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PubMed1994

[Pulmonary inflammation in systemic sclerosis. An assessment by bronchoalveolar lavage].

Interstitial pulmonary fibrosis is a common feature of systemic sclerosis (SS). Clinicopathologic observations suggest that interstitial and alveolar inflammation may appear prior to fibrosis. Using the bronchoalveolar lavage (BAL) technique, we have characterized the nature of the inflammatory process in the lower respiratory tract of 15 non-smoking scleroderma patients and compared the results with those found in eight normal subjects. Alveolitis was detected in 11 patients (73%). Abnormalities in the cellular constituent included a significant increase of the proportion of lymphocytes (20.8 +/- 3.2 vs 8.1 +/- 1.2; p = 0.01) and neutrophils (3.3 +/- 0.8 vs 0.8 +/- 0.2; p = 0.04). T cell subsets, evaluated by flow cytometry, showed a significant increase of CD8+ T cells (36.6 +/- 3.2 vs 25, 1 +/- 3.3; p = 0.04) and a decrease of CD4+/CD8+ ratio (1.1 +/- 0.1 vs 2.1 +/- 0.4; p = 0.04). Seven patients with an elevation of proportional BAL (lymphocyte count > 15%) had greater dyspnoea and chest radiographic score than patients without lymphocytic alveolitis (p = 0.02) and they had greater reduction in carbon monoxide diffusing capacity (DLco) (p = 0.04). Furthermore, a statistically significant correlation between the total lymphocyte cell count and the DLco (p = 0.03) was observed. Finally the stepwise multiple regression analysis identified lymphocyte count in lavage fluid as the predictor most strongly associated with an abnormal interstitial radiographic pattern (p = 0.001). Our results report the notion that the lymphocytes, in addition to the macrophages and neutrophils, may play roles in the pathogenesis of lung injury.

Adult↗
PubMed2000

[High resolution multislice CT of the lung: comparison with sequential HRCT slices].

PURPOSE: To compare the image quality of high-resolution (HR) spiral CT scans from a multislice CT scanner with sequential HRCT scans from a singleslice CT scanner. MATERIALS AND METHODS: 20 patients with diffuse lung disease received a high-resolution spiral CT on a multislice scanner (4 slices) and 5 HRCT single slices (1 mm) on a singleslice scanner. Scan parameters of the multislice scanner were: Collimation 4 x 1 mm, pitch 6, slice thickness 1 mm. 5 HRCT slices were compared to the corresponding HR spiral CT slices using a 5-point scale by 5 radiologists with regard to the image quality and the number of artifacts. The evaluation was performed with a multivariate analysis (MANOVA test). RESULTS: Overall impression of image quality, noise, central vessels and bronchi, and all pathological changes were not significantly different between the two CT methods. Sequential HRCT scans were considered to be significantly better than HR spiral CT scans for spatial resolution (p = 0.02), depiction of peripheral vessels (p = 0.02), and of small bronchi (p = 0.05), and significantly worse for depiction of interlobar septa (p < 0.001). Diagnostically relevant differences were found in only 2.2%. Breathing and heartbeat artifacts each were 3 times higher in the sequential HRCT technique than in the multislice-spiral technique (p < 0.0001). CONCLUSION: HR spiral CT scans performed on a multislice CT scanner provide significantly less artifacts and an equal diagnostic image quality compared to sequential HRCT scans performed on a singleslice CT scanner. Multislice spiral CT in HR technique may replace the common scanning technique with conventional spiral CT and additional HRCT scans for diffuse lung diseases.

Adult↗
PubMed2004

Nailfold videocapillaroscopic patterns and serum autoantibodies in systemic sclerosis.

