scholarly journals Osteolysis Affecting the Jaws in Systemic Sclerosis: Clinical and Osseous Changes Based on a Case Presentation

2015 ◽  
Vol 8 ◽  
pp. CMAMD.S30510 ◽  
Author(s):  
A. Delantoni ◽  
E. Matziari
Keyword(s):  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Panoramic Radiograph ◽  
Case Presentation ◽  
Medical Practitioners ◽  
Organ Systems ◽  
Similar Images ◽  
Osseous Changes ◽  
Condylar Head ◽  
Inflammatory Changes

Objectives The aim of the current paper is to present a case of systemic sclerosis of the jaws with all the characteristics and some extensive findings. Methods Systemic sclerosis is a connective tissue condition characterized by chronic inflammatory changes, presenting with a number of symptoms. The paper aims to present a case of systemic sclerosis that had some of the characteristics of the condition unilaterally. The features were prominent and well demarcated on the panoramic radiograph. Results The panoramic radiograph of the patient showed extensive condylar head resorption almost to the level of complete flattening. Discussion The rarity of the condition and the awareness the oral radiologist must have upon presentation of similar images is the main reason for the presentation of the case. The features the case presents are both characteristic and well demarcated. Finally, the authors try to alert the clinician, who should be mindful of the fact that sclerodermatous involvement of organ systems is so pleotropic, that it may include the oral cavity, which is not always thoroughly observed, and is often left unattended by medical practitioners.

Clinical Manifestations of Scleroderma

1995 ◽  
Vol 16 (2) ◽  
pp. 49-49
Author(s):  
Patricia L. Haber
Keyword(s):  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Localized Scleroderma ◽  
Unknown Etiology ◽  
Collagen Deposition ◽  
Muscle Fibrosis ◽  
Favorable Prognosis ◽  
Organ Systems

Scleroderma is a connective tissue disease of unknown etiology. Its most characteristic feature is thickening of the skin due to increased collagen deposition. However, the disease may involve multiple other organ systems. Two broad categories of scleroderma have been defined: localized and systemic. Although all forms of scleroderma are rare, localized scleroderma occurs more frequently than systemic sclerosis and has a more favorable prognosis. Several types of localized scleroderma exist. Morphea is characterized by the presence of one or more patches of hard, ivory-colored skin lesions. They begin with erythema and progress to nonpitting edema before becoming sclerotic. The margins of active lesions often have a violaceous hue. Underlying muscle fibrosis and atrophy may occur.

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

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1598.2-1599
Author(s):  
I. Rusu ◽  
L. Muntean ◽  
M. M. Tamas ◽  
I. Felea ◽  
L. Damian ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Systemic Sclerosis ◽  
High Resolution ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Connective Tissue Diseases ◽  
High Resolution Computed Tomography ◽  
Hrct Patterns

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

Histologic Study of Homograft Cartilages Implanted in the Middle Ear

1988 ◽  
Vol 98 (6) ◽  
pp. 546-551 ◽  
Author(s):  
Etsuo Yamamoto ◽  
Michitaka Iwanaga ◽  
Manabu Fukumoto
Keyword(s):  
Foreign Body ◽  
Connective Tissue ◽  
Middle Ear ◽  
Foreign Body Reaction ◽  
Fibrous Connective Tissue ◽  
Histologic Study ◽  
Second Stage ◽  
The Matrix ◽  
Inflammatory Changes ◽  
Histologic Changes

We examined conditions of the micro-sliced homograft cartilages implanted in the middle ear, implanted cartilages removed at revision surgery or implanted cartilages removed at the second stage of staged tympanoplasty, both macroscopically and histologically. Macroscopically, the appearance and shape of the cartilages remained unchanged, with no evidence of erosion. There was no evidence of any foreign body reaction or rejection phenomenon. In general, no marked histologic changes of the matrix tissues were found, although chondrocytes showed degenerative changes. There was partial absorption of cartilage and replacement by fibrous connective tissue when inflammatory changes occurred in the middle ear. It is concluded that implanted homograft cartilage maintains its stiffness for more than 6 months in a healthy, aerated middle ear and appears to be clinically useful for tympanoplasty.

