Diagnosis Of Connective Tissue Disease Research Articles

The Relationship Between Anti-SSA-52 and Interstitial Lung Disease.

The aims of this study were to assess whether a relationship between anti-SSA-52 and interstitial lung disease (ILD) can be further defined, and to enhance screening, detection, and potentially guide treatment. A historical cohort study of 201 patients was conducted at a single tertiary care center between January 1, 2016 and December 31, 2020. All included patients were anti-SSA-52 antibody positive. Chart review was performed for laboratory values, symptoms, pulmonary function tests, treatment, and imaging. Chest computed tomographies were reviewed by chest radiologists. Among anti-SSA-52 antibody-positive patients, ILD was found in 125 (62.2%) compared with 76 (37.8%) with no ILD (p = 0.001). For those with ILD, 78 (62.4%) were diagnosed with connective tissue disease (CTD)-associated ILD, 28 (22.4%) were diagnosed ILD only, and 19 (15.2%) met the criteria for interstitial pneumonia with autoimmune features. In patients with CTD-ILD, 18 (23.0%) had their ILD diagnosis made over 6 months before a CTD diagnosis, and an additional 43 (55.1%) had their ILD and CTD diagnosed within 6 months of each other (p < 0.001). Common computed tomography patterns were nonspecific interstitial pneumonia/organizing pneumonia overlap in 44 (35.2%), 25 (20.0%) nonspecific interstitial pneumonia, and 15 (12%) usual interstitial pneumonia. Twenty-eight (35.9%) had antisynthetase syndrome, followed by 16 (20.5%) with dermatomyositis, 10 (12.8%) with CTD overlap, and 6 (7.7%) with systemic scleroderma. There was a significant association between anti-SSA-52 antibodies and ILD across a wide spectrum of rheumatological diagnoses. A significant portion of patients were diagnosed with ILD either at the same time or before their CTD diagnosis. Further study will be needed to assess effective treatment and response.

Spontaneous Iliac Artery Dissection in a 66-Year Old Male Complicated By Hemorrhage, GI Bleeding, and Bilateral DVT/PE

Spontaneous iliac artery dissection (IAD) is a rare medical condition with only a few cases being reported in the medical literature. Dissections typically involve the aorta and are not localized to the iliac arteries. Patients with spontaneous IAD typically present with symptoms that resemble peripheral artery disease, and cases are typically associated with connective tissue diseases such as Marfan syndrome and Ehlers-Danlos syndrome or high-risk states such as pregnancy and extreme athletics. There are some case reports that showed an association with hyperlipidemia, hypertension, and tobacco use. The most frequent complication of spontaneous IAD is rupture, and cases are typically treated endovascularly but may be treated with classical surgery such as in the case of rupture. Some cases have reportedly been treated with conservative management alone. In this case, we discuss a 66-year-old male with a past medical history of hyperlipidemia, hypertension, and tobacco use with no known diagnosis of connective tissue disease, and not in a high-risk state, who was found to have a spontaneous iliac artery dissection. He initially presented with sudden onset back pain that he described as a shooting pain that radiated into his head. He was also having right lower extremity discomfort. Initial vital signs were significant for tachycardia and hypertension, and physical exam was significant for tachycardia and diminished right posterior tibial and dorsalis pedis pulses. Laboratory workup was significant for a leukocytosis of 10.06 and creatinine of 1.52, consistent with acute kidney injury. Labs were otherwise unremarkable. Computed tomography angiogram (CTA) of the head and neck was negative for acute pathology, but CTA chest/abdomen/pelvis demonstrated arterial dissection that began at the origin of the right common iliac artery extending down to the right common femoral artery and also demonstrated a left renal infarct. A bare metal stent was placed by Interventional Radiology and the patient was started on dual antiplatelet therapy (DAPT) with a heparin drip and clopidogrel. Connective tissue disease workup was later performed including antinuclear antibody, rheumatoid factor, cyclic citrullinated peptide, and extractable nuclear antigen antibodies panel, which were all negative. Hospital stay was complicated by development of subarachnoid hemorrhage, intraventricular hemorrhage, subdural hematoma, and intradural hematoma that required laminoplasty and evacuation by Neurosurgery. DAPT was discontinued and the patient was later restarted on aspirin only. The patient then developed a cecal bleed that required clipping by Gastroenterology. Aspirin was discontinued and he then developed bilateral lower extremity deep venous thromboses and pulmonary emboli. He was started on enoxaparin with the assistance of Hematology and was later transitioned to apixaban with no complications. This case supports comorbidities as risk factors for spontaneous IAD and raises the need for formal screening and surveillance guidelines for these patients, as there are currently no guidelines in place. This case also demonstrates the complications that can occur with DAPT and anticoagulants and provides a manner in which to cautiously initiate anticoagulation in patients with high risk of bleeding while also preventing the development of further thrombi.