BACKGROUND: Microvascular lesions are a predominant feature in systemic sclerosis (SSc) and seem to play a central pathogenetic role. Recently, we graded scleroderma microangiopathy by nailfold videocapillaroscopy (NVC) into three NVC patterns (early, active and late). The aim of the present study was to confirm, in a larger number of SSc patients, the presence of three patterns of microvascular damage, and to detect any possible relationship between these patterns and both specific serum autoantibodies and the subsets of cutaneous involvement. METHODS: Two hundred and forty-one consecutive patients (227 women and 14 men) affected by SSc were recruited. One hundred and forty-eight patients were affected by limited cutaneous SSc (lSSc) and 93 patients by diffuse cutaneous SSc (dSSc). The ages at onset of Raynaud's phenomenon (RP) and SSc, the durations of RP and SSc, ANA and antitopoisomerase I (anti-Scl70) and anticentromere (ACA) antibodies were investigated in all patients. The SSc patients were subdivided on the basis of the NVC pattern into three groups. RESULTS: A statistically significant correlation was found between the NVC patterns and the durations of both RP and SSc (P<0.001). Enlarged and giant capillaries, together with haemorrhages, constituted the earliest NVC finding in SSc (early NVC pattern). These abnormalities were mostly expressed in the active NVC pattern. Loss of capillaries, ramified capillaries and vascular architectural disorganization were increased in the late NVC pattern. Age and the duration of both RP and SSc were lower in 24 patients complaining of RP alone. Anti-Scl70 antibodies were statistically less frequent in the early vs both the active and the late NVC pattern, whereas no significant correlation was found between the presence of anti-Scl70 antibodies and the duration of either RP or SSc. ACA positivity was more frequent in patients with longer RP duration. Patients with lSSc had shorter SSc duration and showed the early or active NVC pattern more frequently. Conversely, patients with dSSc showed longer disease duration and mostly showed the late NVC pattern. CONCLUSIONS: NVC is an appropriate tool for differential diagnosis between primary and secondary RP through the clear recognition of the early NVC scleroderma pattern. This study confirms, in a large number of SSc patients, the existence of three distinct NVC patterns that might reflect the evolution of SSc microangiopathy. The presence of anti-Scl70 antibodies seems be related to earlier expression of the active and late NVC patterns of SSc microvascular damage. The presence of ACA seems to be related to delayed expression of the late NVC pattern.

Adult↗
PubMed1998

Mortality and causes of death of 344 Danish patients with systemic sclerosis (scleroderma)

OBJECTIVE: To determine survival, mortality and causes of death in Danish patients with systemic sclerosis (scleroderma), and to analyse how these parameters are influenced by demographic variables and the extent of skin involvement. METHODS: A cohort of 344 patients with incident systemic sclerosis (SSc) after 1 January 1960 was retrospectively identified, representing 3716 patient-years of follow-up. The vital status at the end of the study ultimo 1996 was established by reviewing the clinical charts and by contacting the Danish Central Person Register. The causes of death were based on information obtained from the hospital charts and the autopsy reports (80%) and death certificates (20%). The extent of skin sclerosis was described by means of a two-subset model (limited and diffuse involvement) and a three-subset model (digital, extremity and truncal involvement) in all patients. Expected numbers of deaths were calculated by means of age- and sex-specific mortality rates of the general Danish population. RESULTS: Crude mortality rates were 6.5 and 3.9%/yr, respectively, in men and women. The standardized mortality ratio (SMR) was 2.9 (95% CI 2.5-3.4) and was not significantly influenced by sex or age at disease onset. Mortality rates were significantly increased in patients with diffuse skin sclerosis (SMR 4.5, 95% CI 3.5-5.7) and patients under the age of 35 yr (SMR 13, 95% CI 2.7-37). Of the 160 patients who died during the study, 41 deaths (26%) were SSc related, accounting for one-third of the excess mortality. CONCLUSION: We found a significantly increased mortality in patients with SSc, particularly in the subset of patients with diffuse skin sclerosis and in young patients. The excess mortality was due to an increase in both the SSc-related mortality and the unrelated mortality. However, all other subsets also had a significantly increased risk of death, mainly due to an increased risk of death due to causes unrelated to SSc, cancer among others. The fact that the unrelated mortality in SSc was found to be 2-fold increased suggests that the excess mortality that was classified as unrelated should more appropriately be termed indirectly SSc related.

Adolescent↗
PubMed1994

Autoantibodies to RNA polymerase II are common in systemic lupus erythematosus and overlap syndrome. Specific recognition of the phosphorylated (IIO) form by a subset of human sera.

