scholarly journals Systemic sclerosis – metamorphosis of a life

2021 ◽  
Vol 30 (3) ◽  
pp. 115-120
Author(s):  
Andra-Patricia Stanciu ◽  
◽  
Laura Groseanu ◽  
Ruxandra Ionescu ◽  
◽  
...  

Systemic sclerosis is a complex autoimmune disorder marked by heterogeneous clinical manifestations and variable disease course. We present the case of a patient with diffuse cutaneous systemic sclerosis with anti-PM/Scl antibodies and associated calcinosis cutis. Currently, there is no uniformly effective therapy for calcinosis, but in the present case study combined therapy (calcium channel blocker, colchicine, bisphosphonate and minocycline) showed a good outcome with significant clinical improvement. Calcinosis in patients with systemic sclerosis is relatively common and it represents a challenge that requires appropriate management.

Author(s):  
Nandkishor Bhaurao Kale

Rajyakshma is oldest disease known to mankind as its first reference found in oldest literature Rugveda. It is called as King of Diseases, which indicates its severity. Tuberculosis remains major public health problem not only in India but also in world. According to WHO one third of global population is suffering from tuberculosis. Anti-tubercular therapy is long lasting and has many side effects, so it is necessary to develop adjunct therapy which will minimize the hazards and help patient to improve the immune status. Aims and objective – To evaluate the toxicity reduction and early restoration by adjunct therapy of Jivantyadi Ghrit.  Methodology - In the present case study DOTs therapy in the dose of 3 tablets once per day was given as an adjunct with Jivantyadi Ghrit 10 ml BD per day was administered for a period of 6 months in the diagnosed case of pulmonary tuberculosis. Assessment was recorded. Results – WBC count and ESR rate reduced to normal, Haemoglobin level increased, SGPT and Sr. Bilurubin level remains normal. Also significant resolution of lung Koch’s lesions was noted radiologically. Conclusion - Jivantyadi Ghrit as an adjunct therapy with DOTs reduces the side effects (especially hepatic) of DOTs therapy and also shows significant clinical improvement in sign and symptoms of PTB.


2021 ◽  
Vol 12 (4) ◽  
pp. 9-11
Author(s):  
Pooja J Kotian ◽  
Seetha P Devi

Hashimoto's thyroiditis is chronic inflammation of the thyroid gland due to the formation of autoantibodies. It is an autoimmune disorder that would lead to hypothyroidism. Failures of host defense do occur, however, and fall into three broad categories: immune deficiencies, autoimmunity and hypersensitivities. Ayurveda has a unique approach in treating the auto immune disorders through Shodhana and Rasayana Therapies. Due to Nidana Sevana, Kapha - pitta vata dushti takes place leading to Jatharagni Vaishamya and Ama Utpatti. This causes Asamyak Ahara Pachana, Rasavaha Srotodushti, Rasa Dhatwagni Vaishamya leads to Uttarottara dhatwagni and Dhatu Vaishamya. When Agni becomes too low, metabolism is affected. Shodhana karma has a great efficacy in Sroto-shodhana and in turn it corrects the functioning of Jatharagni, dhatwagni Srotas and Doshas. The present case study includes a female patient of 26 years age suffering from Hashimoto's thyroiditis complaints of gradual increase in size of swelling over neck for 3 years She was treated with Shodhana and Shamana Aushadhis for 3 months and found effective in reducing the levels of antibodies.


2016 ◽  
Vol 2016 ◽  
pp. 1-4
Author(s):  
Cody M. Lee ◽  
Diana Girnita ◽  
Arundhati Sharma ◽  
Surabhi Khanna ◽  
Jean M. Elwing

Systemic sclerosis is a rare autoimmune disorder with a wide spectrum of clinical manifestations and a multitude of autoantibodies that are associated with it. In the past several years, advances in serologic testing have led to research indicating important prognostic and phenotypic associations with certain subsets of autoantibodies. In particular, anti-RNA polymerase III (anti-RNAP III) has been associated with diffuse cutaneous disease, scleroderma renal crisis, a temporal relationship with malignancy, myositis, synovitis, joint contractures, and gastric antral vascular ectasia. However, anti-RNAP III has not been associated with systemic sclerosis sine scleroderma. We describe a patient with an atypical presentation of anti-RNAP III positive systemic sclerosis sine scleroderma who presented without the typical features of anti-RNAP III disease. Instead, she presented with critical digital ischemia, pulmonary arterial hypertension, gastroesophageal reflux disease, interstitial lung disease, and no clinically detectable sclerodactyly.