scholarly journals A Case Report of Marfan Syndrome with Literature Review

2015 ◽  
Vol 4 (4) ◽  
pp. 269
Author(s):  
Hemanth Kumar Kalla ◽  
Swarna Kumari ◽  
CH Rama rao ◽  
MKR Parthasarathy ◽  
S Surya prakash Reddy ◽  
...  
Keyword(s):  
Case Report ◽  
Connective Tissue ◽  
Marfan Syndrome ◽  
Clinical Signs ◽  
Connective Tissue Disorder ◽  
Multiple Organ ◽  
Review Of The Literature ◽  
Organ Systems ◽  
Loin Pain ◽  
Routine Physical Examination

Marfan syndrome(MFS) is a connective tissue disorder that affects multiple organ systems. Cardiovascular, ocular, and skeletal abnormalities are cardinal features of the syndrome. Its incidence is among the highest of any heritable disorder.Most patients who have Marfan syndrome are usually diagnosed incidentally when they present for a routine physical examination for various reasons. The purpose of this paper is to provide a review of the literature, as well as describe a 22-year-old male with MFS and right hydroureteronephrosis diagnosed incidentally when he attended our hospital for complaints of fever and right loin pain. This case report emphasizes importance of “Revised Ghent criteria” for the diagnosis of MFS and highlights various clinical signs of MFS<strong>.</strong>

Autoimmune atrophic gastritis in systemic sclerosis

2021 ◽  
Vol 14 (8) ◽  
pp. e242851
Author(s):  
Deepti Avasthi ◽  
Jean Thomas ◽  
Leela Krishna Vamsee Miriyala ◽  
Salil Avasthi
Keyword(s):  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Literature Review ◽  
Atrophic Gastritis ◽  
Vascular Dysfunction ◽  
Connective Tissue Disorder ◽  
Gastric Antral Vascular Ectasia ◽  
Innate And Adaptive Immunity ◽  
Common Presentation ◽  
Autoimmune Atrophic Gastritis

Systemic sclerosis (SSc) is a rare connective tissue disorder with a complex pathogenesis involving vascular dysfunction, small vessel proliferation as well as alterations of innate and adaptive immunity. Gastrointestinal (GI) involvement in SSc is almost universal and affects nearly 90% of the patients. Of all the GI manifestations, 30%–75% are oesophageal abnormalities, including gastro-oesophageal reflux disease, reflux oesophagitis and Barret’s oesophagus. The incidence of gastric manifestations is about 22% with a common presentation of gastric antral vascular ectasia (GAVE). However, autoimmune atrophic gastritis (AIG) is not a known manifestation of SSc. Our case has a unique presentation of the coexistence of GAVE and AIG. We have conducted a thorough literature review to study a possible association of AIG and SSc and understand the pathology of SSc.

The Displays of Connective Tissue Dysplasia (Hypermobility) in Patients with Vertebral Syndrome of Lumbosacral Spine

2016 ◽  
pp. 149-154
Author(s):  
Oleksandr Piantkovskiy
Keyword(s):  
Connective Tissue ◽  
Varicose Veins ◽  
Pain Syndrome ◽  
Lumbosacral Spine ◽  
Duration Of Treatment ◽  
Medical Practitioners ◽  
Clinical Neurological Examination ◽  
Connective Tissue Dysplasia ◽  
Neurological Features