Autoantibodies to RNA polymerases (RNAP) I, II, and III are reported to be highly specific for the diagnosis of scleroderma (systemic sclerosis, SSc). In the present study, the specificity of autoantibodies to RNAP I and III for SSc was confirmed by immunoprecipitation of 35S-labeled proteins. However, we report here the previously unrecognized production of anti-RNAP II autoantibodies by 9-14% of patients with SLE and mixed connective tissue disease/overlap syndrome. 12 out of 32 anti-RNAP II positive sera (group 1) immunoprecipitated a diffuse 220-240-kD band identified as the largest subunit of RNAP II whereas the remaining 20 (group 2) immunoprecipitated preferentially the 240-kD phosphorylated (IIo) form of the large subunit. After pulse labeling, group 1 sera immunoprecipitated only the 220-kD (IIa) RNAP II subunit, whereas the diffuse IIa/IIo band plus the 145-kD second largest RNAP II subunit (IIc) were immunoprecipitated after several hours of cold chase, suggesting that these sera recognized primarily the largest subunit of RNAP II. Group 2 sera recognized the IIc subunit after pulse labeling, and immunoprecipitated the IIc and IIo, but not the IIa, subunits after cold chase. Although it has been suggested that autoantibodies to RNAP II are usually accompanied by anti-RNAP I/III in SSc, all but one of the anti-RNAP II positive sera from SLE or mixed connective tissue disease/overlap syndrome patients, as well as most of the SSc sera, were negative for anti-RNAP I/III. Moreover, in contrast to previous reports suggesting that anti-RNAP antibodies rarely coexist with other SSc subset marker antibodies, anti-RNAP II antibodies were often accompanied by anti-Ku, anti-nRNP, or anti-topoisomerase I autoantibodies in the present study. We conclude that autoantibodies to RNAP II are not a specific marker for SSc, whereas autoantibodies to RNAP I/III are associated primarily with SSc. In addition, we have identified two distinctive patterns of RNAP II antigen recognition by autoantibodies, one of them characterized by specific recognition of the transcriptionally active (phosphorylated) form of RNAP II. The clinical significance of these different patterns remains to be determined.

Adult↗
PubMed1999

[Renal crisis in systemic sclerosis].

Systemic sclerosis is a rare connective tissue disorder, potentially affecting several internal organs. Renal disease is seen in 10-40% of the cases, while autopsy studies have shown interstitial renal changes in as many as 80%. Renal involvement is clinically divided into two separate forms; chronic and acute nephritis. Acute nephritis in a patient suffering from systemic sclerosis is termed renal chrisis, and is associated with high morbidity and mortality. Renal crisis in systemic sclerosis is discussed based on a case report and a review of the literature. Treatment with ACE-inhibitors has dramatically improved the prognosis of this complication. Careful surveillance of patients at risk, especially patients with the diffuse type of systemic sclerosis and showing antibodies against RNA-polymerase, as well as early treatment is considered important for the outcome.

Acute Kidney Injury↗
PubMedDate not supplied

Bronchiectasis in systemic sclerosis. A study using high resolution computed tomography.

OBJECTIVE: To detect noninvasively the presence of bronchiectasis in patients with systemic sclerosis (SSc), through the use of high resolution chest computed tomography (HRCT). METHODS: Twenty two patients with SSc, of whom 13 with diffuse and 9 with limited disease, besides a complete history, physical and routine laboratory and immunologic profile, were evaluated by pulmonary function testing and HRCT. The chi square test with Yates' correction, the Fisher's exact test, the Fisher's test (F test) and the "t" test were used for statistical analysis of the results. RESULTS: Eleven patients (50.0%) had decreased carbon monoxide diffusing lung capacity (DLCO) and, out of these, four had restrictive lung disease, based on a combined decrease of forced vital capacity (FVC) and total lung capacity (TLC). Another two patients exhibited this pattern without DLCO impairment. HRCT revealed a ground glass picture in 15 patients (68.2%), fibrosis in 9 (40.9%) (of which 5 with ground glass as well), and cylindrical bronchiectasis in 13 (59.1%). Bronchiectasis was more common in diffuse than in limited SSc, and the difference approached but did not reach the level of statistical significance. On the other hand, it was not correlated with either decreased DLCO, presence of ground glass and fibrosis, or with patients' age and disease duration. CONCLUSION: Although the number of patients included in our study is relatively small, our data, for the first time in the literature, indicate a significant association between scleroderma and bronchiectasis. Bronchiectasis should be included in the list of pulmonary manifestations of SSc, and SSc in the list of conditions causing bronchiectasis.

Adult↗
PubMed1985

An enzyme-linked immunosorbent assay for autoantibodies against the nuclear protein Scl-70.