2020 ◽  
Vol 66 (1) ◽  
pp. 48-54
Author(s):  
Juliana Markus ◽  
Rogério de Melo Costa Pinto ◽  
Abadia Gilda Buso Matoso ◽  
Roberto Ranza

SUMMARY INTRODUCTION Systemic sclerosis (SSC) is an autoimmune disorder that affects several organs of unknown etiology, characterized by vascular damage and fibrosis of the skin and organs. Among the organs involved are the esophagus and the lung. OBJECTIVES To relate the profile of changes in esophageal electromanometry (EM), the profile of skin involvement, interstitial pneumopathy (ILD), and esophageal symptoms in SSC patients. METHODS This is an observational, cross-sectional study carried out at the SSC outpatient clinic of the Hospital de Clínicas of the Federal University of Uberlândia. After approval by the Ethics Committee and signed the terms of consent, 50 patients were initially enrolled, from 04/12/2014 to 06/25/2015. They were submitted to the usual investigations according to the clinical picture. The statistical analysis was descriptive in percentage, means, and standard deviation. The Chi-square test was used to evaluate the relationship between EM, high-resolution tomography, and esophageal symptoms. RESULTS 91.9% of the patients had some manometric alterations. 37.8% had involvement of the esophageal body and lower esophageal sphincter. 37.8% had ILD. 24.3% presented the diffuse form of SSC. No association was found between manometric changes and clinical manifestations (cutaneous, pulmonary, and gastrointestinal symptoms). CONCLUSION The present study confirms that esophageal motility alterations detected by EM are frequent in SSC patients, but may not be related to cutaneous extension involvement, the presence of ILD, or the gastrointestinal complaints of patients.


2020 ◽  
Vol 11 (1) ◽  
pp. 131-135
Author(s):  
Padma Priya Nakka ◽  
Praveen Kumar Madikonda ◽  
Johar B

Ayurveda described various skin diseases under the single heading Kushta. In Charaka Samhita, Acharya Charaka explained Ashtadasha Kushtas in two main categories namely ‘Maha Kushta’ and ‘Kshudra Kushta’. Kushta is tridoshaja vyadi, however, the symptoms appear according to the dosha predominance. Vipadika is one of the Kshudra Kushta with predominance of vata kapha doshas and it is charactrised by Pani Pada sputana (fissures in palms and soles) and Teevra vedana (severe pain). According to clinical manifestations, Vipadika is more similar to ‘Planto Palmar Psoriasis’ which is long lasting Auto Immune disease. It is found to be 3-4% of all Psoriasis cases. In modern science, many treatment modalities are available to treat the disease. They may cause many side effects and recurrence after subside is also very common which gave a big scope to alternative system of medicines to treat Vipadika. In the present case study, the line of management followed was mentioned by Acharya Charaka, according to dosha Predominance. The patient, initially administered sneha pana with Maha Tiktaka gritha for 7 days which was beneficial to pacify pitta dosha and kapha dosha utkleshana before Vamana. Later on, Vamana therapy was conducted to eliminate kapha dosha which gave good symptomatic relief to the patient. However, in view of the severity, chronicity and recurrence of the disease, Mridu Lavana jala Avagaha Sweda followed by Padhabyanga with pinda taila was also performed which resulted in excellent improvement in very short period of time.


2020 ◽  
pp. 239719832096196
Author(s):  
Alain Lescoat ◽  
Susan L Murphy ◽  
David Roofeh ◽  
John D Pauling ◽  
Michael Hughes ◽  
...  

