher patient activity scores and strongly associated with patient self-response questions. The role of depression should be carefully considered in future applications of the SDAI, particularly as several components of the score rely upon patient recall.

scholarly journals Digital ulcers in systemic sclerosis: their epidemiology, clinical characteristics, and associated clinical and economic burden

2019 ◽  
Vol 21 (1) ◽  
Author(s):  
Kathleen Morrisroe ◽  
Wendy Stevens ◽  
Joanne Sahhar ◽  
Gene-Siew Ngian ◽  
Nava Ferdowsi ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Systemic Sclerosis ◽  
Ambulatory Care ◽  
Clinical Characteristics ◽  
Hospital Admissions ◽  
Healthcare Services ◽  
Healthcare Resource Utilization ◽  
Digital Ulcers ◽  
Diffuse Disease

Abstract Background To determine the frequency and clinical characteristics of systemic sclerosis-related digital ulcers, and associated direct health care costs, quality of life, and survival. Methods Digital ulcers (DUs) were defined as an area with a visually discernible depth and a loss of continuity of epithelial coverage. DU severity was calculated based on the physician reported highest number of new DUs at clinical review (mild = 1–5 DUs, moderate 6–10 DUs, severe > 10 DUs). Healthcare use was captured through data linkage, wherein SSc clinical data captured prospectively in a dedicated clinical database were linked with health services databases to capture hospital admissions, emergency department (ED) presentations and ambulatory care (MBS) utilization and cost for the period 2008–2015. Healthcare cost determinants were estimated using logistic regression. Results Among 1085 SSc patients, 48.6% experienced a DU over a mean follow-up of 5.2 ± 2.5 years. Those who developed DUs were more likely to have diffuse disease subtype (34.9% vs 18.2%, p < 0.001), anti-Scl-70 antibody (18.9% vs 9.3%, p < 0.001), and a younger age at SSc onset (43.6 ± 13.9 vs 48.8 ± 14.0 years, p < 0.001) in addition to reduced health-related quality of life (HRQoL) measured by the SF-36 but without a significant impact on survival. SSc patients with a history of a DU utilized significantly more healthcare resources per annum than those without a DU, including hospitalizations, ED presentation, and ambulatory care services. Total healthcare services, excluding medications, were associated with an annual excess cost per DU patient of AUD$12,474 (8574-25,677), p < 0.001, driven by hospital admission and ED presentation costs. Conclusion DUs place a large burden on the patient and healthcare system through reduced HRQoL and increased healthcare resource utilization and associated cost.

Paradoxical Reaction of Raynaud Phenomenon following the Repeated Administration of Lloprost in a Patient with Diffuse Cutaneous Systemic Sclerosis

2012 ◽  
Vol 46 (10) ◽  
pp. 1439-1439 ◽  
Author(s):  
Rebeca Iglesias Barreira ◽  
Belén Bardán García ◽  
Mónica Granero López ◽  
Iria Rodríguez Legazpi ◽  
Hortensia Álvarez Díaz ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Repeated Administration ◽  
Vascular Involvement ◽  
Digital Ulcers ◽  
Probability Scale ◽  
Paradoxical Reaction ◽  
Raynaud Phenomenon ◽  
Naranjo Probability Scale ◽  
Case Summary ◽  
Diffuse Cutaneous Systemic Sclerosis

Objective TO report a paradoxical reaction of Raynaud phenomenon following the repeated administration of iloprost in a patient with diffuse cutaneous systemic sclerosis with vascular involvement. Case Summary In January 2006, a 40-year-old male was diagnosed with diffuse cutaneous systemic sclerosis with pulmonary, esophageal, cutaneous, and vascular involvement (Raynaud phenomenon, with digital ulcers on his hands). In December 2008, treatment with iloprost was started due to worsening disease. Nine cycles of iloprost were administered at a rate of 0.5–1 ng/kg/min (6 hours per day, for 5 days every 6–8 weeks); the patient tolerated this treatment well. However, on the fourth day of cycles 10 and 11, the patient developed paradoxical Raynaud phenomenon in the hand with perfusion when the infusion was increased to 1 ng/kg/min, requiring treatment to be stopped. Treatment was continued during cycles 12 and 13 at 0.5 ng/kg/min; the patient tolerated the treatment well, although paradoxical Raynaud phenomenon occurred when the rate of infusion was increased. Discussion Raynaud phenomenon is extremely common in patients with scleroderma, and often is severe. Iloprost has vasodilating, antiplatelet, cytoprotective, and immunomodulating properties, and has been found to be an efficacious alternative to nifedipine for the treatment of Raynaud phenomenon in patients with scleroderma. The Naranjo probability scale indicated that iloprost was the probable cause of the paradoxical Raynaud phenomenon in this patient. Conclusions This case demonstrates a probable relationship between the rate of infusion of iloprost and the paradoxical reaction of Raynaud phenomenon.

