scholarly journals Positivity Rates of Tests Used at Immune Thrombocytopenia Diagnosis to Detect Associated Diseases. a Prospective Multicenter Cohort Study of 218 Patients

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 1367-1367
Author(s):  
Guillaume Moulis ◽  
Thibault Comont ◽  
Johanne Germain ◽  
Anaïs Essilini ◽  
Natacha Brun ◽  
...  
Keyword(s):  
Platelet Count ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Thyroid Dysfunction ◽  
Clinical Signs ◽  
Fetal Loss ◽  
Thyroid Stimulating Hormone ◽  
Multicenter Cohort Study ◽  
Associated Diseases ◽  
History Of

Abstract I ntroduction:The frequency ofimmune thrombocytopenia (ITP)-associated diseases detected at ITP onset is not well known. The positivity rates of tests performed at ITP onset in order to detect these diseases are discussed or unknown, leading to discrepancies among recommendations. The aim of this study was to compare the positivity rates of tests used at ITP diagnosis to detect ITP-associated diseases in the overall ITP population, in the presence of signs evocative of these diseases and in the absence of these signs. Methods:We studied all patients included between June 2013 and May 2016 in the CARMEN (CytopéniesAuto-immunes : Registre Midi-PyréneEN) registry. This multicenter registry is aimed at the prospective follow-up of all incident ITP adults in the French Midi-Pyrénées region (South of France, 3 million inhabitants). Each investigator prospectively follows every patient newly diagnosed for ITP in routine visit or hospital stay. ITP is defined in accordance with French guidelines: platelet count <150 x 109/L and exclusion of other causes of thrombocytopenia. Investigations performed at ITP diagnosis are recorded with their results. We assessed their positivity rates in the entire cohort and depending on the clinical and biological context. Patients with a cause of secondary ITP already known at ITP diagnosis were excluded for these calculations. Results:We included 218patients. Median age was 66 years (range: 18-96), 47.3% were female, 34 (15.8%) had a secondary ITP, 104 (48.2%) had bleeding signs, median platelet count was 18 x109/L (range: 1-135) and 144 (66.1%) were treated for ITP within the month following the diagnosis. Bone marrow examination was performed in 167patients. Fivemyelodysplastic syndromes (MDS) were found (3 refractory cytopenia with unilineage dysplasia and 2 with multilineage dysplasia). These 5 ITP patients had platelet count <15x 109/L and responded to corticosteroids. The positivity rate among patients with anemia or neutropenia was 6.6% (4/61 patients) and 0.9% in case of isolated thrombocytopenia (1/106). Among patients aged >60 years, these rates were 8.3% and 1.5%, respectively. Antinuclear antibodies (ANAs) were tested in 170 patients without known connective tissue disease and were positive (titer³1/160) in 73 (42.9%). The positivity rates were 40.0% incase of clinical signs of connective tissue disease and 41.8% otherwise. Antiphospholipid antibodies were positive in 7 patients (73 tested). The positivity rates were 11.1%in case of history of thrombosis or fetal loss and 9.4% in the absence of evocative context. The positivity rates ofdirectantiglobulintest (tested in 64 patients) were 30.4% in case of anemia or lowhaptoglobinlevel, and 7.3% otherwise. Serum protein electrophoresis was performed in 158 patients. None had a history of repeated or severe infections.Hypogammaglobulinemia(<5g/L) was detected in 1 patient over 76 with lymphopenia and in1 patient without lymphopenia. HCV, HBV and HIV were tested in respectively 143, 140 and 159 patients. No new infection was detected. Nine patients were tested for Helicobacter pylori infection and 3 were positive. One patient had symptoms evocative of gastritis and was positive for Helicobacter. Among the 7 patients without symptoms of gastritis, 2 were positive (28.6%). Thyroid stimulating hormone (TSH) was tested in 112 patients without history of thyroid dysfunction. The positivity rates were 50.0% in case of symptoms evocative of thyroid dysfunction and 7.3% otherwise. Conclusions: Except for ANAs (whose presence may lead to hydroxychloroquine prescription) andhypogammaglobulinemia, the positivity rates were at least three-fold higher in case of evocative context, discussing the systematic use of these tests. HIV, HCV and HBV infections revealed by ITP seem very rare in France. Disclosures Beyne-Rauzy: Celgene, Novartis: Honoraria.