Systemic sclerosis (systemic scleroderma) is characterized by a heterogeneous range of clinical manifestations. Systemic sclerosis is classified into limited cutaneous systemic sclerosis and diffuse cutaneous systemic sclerosis subgroups based on the extent of skin involvement. Randomized controlled trials in scleroderma have mainly focused on diffuse cutaneous systemic sclerosis partly because the measurement of skin involvement, critical for evaluating a therapeutic intervention, is more dynamic in this subset. Nonetheless, limited cutaneous systemic sclerosis, the most common cutaneous subset (about two-third), is also associated with significant morbidity and detrimental impact on health-related quality of life. The lack of interventional studies in limited cutaneous systemic sclerosis is partly due to a lack of relevant outcome measures to evaluate this subgroup. Combining several clinically meaningful outcomes selected specifically for limited cutaneous systemic sclerosis may improve representativeness in clinical trials and responsiveness of outcomes measured in randomized controlled trials. A composite index dedicated to limited cutaneous systemic sclerosis combining such relevant outcomes could advance clinical trial development for limited cutaneous systemic sclerosis by providing the opportunity to test and select among candidate drugs that could act as disease-modifying treatments for this neglected subgroup of systemic sclerosis. This proposed index would include items selected by expert physicians and patients with limited cutaneous systemic sclerosis across domains grounded in the lived experience of limited cutaneous systemic sclerosis. This article reviews the reasons behind the relative neglect of limited cutaneous systemic sclerosis, discusses the current state of outcome measures for limited cutaneous systemic sclerosis, identifies challenges, and proposes a roadmap for a combined limited cutaneous systemic sclerosis-specific treatment response index.


Author(s):  
Sreenithya.M ◽  
Neelakanta. J. Sajjanar ◽  
Sowmyashree.U.P ◽  
Gopalakrishna.G

Systemic sclerosis (SSc) is a multisystem autoimmune disorder caused by unknown factors. Which results in fibrosis of the skin, blood vessels, and visceral organs, including the gastrointestinal tract, lungs, heart, and kidneys. One among subset is referred to as diffuse cutaneous scleroderma and characterized by the rapid development of symmetric skin thickening of proximal and distal extremity, face, and trunk. When it affects on musculoskeletal system, the synovium in patients with arthritis is similar to that seen in early rheumatoid arthritis and shows edema with infiltration of lymphocytes and plasma cells. A 35-year-old house wife with this diagnosis came to OPD, which is managed by the principle of Amavata (diseases of connective tissue in Ayurveda) line of treatment. Vaitarana basti and Valuka sweda has done for 7 days with oral medication Vaishwanara churna ½ teaspoon with first morsal of food for 3 times. The QOL (quality of life) has been improved with in 7 days of treatment.


2015 ◽  
Vol 112 (49) ◽  
pp. 15136-15141 ◽  
Author(s):  
Ryosuke Saigusa ◽  
Yoshihide Asano ◽  
Takashi Taniguchi ◽  
Takashi Yamashita ◽  
Yohei Ichimura ◽  
...  

Systemic sclerosis (SSc) is a multisystem autoimmune disorder with clinical manifestations resulting from tissue fibrosis and extensive vasculopathy. A potential disease susceptibility gene for SSc is IFN regulatory factor 5 (IRF5), whose SNP is associated with milder clinical manifestations; however, the underlying mechanisms of this association remain elusive. In this study we examined IRF5-deficient (Irf5−/−) mice in the bleomycin-treated SSc murine model. We show that dermal and pulmonary fibrosis induced by bleomycin is attenuated in Irf5−/− mice. Interestingly, we find that multiple SSc-associated events, such as fibroblast activation, inflammatory cell infiltration, endothelial-to-mesenchymal transition, vascular destabilization, Th2/Th17 skewed immune polarization, and B-cell activation, are suppressed in these mice. We further provide evidence that IRF5, activated by Toll-like receptor 4 (TLR4), binds to the promoters of various key genes involved in SSc disease pathology. These observations are congruent with the high level of expression of IRF5, TLR4, and potential endogenous TLR4 ligands in SSc skin lesions. Our study sheds light on the TLR4-IRF5 pathway in the pathology of SSc with clinical implications of targeting the IRF5 pathways in the suppression of disease development.


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