Anti-SSA Ro52 and anti-Ro60 autoantibodies: association with clinical phenotypes.

Anti-SSA autoantibodies can be differentiated according to their antigenic target proteins as anti-Ro60 (60 kDa) or anti-Ro52 (52 kDa). Anti-SSA(Ro60) antibodies are clearly associated with connective tissue diseases (CTD), but the clinical significance of anti-SSA(Ro52) antibodies remains unclear. The aim of the present study was to analyse the disease phenotype of patients with anti-Ro52 and/or anti-Ro60 antibodies. A multicentre, cross-sectional study was carried out of positive anti-Ro52 and/or Ro60 antibodies patients followed at 10 Rheumatology centres from January 2018 until December 2021. Patients were categorised into 3 groups: group 1 (Ro52+/Ro60-); group 2 (Ro52-/Ro60+); group 3 (Ro52+/Ro60+). Antinuclear antibodies were evaluated by indirect immunofluorescence assay and further screened for anti-extractable nuclear antigen (ENA) antibodies. Demographicsand clinical data were compared between the 3 groups, by patients' medical chart review. Univariate analysis was performed and subsequently logistic regression was used to identify intergroup differences and calculate the odds ratio with a 95% confidence interval (95% CI). We included 776 patients [female: 83.1%; median age: 59 (46-71) years]. Groups 1, 2, and 3 comprised 31.1%, 32.6%, and 36.3% of the patients, respectively. Anti-Ro52 antibody alone was more frequently associated with non-rheumatic diseases, older age, and men (p<0.05). Among patients with CTD, the diagnosis of systemic lupus erythematosus is 3 and 2 times more prevalent in groups 2 and 3, respectively, than in group 1 [OR 2.8 (95% CI 1.60, 4.97), p<0.001; OR 2.2 (95% CI 1.28, 3.86), p<0.01]. In group 2, the diagnosis of undifferentiated CTD is more frequent than in the other groups. Group 1 was more frequently associated with inflammatory myositis than group 2 [OR 0.09 (95% CI 0.01, 0.33), p<0.001] or group 3 [OR 0.08 (95% CI 0.01, 0.29), p<0.001]. Group 1 was also more frequently associated with arthritis (p<0.01), interstitial lung disease (p<0.01), and myositis (p<0.01). Anti-Ro52+ antibody alone is frequently found in patients with non-rheumatic diseases. In addition, anti-Ro52+ antibody is also prevalent in patients with CTD and associated with clinical phenotypes that are different from anti-Ro60+ antibody.

Infectious “masks” of connective tissue diseases (clinical cases)

For many centuries, infectious diseases have remained an important medical and social problem of humanity. To date high rates of morbidity remain among both the adult and child population. The similarity of clinical symptoms in the onset of some infections with diseases of different nature, including connective tissue diseases, often presents difficulties for verifying the diagnosis. Different clinical manifestations of connective tissue diseases dictate the need for an extended examination of patients to clarify the nature of the disease, to determine the role of the infectious agent, if it is detected: whether it is the cause of the existing symptoms or a trigger in the development of an autoimmune disease. The article presents 2 clinical observations of the infectious debut of connective tissue diseases. Two girls, 13 years old and 5 years old, were hospitalized at the Pediatric Research and Clinical Center for Infectious Diseases with suspected acute infectious pathology. The dominant complaint was fever. In the first case cough and pain syndrome of various localization were also noted and in the second case exanthema was observed. After examination it was impossible to verify the etiological pathogen. Сonnective tissue diseases were suspected, namely, in the first clinical case systemic lupus erythematosus was suspected, in the second juvenile dermatomyositis, which were confirmed by the detection of specific autoantibodies. Clinical cases demonstrate the difficulty of establishing connective tissue diseases diagnosis due to similarity these diseases clinical picture with the onset of infectious pathology. Taking into account the dependence of the course and outcomes of connective tissue diseases on the start of specific therapy, it is necessary to increase the alertness of infectious disease doctors and pediatricians for the timely detection of this cohort of patients.