This paper describes the development of an enzyme-linked immunosorbent assay (ELISA) for the detection and quantitation of autoantibodies against the nuclear protein Scl-70. The isolation of Scl-70 from rat livers and the conditions for the ELISA are described. Compared with the already established double diffusion in gel for detection of anti-Scl-70 antibodies this ELISA has advantages.

Absorption↗
PubMed2005

Endothelin-1 response to mental stress in early ischemic lesions of the extremities due to systemic sclerosis.

We studied circulating levels of endothelin-1, catecholamines and nitric oxide after a mental arithmetic test in 14 patients with early ischemic lesions of the extremities due to systemic sclerosis and slightly impaired peripheral vascular flow. The test induced an increase (P<0.01) in blood pressure, heart rate, endothelin-1 and catecholamine levels, whereas it did not change the low basal levels of nitric oxide. In healthy subjects (n=20) the test significantly (P<0.01) decreased endothelin-1 without affecting nitric oxide. The low basal levels of nitric oxide and the high plasma concentration of endothelin-1 after psychological stress cannot be explained by an impaired release from the limited ischemic lesions alone. This suggests a diffuse microvascular derangement that aggravates the course of peripheral microvascular ischemic lesions.

Adult↗
PubMed2003

New insights into the pathogenesis of systemic sclerosis.

Systemic sclerosis (SSc) is a connective tissue disorder characterized by vascular abnormalities and excessive collagen synthesis. Extracellular matrix overproduction by fibroblasts results from abnormal interactions among endothelial cells, mononuclear cells (lymphocytes and monocytes) and fibroblasts, in a setting of vascular hyperreactivity and tissue hypoxia. Many autoantibodies have been identified in the sera of SSc patients; some of them are specific to the disease, such as anti-centromere antibodies in limited SSc, anti-topoisomerase 1 and anti-RNA polymerase I/III antibodies in diffuse SSc. Their pathogenetic role(s) remains uncertain. However, genetic, environmental and possibly alloreactive factors might also contribute to disease susceptibility.

Autoantibodies↗
PubMed1977

Progressive systemic sclerosis complicated by diffuse pulmonary haemorrhage.

A case is reported of progressive systemic sclerosis with pulmonary fibrosis which was complicated by recurrent haemoptyses due to diffuse pulmonary haemorrhage. We have found no other report of this association. The haemorrhage finally remitted after treatment was started with 40 mg prednisone daily, though previously spontaneous remissions had occurred.

Hemoptysis↗
PubMed1982

Severe involvement of the conduction system in a patient with sclerodermal heart disease. An electrophysiological study.

A case of progressive systemic sclerosis with syncopal symptoms is reported. The presenting ECG pattern was that of an anterior myocardial infarction. The clinical history and the coronary angiography excluded significant coronary atherosclerotic heart disease. The ECG pattern evolved from the infarctual pattern associated with right bundle branch block to probably major degree of right bundle branch block associated with left posterior fascicular block. M-mode echocardiography, heart catheterization and angiographic studies did not reveal significant mechanical impairment of the left or right ventricle function. His bundle electrogram documented a markedly prolonged H-V interval, confirming an advanced impairment of distal conducting system. This case supports the suggestion that intraventricular conduction disorders in sclerodermal heart disease are not always related to diffuse myocardial involvement. The risk of sudden death justifies accurate electrophysiological evaluation in selected patients with sclerodermal cardiopathy.

Bundle-Branch Block↗
PubMed1996

Comparison of methotrexate with placebo in the treatment of systemic sclerosis: a 24 week randomized double-blind trial, followed by a 24 week observational trial.