scholarly journals 14. A severe case of connective tissue disease related interstitial lung disease in a young woman

2019 ◽  
Vol 3 (Supplement_1) ◽  
Author(s):  
Kate Grant ◽  
Patrick McShane ◽  
Kathryn Kerr ◽  
Martin Kelly ◽  
Philip Gardiner ◽  
...  
Keyword(s):  
Weight Loss ◽  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Lung Biopsy ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Connective Tissue Disorder ◽  
Young Age ◽  
History Of

Abstract Introduction Interstitial lung disease (ILD) can occur in any connective tissue disease, with varying degrees of respiratory clinical manifestations. In the majority of cases, patients have an established connective tissue diagnosis that precedes the development of ILD by many years. This discussion will focus on the unusual presentation of an 18 year old female admitted with a short history of weight loss and breathlessness. Investigations showed extensive established ILD with strongly positive autoantibodies, but in the absence of clinical signs of an underlying connective tissue disorder apart from Raynaud’s phenomenon. Case description 18-year-old female presented with a three-month history of unintentional 25kg weight loss, six weeks of fatigue/malaise, and a two-week history of worsening breathlessness. She was a student, non-smoker, with no past medical history except for class I obesity, and not on regular medications. On examination she had fine bibasal end-inspiratory crackles, SaO2 96% RA and Raynaud’s phenomenon was observed. Her CXR demonstrated bibasal consolidation. CT imaging identified bilateral symmetrical peripheral patchy ground glass opacities and patchy consolidation with basal predominance. Bloods revealed rheumatoid factor 491.2, anti-RNP A ab 7.91, anti-Sm ab > 8 and anti-chromatin ab 7.3, speckled ANA positive titre of 40, Complement C4 0.08, ESR 29 and HIV negative. Pulmonary function tests demonstrated a restrictive pattern FEV1 2.08L (72%), FVC 2.43L (73%), Ratio 85% and reduced transfer factor - DLCO 41%, KCO 61%. Ambulatory oxygen assessment showed desaturation to 77% RA. Bronchoscopy revealed inflamed airways and a bronchoalveolar lavage (BAL) cell count of 0.6 x 106 - 42% macrophages, 32% neutrophils, 24% eosinophils, 2% lymphocytes. At the local ILD MDT a differential diagnosis of LIP or NSIP was considered. Following discussion with rheumatology she was referred to the thoracic surgical team for lung biopsy. She proceeded to surgical biopsy of her right lung without complication. Unfortunately, she continued to experience worsening breathlessness and myalgia and she was commenced on prednisolone (40mg), with some radiological improvement but no symptomatic benefit. The pathology from her lung biopsy demonstrated significant fibrosis with scattered lymphoid aggregates, microscopic honeycombing with multiple fibroblastic foci and diffuse changes, in keeping with a fibrotic NSIP pattern. Her case was discussed at Freeman Hospital Newcastle ILD MDT who advised that her presentation was in keeping with a mixed connective tissue/lupus-related NSIP, and suggested commencing methylprednisolone, cyclophosphamide and rituximab. Discussion On initial assessment, the patient’s age and symptoms of rapid weight loss and profound exertional dyspnoea were concerning. Her resting oxygen saturations were satisfactory, but she became markedly hypoxic on ambulating short distances, indicating serious respiratory pathology. The initial CXR showed ‘faint patchy consolidation’, but CT scan showed extensive interstitial changes, accounting for her dyspnoea and desaturation on exertion. Further investigations including rheumatoid factor, anti-RNP and anti-Sm antibody were found to be strongly positive, suggesting an underlying mixed connective tissue disorder. However, the patient did not complain of any symptoms related to arthritis, SLE, systemic sclerosis or polymyositis and no positive clinical findings were noted on examination in support of these diagnoses. The BAL analysis was consistent with CT-ILD but not specific enough for diagnosis. A lung biopsy was performed on advice of the ILD lung MDT as the abnormalities on CT imaging could be in keeping with several pathologies with very different associated prognosis and management. The biopsy appearance correlated poorly with the cell count in BAL fluid. Discussion at local and regional ILD MDTs was particularly helpful given the severity of ILD and her young age. The ILD MDT provided a consensus of expert advice on optimal management and confirmed our concern about the extent of established fibrosis and the need for aggressive management. This obviously has significant implications for the patient in many ways, but particularly regarding fertility given her young age and she was therefore referred to the regional fertility clinic for counselling. Key learning points This was a particularly unusual case because the patient presented acutely at a very young age with established fibrotic damage on lung biopsy. It is also noteworthy that she presented so acutely with advanced ILD even though there were no positive clinical signs on examination, and no symptoms or signs of an underlying connective tissue disease. Lung biopsy is not routinely indicated in patients with progressive (respiratory) clinical manifestations of CT ILD, particularly in patients with an established diagnosis of rheumatoid arthritis or systemic sclerosis, as corticosteroids and/or immunosuppression are the mainstay of treatment regardless of the underlying CT pathology. However, lung biopsy is indicated where there is diagnostic uncertainty due to atypical presentations. In this case the biopsy findings were unexpected and resulted in a change to the initial management plan. Considerations about fertility and long term toxicity further complicated our choice of optimal therapy. This was a challenging case and highlighted the importance of multidisciplinary management of complex ILD cases. Discussions between local rheumatology, radiology and respiratory clinicians led to the decision that a biopsy was necessary. Subsequently the ILD MDT in the Freeman hospital provided clear expert guidance on in favour of a more aggressive treatment regimen than may have been otherwise initially considered. Conflict of interest The authors declare no conflicts of interest.