In recent years medical practitioners more often pay their attention on role of pathology of different organs and systems of human’s body which are associated with connective tissue dysplasia. The importance of this problem is caused by the great prevalence of connective tissue dysplasia, systemacity of damage, high probability of different diseases’ formation. Connective tissue dysplasia is the violation of the connective tissue structure during embryonic and postnatal periods because of genetically modified fibrillogenesis of extracellular matrix, leading to homeostasis disorder on tissue and organ levels with the progressive course. There was held the clinical neurological examination with 120 patients, who had neurological features of vertebral syndrome of lumbosacral spine. Analysis of the clinical examination results demonstrated that patients with vertebral syndrome of lumbosacral spine (p<0,05) more common can occur pathology of joints and varicose veins of the lower extremities. Significantly (p<0,05) (scoliosis, kyphosis, kyphoscoliosis, tendency to dislocation, stretching the ligaments) increase the duration of treatment and the expression of a pain syndrome. The results of examination and monitoring of patients in the dynamics of the treatment showed that patients with vertebral syndrome of lumbosacral spine (p<0,05) more often have anatomical changes in the lumbosacral spine than patients without evidence of DST.

Bone, joint and connective tissue disorders

2021 ◽  
pp. 245-252
Author(s):  
David Howell
Keyword(s):  
Rheumatoid Arthritis ◽  
Systemic Lupus Erythematosus ◽  
Systemic Sclerosis ◽  
Ankylosing Spondylitis ◽  
Connective Tissue ◽  
Musculoskeletal Disorders ◽  
Lupus Erythematosus ◽  
Anaesthetic Management ◽  
Systemic Lupus ◽  
Anaesthetic Practice

This chapter describes the anaesthetic management of the patient with those musculoskeletal disorders which are relevant to anaesthetic practice. Topics covered include rheumatoid arthritis, ankylosing spondylitis, systemic lupus erythematosus (SLE); systemic sclerosis; scoliosis and achondroplasia. For each topic, pre-operative investigation and optimisation, treatment, and anaesthetic management are described.

scholarly journals Hydrogen Sulfide: A Therapeutic Option in Systemic Sclerosis

2018 ◽  
Vol 19 (12) ◽  
pp. 4121 ◽  
Author(s):  
Amaal Abdulle ◽  
Harry van Goor ◽  
Douwe Mulder
Keyword(s):  
Hydrogen Sulfide ◽  
Systemic Sclerosis ◽  
Animal Studies ◽  
Therapeutic Option ◽  
Multiple Organ ◽  
Current Data ◽  
Organ Systems ◽  
Transsulfuration Pathway ◽  
Exact Etiology ◽  
Vascular Physiology

Systemic sclerosis (SSc) is a lethal disease that is characterized by auto-immunity, vascular injury, and progressive fibrosis of multiple organ systems. Despite the fact that the exact etiology of SSc remains unknown, oxidative stress has been associated with a large range of SSc-related complications. In addition to the well-known detrimental properties of reactive oxygen species (ROS), gasotransmitters (e.g., nitric oxide (NO), carbon monoxide (CO), and hydrogen sulfide (H2S)) are also thought to play an important role in SSc. Accordingly, the diverse physiologic actions of NO and CO and their role in SSc have been previously studied. Recently, multiple studies have also shown the importance of the third gasotransmitter H2S in both vascular physiology and pathophysiology. Interestingly, homocysteine (which is converted into H2S through the transsulfuration pathway) is often found to be elevated in SSc patients; suggesting defects in the transsulfuration pathway. Hydrogen sulfide, which is known to have several effects, including a strong antioxidant and vasodilator effect, could potentially play a prominent role in the initiation and progression of vasculopathy. A better understanding of the actions of gasotransmitters, like H2S, in the development of SSc-related vasculopathy, could help to create early interventions to attenuate the disease course. This paper will review the role of H2S in vascular (patho-)physiology and potential disturbances in SSc. Moreover, current data from experimental animal studies will be reviewed. Lastly, we will evaluate potential interventional strategies.

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

2013 ◽  
Vol 71 (Suppl 3) ◽  
pp. 343.1-343
Author(s):  
A. Soriano ◽  
D. Margiotta ◽  
B. Marigliano ◽  
P. Alemanno ◽  
M. Vadacca ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Pulmonary Fibrosis ◽  
Connective Tissue Diseases ◽  
Lung Involvement
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