Diagnostic Value of Capillary Density at Periungual Capillaroscopy in the Diagnosis of Connective Tissue Disease

Our study aims to demonstrate the contribution of quantitative analysis of capillaroscopic study in the early diagnosis of connective tissue diseases. One hundred and fifteen patients were evaluated by capillaroscopy. Raynaud's phenomenon is the most frequently encountered acro-syndrome leading to the request for capillaroscopic examination. capillaroscopy reveals minor dystrophy in 83.5% of patients and major dystrophy in 60% of them. On a quantitative level, 77 patients present, among the four examined fingers, at least one finger with a capillary density &lt;25 capillaries/3mm. For the diagnosis of connective tissue disease, this threshold is statistically significant with an odds ratio of 4.21 (95% CI = 1.47-11.99; p = 0.008). For this same diagnosis, its positive predictive value is 38%, its negative predictive value is 86%. A low capillary density, as a diagnostic test for connective tissue disease, has a sensitivity of 85% and a specificity of 41%. There is no significant difference between the decrease in capillary density for the diagnosis of connective tissue disease for each examined finger in terms of positive predictive value, negative predictive value, sensitivity, and specificity

Dense fine speckled nuclear immunofluorescence: A mildly reassuring antinuclear antibody pattern meriting consideration.

Antinuclear antibodies (ANAs) are regarded as a hallmark of connective tissue diseases (CTDs) and play a key role in their diagnosis, but the value of some particular antibodies in management of patients and the disease prognosis is controversial. The mechanism underlying the production of ANAs in CTDs, other chronic inflammatory conditions and even in healthy people, is not completely elucidated. Anti-DFS70 antibodies connected with the dense fine speckled autoantigen of 70 kD, known as the lens epithelium-derived growth factor p75, are a subgroup of ANAs. Their presence and coexistence with other antibodies and their clinical significance are the matter of debate. Based on literature data, the authors focused on current knowledge explaining the role of anti-DFS70 antibodies in selected CTDs. However, the literature data is ambiguous and does not fully support the validity of the anti-DFS70 assay for a specific CTD diagnosis. Most researchers claim that the presence of anti-DFS70 as the only one usually exclude the diagnosis of CTD. Nevertheless, its coexistence with other ANAs is not an excluding factor but has predictive value due to more favorable course of CTD. Such situations may also suggest an enhanced risk of the development of a CTD in the future. Although more studies are needed in this field, it seems reasonable to ascertain the presence of anti-DFS70 in routine clinical practice.

Recurrent Spontaneous Pneumothorax of Unknown Etiology in a Young Patient: Case Report

Introduction: Spontaneous pneumothorax is defined as the abnormal accumulation of air in the pleural space between the lungs and the chest wall, it occurs without an obvious etiology, such as trauma or iatrogenic causes. Primary spontaneous pneumothorax occurs when ther e is no history of underlying lung disease, while secondary spontaneous pneumothorax is associated with a history of underlying lung disease; its diagnosis is based on clinical suspicion and can be confirmed with imaging tests. Case presentation: We prese nt a 17 year old patient who presented spontaneous right pneumothorax, requiring placement of endopleural tube and Heimlich cannula with long stay, discharged for resolution and later relapsing in initial symptomatology one week later, requiring hospital readmission. Conclusion The recidivant spontaneous pneumothorax in the context of a young patient with clinical characteristics like those of our patient: tall stature, pectus excavatum, hyperlaxity of the integuments and aortic-mitral murmur, gives us hig h suspicion of a secondary cause, a good clinical history, with particular focus on the maternalfetal history and a detailed physical examination, are the most useful tools to integrate a diagnosis of connective tissue diseases.

Aortic Dissection in a Young Patient With Unsuspected Aortopathy.