Pulmonary Hypertension Associated with Connective Tissue Disease

2019 ◽  
Vol 40 (02) ◽  
pp. 173-183 ◽  
Author(s):  
Hossam Fayed ◽  
J. Gerry Coghlan
Keyword(s):  
Rheumatoid Arthritis ◽  
Pulmonary Hypertension ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Best Practice ◽  
Reference Source ◽  
History Of ◽  
Pulmonary Arterial ◽  
Primary Physicians

Pulmonary hypertension (PH) is common in most forms of connective tissue disease (CTD); the prevalent type of PH depends on the particular CTD. Thus, pulmonary arterial hypertension (PAH) is dominantly associated with scleroderma, while postcapillary PH is most common in rheumatoid arthritis and lung disease-associated PH is typically found in myositis and sarcoidosis.Considerable expertise is required to identify, diagnose, and manage CTD-PH, as the primary physicians providing the majority of care for this population, rheumatologists, need a good working knowledge of CTD-PH, its rather subtle presentation, and how to access the necessary investigations to screen for and identify patients with PH. The role of the rheumatologist does not stop at diagnosis; in some conditions such as lupus, optimizing immunosuppression is key to the management of PH, and unlike simple idiopathic PAH, the natural history of CTD-PH is often punctuated by complications of the CTD rather than just events due to progression of PH or therapy-related adverse events.The aim of this article is to provide an overview of all forms of CTD-PH, and to provide an easy reference source on current best practice.

scholarly journals Interpretation of autoantibody positivity in interstitial lung disease and lung-dominant connective tissue disease

2013 ◽  
Vol 39 (6) ◽  
pp. 728-741 ◽  
Author(s):  
Daniel Antunes Silva Pereira ◽  
Alexandre de Melo Kawassaki ◽  
Bruno Guedes Baldi
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Current Knowledge ◽  
Pulmonary Involvement ◽  
Pathophysiological Role ◽  
History Of