In this study, methotrexate (MTX) was compared with placebo in the treatment of systemic sclerosis (scleroderma, SSc) in a 24 week randomized double-blind trial, followed by an observational trial of 24 weeks duration. Twenty-nine scleroderma patients were allocated to receive weekly injections of either 15 mg MTX or placebo. Patients who responded favourably after 24 weeks continued with the same regimen for a further 24 weeks; those who showed a poor response on placebo were allocated to further treatment with 15 mg MTX weekly, and those who responded poorly to treatment with 15 mg MTX had their doses increased to 25 mg. A favourable response was defined as an improvement of total skin score (TSS) by > or = 30%, of single breath diffusion capacity (DLCO) by > or = 15%, or of the score on a visual analogue scale of general well-being (VAS) by > or = 30%, provided that such improvements were not accompanied by persistent digital ulcerations or worsening of DLCO > or = 15%. Seventeen patients were allocated to MTX treatment and 12 to treatment with placebo. After 24 weeks, a significantly larger number of patients receiving MTX (n = 8, 53%) who completed the first 24 weeks of the study had responded favourably compared to patients receiving placebo (n = 1, 10%, P = 0.03). Comparison of separate variables between the two treatment groups by intention-to-treat analysis at week 24 showed improvement in the MTX group of TSS (P = 0.06) and creatinine clearance (P = 0.07). At week 48, 13 patients received MTX from the start of the study and nine during 24 weeks. From these 22 patients, 15(68%) responded favourably and compared with the start of the study they showed significant improvement of TSS (P = 0.04), VAS (P = 0.02), grip strength of the right hand (P = 0.02) and ESR (P = 0.01). Although the number of patients enrolled in this study is small, these results suggest that in a group of patients with active systemic sclerosis, low-dose MTX seems to be more effective than placebo according to pre-defined response criteria.

Adult↗
PubMed2006

Bosentan in pulmonary arterial hypertension secondary to scleroderma.

OBJECTIVE: . To assess the efficacy and tolerability of bosentan in pulmonary arterial hypertension secondary to systemic sclerosis (SSc-PAH) including patients with restrictive lung disease. METHODS: We retrospectively reviewed 23 SSc-PAH patients with PAH at baseline [PA systolic pressure (PASP) >or= 45 mm Hg by echocardiogram or mean PA pressure > 25 mm Hg at rest by cardiac catheterization], World Health Organization (WHO) functional classes II-IV, and with data available for 18 months. Bosentan dose was 62.5 mg twice daily for 1 month then 125 mg twice daily. Outcomes were WHO functional class, PASP, and pulmonary function tests (PFT) at 3-month intervals for 18 months. RESULTS: WHO class at baseline 3.1 +/- 0.1 (mean +/- SE); 3 months, 2.5 +/- 0.2*; 6 months, 2.4 +/- 0.2*; 9 months, 2.5 +/- 0.2* (*p < 0.02 vs baseline, n = 21 to 23), indicating clinical improvement at 9 months. After 9 months, results were not significant versus baseline. Reduction in WHO class by at least one rank was 57% at 3 months; none worsened. After 9 months, WHO class tended to worsen compared to baseline. Baseline PASP was 54 +/- 2 mm Hg (n = 23) and did not change significantly with therapy. Restriction (total lung capacity 76% +/- 4% of predicted) and reduced diffusing capacity (39% +/- 3% of predicted) were unchanged during therapy. Abnormal transaminases in 2 patients (9%) necessitated discontinuing drug in both. CONCLUSION: Bosentan is clinically beneficial in patients with SSc-PAH including patients with restrictive lung disease, but pulmonary hemodynamics and PFT results remained stable during treatment.

Adult↗
PubMed2005

The role of bronchoalveolar lavage in diffuse parenchymal lung diseases.

PURPOSE OF REVIEW: To evaluate the role of bronchoalveolar lavage in the diagnosis of diffuse parenchymal lung diseases and review the recent literature published within the past 12 months. RECENT FINDINGS: Diffuse parenchymal lung diseases are a heterogeneous group of disorders originating from the distal lung parenchyma. Despite different etiologies, the clinico-radiologic findings are usually alike and diagnosis can be challenging. Bronchoalveolar lavage and the analysis of cell counts are routine investigations for this group of disorders. Not only it can be used to see serial changes, it also important for providing information from a broader lung area. Although rarely diagnostic for a particular etiology, the predictive value of bronchoalveolar lavage differentials are reported to make some diagnoses more likely and exclude others like infections and tumors. This result is especially significant for the frequently encountered diffuse parenchymal diseases like sarcoidosis, usual interstitial pneumonia or extrinsic allergic alveolitis. SUMMARY: Bronchoalveolar lavage is a convenient and safe procedure to apply for the diagnosis of diffuse parenchymal lung diseases especially to provide more information of the likelihood of the more common forms like sarcoidosis, extrinsic allergic alveolitis and usual interstitial pneumonia.