Aortic dissection is often a fatal condition if not recognized and treated emergently. Fortunately, it is extremely rare in children and adolescents. We report a case of an adolescent boy who survived an aortic dissection due to severe aortic root dilation. A comprehensive history and physical examination, including family history, can facilitate an early diagnosis of connective tissue diseases, such as Loeys-Dietz syndrome (LDS).

Clinical features and prognosis of lung cancer in patients with connective tissue diseases: a retrospective cohort study.

Studies have demonstrated a close association between connective tissue diseases (CTDs) and lung cancer (LC). Evidence supports that poor survival may be associated with the presence of CTDs in patients with LC. This retrospective cohort study investigated 29 patients with LC with CTDs, and 116 patients with LC without CTDs were enrolled as case-matched control cohorts. Medical records, therapeutic efficacy of cancer, and outcomes were analyzed. The median duration from the diagnosis of CTDs to LC was 17 years. The Eastern Cooperative Oncology Group (ECOG) performance score for LC-CTD patients was worse than that for matched non-CTD LC patients. The median progression-free survival (mPFS) and overall survival (mOS) of first-line chemotherapy did not differ between patients with lung adenocarcinoma (AC) with and without CTDs. A significant difference was observed in mPFS [4 months vs. 17 months; hazard ratio (HR), 9.987; p = 0.004] and mOS (6 months vs. 35 months; HR, 26.009; p < 0.001) of first-line epidermal growth factor receptor tyrosine kinase inhibitor (EGFR-TKI) treatment between patients with AC with and without CTDs. The presence of CTD, sex, ECOG performance status, and tumor-node-metastasis clinical stage were the independent prognostic factors in all patients with non-small cell LC (NSCLC). ECOG performance status was determined to be an independent prognostic factor in patients with LC-CTD. In patients with NSCLC with CTD (n = 26), sex (male) and worse ECOG score were the independent poor prognostic factors. CTDs were associated with poor survival in patients with LC. The therapeutic efficacy of first-line EGFR-TKI therapy was significantly worse in patients with lung AC with CTDs than in those without CTDs. ECOG performance status was determined as an independent prognostic factor for patients with LC and CTDs.

Supporting the differential diagnosis of connective tissue diseases with neurological involvement by blood and cerebrospinal fluid flow cytometry

ObjectiveNeurological manifestations of autoimmune connective tissue diseases (CTD) are poorly understood and difficult to diagnose. We here aimed to address this shortcoming by studying immune cell compositions in CTD patients with and without neurological manifestation.MethodsUsing flow cytometry, we retrospectively investigated paired cerebrospinal fluid (CSF) and blood samples of 28 CTD patients without neurological manifestation, 38 CTD patients with neurological manifestation (N-CTD), 38 non-inflammatory controls, and 38 multiple sclerosis (MS) patients, a paradigmatic primary neuroinflammatory disease.ResultsWe detected an expansion of plasma cells in the blood of both N-CTD and CTD compared to non-inflammatory controls and MS. Blood plasma cells alone distinguished the clinically similar entities N-CTD and MS with high discriminatory performance (AUC: 0.81). Classical blood monocytes indicated higher disease activity in systemic lupus erythematosus (SLE) patients. Surprisingly, immune cells in the CSF did not differ significantly between N-CTD and CTD, while CD4+ T cells and the CD4+/CD8+ ratio were elevated in the blood of N-CTD compared to CTD. Several B cell-associated parameters partially overlapped in the CSF in MS and N-CTD. We built a machine learning model that distinguished N-CTD from MS with high discriminatory power using either blood or CSF.ConclusionWe here find that blood flow cytometry alone surprisingly suffices to distinguish CTD with neurological manifestations from clinically similar entities, suggesting that a rapid blood test could support clinicians in the differential diagnosis of N-CTD.