The initial evaluation of patients with interstitial lung disease (ILD) primarily involves a comprehensive, active search for the cause. Autoantibody assays, which can suggest the presence of a rheumatic disease, are routinely performed at various referral centers. When interstitial lung involvement is the condition that allows the definitive diagnosis of connective tissue disease and the classical criteria are met, there is little debate. However, there is still debate regarding the significance, relevance, specificity, and pathophysiological role of autoimmunity in patients with predominant pulmonary involvement and only mild symptoms or formes frustes of connective tissue disease. The purpose of this article was to review the current knowledge of autoantibody positivity and to discuss its possible interpretations in patients with ILD and without clear etiologic associations, as well as to enhance the understanding of the natural history of an allegedly new disease and to describe the possible prognostic implications. We also discuss the proposition of a new term to be used in the classification of ILDs: lung-dominant connective tissue disease.

scholarly journals Clinical Implication of TSH Screening in Venous Thromboembolism Patients

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A826-A827
Author(s):  
Siroj Dejhansathit ◽  
Ana Marcella Rivas Mejia ◽  
Kenneth Nugent
Keyword(s):  
Cohort Study ◽  
Venous Thromboembolism ◽  
Thyroid Dysfunction ◽  
Chart Review ◽  
Medical Center ◽  
Prior History ◽  
Subclinical Hyperthyroidism ◽  
Multicenter Cohort Study ◽  
Increased Risk ◽  
History Of

Abstract Background: Venous thromboembolism (VTE) that have significant morbidity and mortality for patients in the community and hospital. A recent meta-analysis found a significantly increased risk of incidence VTE among patients with hyperthyroidism compared to patients without hyperthyroidism. To our knowledge, no study has attempted to explore whether screening for TSH levels in VTE patients leads to a diagnosis of undiagnosed thyroid dysfunction as VTE could be the first presenting symptom. Method: We conducted a retrospective cohort study and analyzed data of all patients treated at University Medical Center, Lubbock, Texas in 18-85 years of age with a diagnosis of DVT and/or PE in 2019. Qualitative chart review to identify cases of clinically significant TSH screening in VTE patients that leads to thyroid dysfunction diagnosis. Associations between variables tested using Student’s t-test, chi-square, and Fisher’s exact test. Results: Of total of 533 participants with diagnosis of VTE in 2019, 85 participants were included in the study. Seven participants (8.24%) were found to have high TSH level (&gt;4.2 mIU/mL). None of them was found to have low level of TSH. Participants in high TSH group were more likely to be female (71.43%) and Caucasian (71.43%). In high TSH group patients tended to have both PE and DVT diagnosis at the same admission (71.43%). Weight and BMI were significance higher than those with normal TSH level. Segna et al conducted a prospective multicenter cohort study on association between thyroid dysfunction and venous thromboembolism. The study measure thyroid hormones and thrombophilic biomarkers at 1 year after the acute VTE and follow for the recurrent VTE (rVTE). They found that after 20.8 months of follow-up, 9% developed rVTE. However, none of them was found in subclinical hyperthyroidism group. Furthermore, in their multi-variate analyses, the hazard ratio for rVTE was 0.80 (95%CI 0.23-2.81) subclinical hyperthyroidism compared with euthyroid participants. They concluded that subclinical hyperthyroidism may be associated with lower rVTE risks. Similarly, with Liviu study found hyperthyroidism was not associated with an increased risk of VTE. Qualitative chart review in our patients with high TSH resulted that none of them had history of tobacco use. One participant was on birth control pills with the history of cervical carcinoma. Conclusion:The association of thyroid dysfunction and the development of VTE is debated on the literature review. In our study we found multiple patients with high TSH level (8.24%) in VTE patients with no prior history of thyroid dysfunction. TSH could play an important role in hypercoagulable state. Subclinical hypothyroidism and/or hypothyroidism may induce a prothrombotic event. However, larger cohort studies with higher prevalence of high TSH participants are needed to prove a relationship between TSH level and VTE events.