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

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

<p class="abstract"><strong>Background:</strong> 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.</p><p class="abstract"><strong>Methods:</strong> 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.<strong></strong></p><p class="abstract"><strong>Results:</strong> 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.</p><p class="abstract"><strong>Conclusions:</strong> 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.</p>

scholarly journals Personality and Psychopathology in Patients with Systemic Sclerosis

2017 ◽  
Vol 10 (1) ◽  
pp. 41-48
Author(s):  
Krisztina Csókási ◽  
Rita Hargitai ◽  
Róbert Járai ◽  
László Nagy ◽  
László Czirják ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Systemic Sclerosis ◽  
Minnesota Multiphasic Personality Inventory ◽  
Social Withdrawal ◽  
Care Center ◽  
Tertiary Care ◽  
Psychosocial Interventions ◽  
Obsessive Compulsive ◽  
Increased Risk ◽  
Study Results

Background: Systemic sclerosis is an autoimmune connective tissue disease with significant pain, fatigue, and disability. Patients with systemic sclerosis are at increased risk for psychiatric disorders. Objective: This study assessed the personality and psychopathological characteristics of scleroderma patients. Method: Consecutive, in-patient female cases with systemic sclerosis (N=72) from the tertiary care center of the University of Pécs and age- and sex-matched healthy volunteers (N=56) and fifty patients with rheumatoid arthritis were enrolled. Participants completed the Minnesota Multiphasic Personality Inventory-2. Demographical and clinical data were also collected. Results: Rheumatoid arthritis and scleroderma patients had similar profiles with high scores (≥65T) on Scales 1, 2 and 3. Additionally, they achieved moderate scores on Scales 5 and 0. The Scale 7 was elevated only in females with rheumatoid arthritis distinctively. Conclusion: High scores on Scales 1, 2 and 3 may reflect the severe somatic symptoms of systemic sclerosis (and rheumatoid arthritis), but may also refer to the disease-related emotional distress, anxiety and depression. Likewise, social withdrawal and less feminine interests may be the results of the chronic and disabling illness. The minor differences between the patient groups indicate that females with systemic sclerosis may be less vulnerable to specific anxiety disorders (obsessive-compulsive disorders, phobias). Study results highlight the importance of developing psychosocial interventions designed to meet the needs of scleroderma patients.

scholarly journals Osteonecrosis of carpal bones in systemic sclerosis

2021 ◽  
Vol 30 (3) ◽  
pp. 93-98
Author(s):  
Alexandra Chitac ◽  
◽  
Codrina Ancuta ◽  
◽  
Keyword(s):  
Systemic Sclerosis ◽  
Rare Complication ◽  
Vascular Supply ◽  
Clinical Findings ◽  
Carpal Bones ◽  
Digital Ulcers ◽  
Raynaud Phenomenon ◽  
Vascular Component ◽  
Anatomic Variants ◽  
Mechanical Factors

Background. Systemic sclerosis (SSc) is a rare chronic disease, with unknown aetiology and complex pathogenesis. The hand is often implied in the main clinical findings, being affected primarily by the vascular component (Raynaud phenomenon, digital ulcers). One of the possible complications of the hand vasculopathy is osteonecrosis (ON) of the carpal bones. In these cases, the lunate is the most frequently affected, followed by the scaphoid. In the ON process, not only the vascular supply is important, but the nearby mechanical factors and anatomic variants of the wrist. Our objective was to reveal the most important aspects regarding the ON of the carpal bones in patients with SSc. Methods. A systematic literature review was performed through July 31, 2021 on Pubmed and Cochrane databases. The eligible articles were read in full text and were included in this paper, in the absence of exclusion criteria and after consensus between two reviewers. Results. Ten articles met the inclusion criteria, their main results being described in this review. In all studies, ON of the lunate and the scaphoid is associated with advanced SSc and severe Raynaud phenomenon. Conclusions. In conclusion, carpal ON is a rare complication of SSc, especially of the diffuse type. There is limited data on this condition, its prevalence being difficult to estimate due to the lack of symptoms.

scholarly journals Serum defensin levels in patients with systemic sclerosis

2020 ◽  
Vol 60 (1) ◽  
Author(s):  
Tugce Emiroglu Gedik ◽  
Hamit Kucuk ◽  
Berna Goker ◽  
Seminur Haznedaroglu ◽  
Hatice Pasaoglu ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Healthy Subjects ◽  
Gastrointestinal Disease ◽  
Pulmonary Function Tests ◽  
Group Analysis ◽  
Pulmonary Involvement ◽  
Digital Ulcers ◽  
Lung Involvement ◽  
Diffuse Panbronchiolitis ◽  
Disease Characteristics

Abstract Background Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of skin and lung as well as involvement of kidney, gastrointestinal system and heart. Aetiology and exact mechanism of disease is poorly understood. The association between antimicrobial peptides (AMPs) and other diseases such as idiopathic pulmonary fibrosis, diffuse panbronchiolitis, pulmoner alveolar proteinosis and psoriasis have been reported. A small number of studies have examined the role of AMPs on autoimmune diseases which has not been studied in scleroderma yet. We aimed to investigate AMP serum levels and their association with disease characteristics of SSc. Methods Forty-two patients (40 female, mean age 42 years) and 38 healthy subjects (32 female, mean age 38 years) were enrolled. For SSc patients, the following data were recorded: disease subset (limited/diffuse), autoantibodies (antinuclear, anti-centromere (ACA), and anti-SCL-70), blood tests, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP), modified Rodnan skin score, presence and history of digital ulcers, kidney, gastrointestinal disease and lung involvement assessed by computed tomography and pulmonary function tests. Association between serum AMPs and disease characteristics were analysed. Results Twenty-nine of the patients had diffuse (69%) and 13 of the patients had limited (31%) systemic sclerosis. Average disease duration was 5.5 years. Pulmonary involvement was detected in 20 patients (47.6%). Serum concentration of alpha defensin was higher than healthy subjects (563 ± 415 vs 377 ± 269 ng/mL, p = 0.02). However, no difference was observed for beta-1 and beta-2 defensins in SSc patients and healthy controls. In sub-group analysis patients with interstitial lung disease had higher levels of alpha defensin than those without lung involvement (684 ± 473 vs 430 ± 299 ng/ml, p = 0.04). There was also correlation between alfa defensin serum concentrations and CRP (r = 0.34). Conclusions Alpha defensin levels are increased in scleroderma patients and correlated with lung involvement indicating a role in the pathogenesis of disease. Trial registration This study is not a clinical trial study.

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