Myositis-Specific and Myositis-Associated Antibodies in Fibromyalgia Patients: A Prospective Study

Fibromyalgia (FM) is a common rheumatologic disorder characterised by widespread muscular pain. Myalgia is also a common clinical feature in Connective Tissue Disease (CTD), and FM should be studied for the concomitant presence of a CTD. The aim of this study is to evaluate the prevalence of Myositis-Specific and Myositis-Associated Antibodies (MSA/MAA) in a cohort of FM patients. We enrolled 233 consecutive FM patients (defined according to the 2016 criteria) that did not report clinical signs of autoimmune disorders and followed them for at least one year. The patients were tested for MSA/MAA with immunoblotting. FM patients were seropositive for Antinuclear Antibodies (ANA) in 24% of cases, for MSA in 9%, and for MAA in 6%. A specific diagnosis of CTD was made in 12 patients (5.2%), namely, 5 cases of primary Sjögren's Syndrome and 7 of Idiopathic Inflammatory Myopathy. Seropositive patients showed clinical features similar to those who were seronegative at baseline. A CTD diagnosis was associated with ANA positivity (p = 0.03, X2 4.9), the presence of a speckled pattern (p = 0.02, X2 5.3), positivity for MAA (p = 0.004, X2 8.1), and MSA (p = 0.003, X2 9.2). In conclusion, a non-negligible proportion of FM patients may be seropositive for MSA/MAA, and that seropositivity might suggest a diagnosis of CTD.

Multiparametric autoantibody analysis: a new paradigm for the diagnosis of connective tissue diseases

BackgroundIn patients affected by connective tissue diseases (CTDs), the identification of wide autoantibody profiles may prove useful in early diagnosis, in the evaluation of prognosis (risk stratification), and in predicting response to therapy. The aim of the present study was to evaluate the utility of multiparametric autoantibody analysis performed by a new fully automated particle-based multi-analyte technology (PMAT) digital system in a large multicenter cohort of CTD patients and controls.MethodsSerum samples from 787 patients with CTD (166 systemic lupus erythematosus; 133 systemic sclerosis; 279 Sjögren’s syndrome; 106 idiopathic inflammatory myopathies; 103 undifferentiated CTD), 339 patients with other disorders (disease controls) (118 infectious diseases, 110 organ-specific autoimmune diseases, 111 other rheumatic diseases), and 121 healthy subjects were collected in 13 rheumatologic centers of the FIRMA group. Sera were analyzed with the Aptiva-PMAT instrument (Inova Diagnostics) for a panel of 29 autoantibodies.ResultsMultiparametric logistic regression showed that enlarged antibody profiles have a higher diagnostic efficiency than that of individual antibodies or of antibodies that constitute classification criteria for a given disease and that probability of disease increases with multiple positive autoantibodies.ConclusionsThis is the first study that analyzes the clinical and diagnostic impact of autoantibody profiling in CTD. The results obtained with the new Aptiva-PMAT method may open interesting perspectives in the diagnosis and sub-classification of patients with autoimmune rheumatic diseases.

MYOPERICARDITIS : AN UNUSUAL INITIAL PRESENTATION OF UCTD

Diagnosis of connective tissue diseases is often delayed because the initial symptoms are few and non specic. A new entity called undifferentiated connective tissue disease(UCTD) has been recently described. It is a diagnosis of exclusion. We report the case of a 40yr old female, not a known case of any connective tissue disorder who presented to us with fever, dyspnea and chest pain as initial symptoms and nally diagnosed as myopericarditis secondary to UCTD.

Multispectral imaging of nailfold capillaries using light-emitting diode illumination.

The capillaries are the smallest blood vessels in the body, typically imaged using video capillaroscopy to aid diagnosis of connective tissue diseases, such as systemic sclerosis. Video capillaroscopy allows visualization of morphological changes in the nailfold capillaries but does not provide any physiological information about the blood contained within the capillary network. Extracting parameters such as hemoglobin oxygenation could increase sensitivity for diagnosis and measurement of microvascular disease progression. To design, construct, and test a low-cost multispectral imaging (MSI) system using light-emitting diode (LED) illumination to assess relative hemoglobin oxygenation in the nailfold capillaries. An LED ring light was first designed and modeled. The ring light was fabricated using four commercially available LED colors and a custom-designed printed circuit board. The experimental system was characterized and results compared with the illumination model. A blood phantom with variable oxygenation was used to determine the feasibility of using the illumination-based MSI system for oximetry. Nailfold capillaries were then imaged in a healthy subject. The illumination modeling results were in close agreement with the constructed system. Imaging of the blood phantom demonstrated sensitivity to changing hemoglobin oxygenation, which was in line with the spectral modeling of reflection. The morphological properties of the volunteer capillaries were comparable to those measured in current gold standard systems. LED-based illumination could be used as a low-cost approach to enable MSI of the nailfold capillaries to provide insight into the oxygenation of the blood contained within the capillary network.