scholarly journals Evolution of Mixed Connective Tissue Disease in 7 Years Old Child: Clinical Case

2020 ◽  
Vol 19 (3) ◽  
pp. 214-219
Author(s):  
Tamara P. Makarova ◽  
Khakim M. Vakhitov ◽  
Dina R. Sabirova ◽  
Dinara I. Sadykova ◽  
Liliya R. Khusnutdinova ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Lupus Erythematosus ◽  
Mixed Connective Tissue Disease ◽  
Clinical Case ◽  
Clinical Signs ◽  
Systemic Scleroderma ◽  
Systemic Diseases ◽  
Autoimmune Pathology ◽  
Short Period

Background. Mixed connective tissue disease (Sharp syndrome) is the rare chronic autoimmune pathology combining various features of systemic lupus erythematosus, systemic scleroderma, rheumatoid arthritis, dermatomyositis and high antibody titer to nuclear ribonucleoprotein. The mixed connective tissue disease may evolve into other systemic diseases over time. Description of any cases of mixed connective tissue disease and its evolution in Russian patients has not been published previously.Clinical Case Description. The results of observations of the child with clinical and immunological signs of the mixed connective tissue disease followed by the progression of systemic scleroderma symptoms and development of Sjogren's syndrome in the short period of time are presented in the article. Improvement (such as pain attenuation, increase in volume of movements in affected joints, decrease of Raynaud syndrome manifestations duration) was observed on treatment (methotrexate 10 mg/week with subsequent addition of prednisolone 0.75 mg/kg/day).Conclusion. Timely diagnostics of clinical signs of the systemic diseases debut is crucial for correct patient routing and for achieving of disease improvement.

scholarly journals THYROID ACROPACHY: A RARE MANIFESTATION OF GRAVES DISEASE IN JOINTS

2019 ◽  
Vol 5 (6) ◽  
pp. e369-e371 ◽  
Author(s):  
Nicolas Perini ◽  
Roberto Bernardo Santos ◽  
João Hamilton Romaldini ◽  
Danilo Villagelin
Keyword(s):  
Elevated Serum ◽  
Clinical Signs ◽  
Thyroid Stimulating Hormone ◽  
Graves Disease ◽  
Free T4 ◽  
Tissue Edema ◽  
Rare Manifestation ◽  
Small Joint ◽  
History Of ◽  
Hands And Feet

Objective: The objective of this report was to describe a patient with Graves acropachy, a rare manifestation of Graves disease (GD) that is clinically defined by skin tightness, digital clubbing, small-joint pain, and soft tissue edema progressing over months or years with gradual curving and enlargement of the fingers. Methods: The patient was evaluated regarding thyroid function (serum free T4 [FT4] and thyroid-stimulating hormone [TSH] quantifications) and autoimmunity biomarkers (thyroid receptor antibody [TRAb]) as well as radiographic investigation of the extremities. Results: A 52-year-old man presented with a history of thyrotoxicosis and clinical signs of Graves orbitopathy. Laboratory tests showed suppressed TSH (0.01 UI/L; normal, 0.4 to 4.5 UI/L) and elevated serum FT4 (7.77 ng/dL; normal, 0.93 to 1.7 ng/dL), with high TRAb levels (40 UI/L; normal, <1.75 UI/L). A diagnosis of thyrotoxicosis due to GD was made and the patient was treated with methimazole. After the patient complained of swelling in hands and feet, X-ray evaluation was conducted and established the thyroid acropachy. Conclusion: We present a case of a patient with GD associated with worsening extrathyroid manifestations during orbitopathy, dermopathy, and developed acropachy in hands and feet.