Alveolitis, Extrinsic Allergic↗
PubMed1992

[An autopsied case of progressive systemic sclerosis with anti Wa antibody who showed a rapid progression].

An anti Wa antibody was reported as a new t-RNA related protein antibody in 1986. This autoantibody is now considered specific for the diagnosis of progressive systemic sclerosis (PSS). Up to date only 5 cases with anti Wa antibody have been identified. We report here an autopsied case of PSS with this antibody. A 53 years old female was admitted to our hospital because of dry cough and dyspnea in Sep 1987. There were fine crackles and chest X ray revealed interstitial pneumonia. The progressive respiratory failure was treated by steroid pulse therapy effectively. Sclerotic skin changes of hand began to appear in Sep 1988 and rapidly progressed to arms, chest and forehead by Dec 1988. A skin biopsy confirmed PSS changes. An anti Wa antibody was detected by double immunodiffusion and the protein antigen was associated with t-RNA when immunoprecipitation was conducted. She died of heart failure in July 1989. An autopsy revealed the diffuse fibrotic change of the heart and the lung. Cases with anti Wa antibody were shortly reviewed from the literature.

Autoantibodies↗
PubMed2006

Natural history of mild-moderate pulmonary hypertension and the risk factors for severe pulmonary hypertension in scleroderma.

OBJECTIVE: To determine risk factors for developing pulmonary hypertension (PH) in patients with scleroderma (SSc, systemic sclerosis). METHODS: We used a cohort of 1136 SSc patients using severe PH as the primary outcome in a natural history study. RESULTS: Among 361 individuals with no initial echocardiographic PH, 92 (26.0%) developed mild-moderate PH and 48 (13.6%) severe PH. Patients developing severe PH had lower initial DLCO (48.8% of predicted) than those who did not develop PH (56.8% of predicted). Patients with mild-moderate PH had a 17% probability of progressing to severe PH, and 15.6% probability of regressing to no PH. Individuals with limited disease, mild-moderate PH, and age > or= 47 years at diagnosis had a 27.3% probability of developing severe PH, compared to 8.5% in individuals with diffuse disease, no evidence of PH, and age < 47 years at diagnosis. Longitudinal regression models estimated that individuals with limited disease, mild-moderate PH, and DLCO < 50% predicted had an age-adjusted odds ratio of 8.6 of developing severe PH within 2 years compared to individuals without these risk factors. CONCLUSION: Development of severe PH is uncommon in certain subgroups of SSc patients. Risk factors for progression of PH include older age, limited skin disease, and elevated pulmonary artery pressures at the time of initial evaluation.

Adult↗
PubMed2001

Class II HLA associations with autoantibodies in scleroderma: a highly significant role for HLA-DP.

Scleroderma is a condition of variable phenotype characterised by fibrosis of the skin and internal organs. There is a range of disease-specific autoantibodies found in the sera of patients. The aims of this study were to: (1) investigate the role of the MHC and particularly HLA-DP in the production of autoantibodies; (2) investigate clinical associations with autoantibodies. We have performed HLA class II typing using PCR with sequence-specific primers on DNA samples from 202 scleroderma patients and 307 UK control subjects. All patients had well defined clinical phenotypes. Sera from patients were examined for the presence of disease specific autoantibodies in particular the anti-topoisomerase autoantibody (ATA), the anti-centromere autoantibody (ACA) and the anti-RNA polymerase autoantibody (ARA). There was a striking association between HLA-DPB1*1301 and ATA (Pcorr = 0.0001). In addition, ATA was associated with HLA-DRB1*11 and the anticentromere autoantibody (ACA) with HLA-DRB1*04, HLA-DRB1*08 (P = 0.001) and HLA-DQB1 alleles with a glycine residue at position 26. Very strong associations were detected between clinical phenotypes and autoantibodies. ATA was associated with pulmonary fibrosis (P = 0.00002), anti-RNA polymerase autoantibody (ARA) with renal involvement (P = 0.0000006) and diffuse skin disease (P = 0.00001), and ACA with limited skin involvement (P = 0.00002) and protection against pulmonary fibrosis (P = 0.0000003). We have identified a significant association between the ATA and HLA-DPB1*1301 which may provide an insight into how this autoantibody is formed. Patient clinical characteristics depend on the autoantibodies they carry.

Autoantibodies↗