Standardized diagnosis and treatment of undifferentiated connective tissue disease and mixed connective tissue disease

Undifferentiated connective tissue disease (CTD) usually refers to patients who are presented with certain symptoms and signs related to CTD, and positive serological evidence of autoimmune diseases but don't fulfill any of the classification criteria for a certain CTD. Mixed CTD refers to patients who are presented with one or more clinical manifestations such as hand swelling, synovitis, myositis, Raynaud's phenomenon, and acrosclerosis. Patients with mixed CTD always have high-titer anti-nuclear antibodies (ANA) of speckled pattern and high-titer anti-U1 ribonuclear protein (RNP) antibody in serum, while with negative anti-Sm antibody. The update of diagnosis and treatment of undifferentiated CTD and mixed CTD lags behind other established CTD. There is a lack of evidence from randomized controlled trials or guidelines/recommendations on the treatment of undifferentiated CTD or mixed CTD. At present, the conventional therapy is mainly adopted according to the specific clinical manifestations of the disease. The standardized diagnosis and treatment of undifferentiated CTD and mixed CTD were drafted by the Chinese Rheumatology Association based on the previous guidelines and the progress of available evidence, so as to improve the management of these patients in China.

SIGLEC-1 in Systemic Sclerosis: A Useful Biomarker for Differential Diagnosis.

Systemic Sclerosis (SSc) is a clinically heterogeneous disease that includes an upregulation of type I interferons (IFNs). The aim of this observational study was to investigate the IFN-regulated protein Sialic Acid–Binding Ig-like Lectin 1 (SIGLEC-1) as a biomarker for disease phenotype, therapeutic response, and differential diagnosis in SSc. Levels of SIGLEC-1 expression on monocytes of 203 SSc patients were determined in a cross-sectional and longitudinal analysis using multicolor flow cytometry, then compared to 119 patients with other rheumatic diseases and 13 healthy controls. SSc patients higher SIGLEC-1 expression on monocytes (2097.94 ± 2134.39) than HCs (1167.45 ± 380.93; p = 0.49), but significantly lower levels than SLE (8761.66 ± 8325.74; p < 0.001) and MCTD (6414.50 ± 1846.55; p < 0.001) patients. A positive SIGELC-1 signature was associated with reduced forced expiratory volume (p = 0.007); however, we were unable to find an association with fibrotic or vascular disease manifestations. SIGLEC-1 remained stable over time and was independent of changes in immunosuppressive therapy. However, SIGLEC-1 is suitable for differentiating SSc from other connective tissue diseases. SIGLEC-1 expression on monocytes can be useful in the differential diagnosis of connective tissue disease but not as a biomarker for SSc disease manifestations or activity.

Interstitial lung disease in patients with connective tissue disease: Subtypes, clinical features and comorbidities in the Western Cape, South Africa.

Background Interstitial lung disease (ILD) is highly prevalent in patients with connective tissue disease (CTD) and is poorly characterised in South Africa. Objectives To describe the clinical, serological and radiological features of CTD-ILD and their associations in patients attending a tertiary referral hospital. Methods A cross-sectional study collating clinical, serological and radiological features of CTD-ILD in patients attending rheumatology and respiratory outpatient clinics in a tertiary referral hospital. ResultsOf 124 CTD-ILD patients, 37 (29.8%) had rheumatoid arthritis (RA), 32 (25.8%) systemic sclerosis (SSc) and 55 (44.4%) other autoimmune connective tissue diseases (OCTD). Most patients were female (86.3%), of mixed racial ancestry (75.0%), and the median age was 55 years. Nonspecific interstitial pneumonia (NSIP) was the most common ILD pattern (63.7%), followed by usual interstitial pneumonia (UIP) (26.6%). Overall, 60.5% were current or past smokers, 33.1% had previous pulmonary tuberculosis infection, and 75.6% had gastro-oesophageal reflux disease. Patients with RA were older, had similar frequencies of NSIP and UIP, and had significantly better pulmonary function tests than the SSc and OCTD groups. Within three years of CTD diagnosis, two-thirds of the SSc and OCTD patients and almost half of the RA patients had developed ILD. Clinical features, chest X-rays and pulmonary function tests correlated poorly with high-resolution computerised tomography (HRCT). No case of acute pneumonitis was documented in CTD-ILD patients treated with methotrexate (MTX). Conclusion We suggest routine HRCT in all newly diagnosed CTD patients, particularly those with SSc and OCTD, where more than two-thirds of the patients had developed ILD within three years of their CTD. The use of MTX was not associated with the development of acute pneumonitis in patients with ILD. Key points Clinical features, chest X-rays and pulmonary function tests correlated poorly with high-resolution computerised tomography (HRCT).Smoking, environmental toxins, gastro-oesophogeal reflux and previous pulmonary tuberculosis infection were significant comorbidities in CTD-ILD patients.Early screening of ILD with HRCT is recommended, particularly in SSc.Use of MTX before and after ILD diagnosis was not associated with acute pneumonitis.