Connective Tissue Graft as a Predictable Curtain for Aesthetics: A Case Report

2021 ◽  
Vol 10 (1) ◽  
pp. 63-66
Author(s):  
Simant Lamichhane ◽  
Manoj Humagain ◽  
Asmita Dawadi
Keyword(s):  
Case Report ◽  
Connective Tissue ◽  
Dental Implants ◽  
Dental Implant ◽  
Clinical Signs ◽  
Coronally Advanced Flap ◽  
Connective Tissue Graft ◽  
Tissue Graft ◽  
Intact Bone ◽  
History Of

  Dental implant practice has now become a major choice for replacement of missing teeth in modern dentistry. Over the years, the success rate of dental implants has increased from 80-90% to 96-98%. However, due to lack of proper availability of soft tissue and hard tissue along with improper alignment of dental implants often pose aesthetic concerns in anterior aesthetic zone though the implant is fully osseointegrated with no signs of clinical mobility. This case report presents a case of a 21 years old male with a history of tooth loss due to trauma 8 months back and rehabilitation with dental implant 6 months back. On examination, mid-labial recession of around 3mm associated with #11 with no clinical signs of overlying inflammation was noted. Intact bone support was revealed by IOPAR. The recessed area around dental implant was managed with connective tissue graft and coronally advanced flap.

scholarly journals Perplexing case of lung mass perfectly mimicking a malignancy

2019 ◽  
Vol 12 (7) ◽  
pp. e229273
Author(s):  
Eid Humaid Alqurashi ◽  
Ahmed Sayeed ◽  
Hasheema Hasheem Alsulami ◽  
Hadeel Mashhour Al-Qurashi
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Wedge Resection ◽  
Biopsy Result ◽  
X Ray ◽  
Breath Sounds ◽  
History Of ◽  
Chest X Ray ◽  
The Right ◽  
Histopathology Examination

A 35-year-old man, a known asthmatic and with a history of smoking presented with a history of recurrent episodes of mild haemoptysis. On examination, there was decreased intensity of breath sounds on the right infraclavicular area. The chest X-ray and CT chest showed a mass in right upper lobe with nodules in the other lobe. The VAT showed large heavily vascularised mass with surface laden with multiple nodules. The wedge resection of the mass was taken and sent for histopathology examination. The biopsy result showed picture suggestive of connective tissue disease associated follicular bronchiolitis. The patient did not have any signs or symptoms of connective tissue disease. However he was positive for Rheumatoid factor, ANA, anti-RO, anti-CCP antibodies. He was started on steroids and azathioprine. After 6 months of treatment, the size of the mass and nodules reduced by 50% and ESR was reduced to 5 from 75.

Immunological Studies of the Placenta in Maternal Connective Tissue Disease

1999 ◽  
Vol 2 (1) ◽  
pp. 19-24 ◽  
Author(s):  
Jeanne Ackerman ◽  
Erick F. Gonzalez ◽  
Enid Gilbert-Barness
Keyword(s):  
Rheumatoid Arthritis ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Lupus Erythematosus ◽  
Fetal Loss ◽  
Second Trimester ◽  
Pathological Changes ◽  
Fibrin Deposition ◽  
Systemic Lupus ◽  
Immunofluorescence Studies

Maternal connective tissue disease is an important cause of second-trimester fetal loss. In order to assess the pathological changes in the placenta in maternal connective tissue disease, we reviewed the clinical histories and performed histologic and immunofluorescence studies on nine placentas: five from mothers with systemic lupus erythematosus (SLE), two from mothers with mixed connective tissue disease (MCTD), one from a mother with rheumatoid arthritis (RA), and one from a mother without prior known connective tissue disease. Excessive intervillous fibrin deposition and infarction were noted in all cases. Immunofluorescent and immunoperoxidase studies showed deposits of fibrinogen, IgG, IgM, IgA, and complement 3 (C3) localized to the trophoblast basement membrane (TBM). Electron microscopy documented thickening of the trophoblast basal lamina in three SLE placentas examined. The use of immunofluorescence may be enhanced further if antitrophoblast antibodies can be linked to placental compromise.

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