Interstitial Lung Disease in Firefighters: An Emerging Occupational Hazard.

IntroductionOccupational risk factors for interstitial lung disease (ILD) are a remediable aspect of this progressive pulmonary disorder. The association between firefighting and ILD is unknown. Our objective was to assess the characteristics of firefighters with ILD from a large single-center ILD registry.MethodsThe University of Chicago ILD database was reviewed for patients with a history of firefighting. Clinical information was abstracted from the medical record. The prevalence rate ratio of firefighters in the database compared to the baseline prevalence of firefighting in the Chicago metropolitan area was calculated via the Poisson distribution.ResultsNineteen firefighters were identified; all were men. A variety of ILD subtypes were seen across the cohort, including four patients with a diagnosis of connective tissue disease. Patients had mild forced vital capacity (FVC) and moderate diffusing capacity for carbon monoxide (DLCO) decrements on presentation; three patients died and two received lung transplantation over an average follow-up time of 76 months. Firefighters were seen at a greater proportion in the ILD registry than in the general population with a prevalence rate ratio of 3.98.ConclusionsFirefighting was overrepresented in our cohort compared to the general population, suggesting that there may be a causative association between firefighting and the presence of ILD. The wide variety of ILD subtypes observed suggest that all ILD patients should be asked about their occupational history. Further investigation to identify occupational exposures and determine the benefit of remediation is needed.

Real World Evidence on the Availability of Anti-Nuclear Antibody (ANA) in Diagnosis of Connective Tissue Diseases; A Retropective Single Center Trial

Real World Evidence on the Availability of Anti-Nuclear Antibody (ANA) in Diagnosis of Connective Tissue Diseases; A Retropective Single Center Trial

A Retrospective Study on the Profile and Treatment Response of Patients with Mononeuritis Multiplex and Connective Tissue Diseases

Introduction: Though Mononeuritis Multiplex (MM) can be caused by many pathological conditions, vasculitis is the most important cause. Aim: To study clinical features of MM in patients with connective tissue diseases, the time of presentation of MM from disease onset, its association with disease activity, and functional outcome after treatment. Materials and Methods: A retrospective study was conducted at Outpatient Department (OPD) of Madras Medical College (Government Hospital), Chennai, Tamil Nadu, India, between April 2015 to April 2017. The study included medical records of 18 patients with connective tissue disease who had attended the OPD, with sensory and motor symptoms and who was also diagnosed to have MM. Paired t-test was used to find associations. Disease activity levels were determined using various measures and modified Rankin Score (mRS) was used to assess the response to treatment. Data analysis was done using Statistical Package for Social Sciences (SPSS) version 28.0. Results: Of the 18 study subjects 14 (77.8%) were 18-40 years of age, and 10 (55.6%) were females. The mean duration between the time of diagnosis of connective tissue disease and the development of MM was 18.17 months. Ulnar nerve was the most common nerve involved, 11 subjects (61.1%) had ulnar nerve involvement. Axonal neuropathy was present on nerve conduction studies in 17 (94.4%) of the study subjects and sensory symptoms on history were present in 100% of the study subjects. There was a statistically significant difference (p-value &lt;0.001) between the mRS before treatment (3.89) and after treatment (2.78) for a duration of six months. Conclusion: It was seen that the disease activity indices for connective tissue diseases were quite high at the time of development of MM, and starting treatment would helpimprovethe functional outcome as is evident by the difference in mRS.