MECHANICAL VENTILATION FOR INTERSTITIAL LUNG DISEASE BY SYSTEMIC SCLEROSIS WITH CREST SYNDROME. PRESENTATION OF A CLINICAL CASE

INTRODUCTION. Interstitial lung disease (ILD) with acute respiratory failure needs ventilatory support poorly documented. One of the interstitial diseases known is the Systemic sclerosis, its advanced stages develop CREST syndrome. Faverio P, et al. (2016) suggested do not close the door to these patients and open the correct protocol, criticizing the little value that the scientic community concede to invasive mechanical ventilation (IMV). CASE REPORT. 85-year-old male is internalized in critical care unit by pneumonia, the complementary evaluation shows a systemic sclerosis disease with CREST syndrome and it is conrmed by elevation of anti-centromere antibody and positive skin biopsy. Tomography highlights pneumonic consolidation plus interstitial lung involvement and echocardiography reveals pulmonary hypertension. The management is done with IMV, keeping the goal of driving pressure less than 15 as lung protection, recovering respiratory function in 3 weeks. Discussion. The evidence is too insufcient to establish the best decision on IMV to the management of ILD.

Clinical and Molecular Features of Anti-CENP-B Autoantibodies

Centromeric proteins are the foundation for assembling the kinetochore, a macromolecular complex that is essential for accurate chromosome segregation during mitosis. Anti-centromere antibodies (ACAs) are polyclonal autoantibodies targeting centromeric proteins (CENP-A, CENP-B, CENP-C), predominantly CENP-B, and are highly associated with rheumatologic disease (lcSSc/CREST syndrome). CENP-B autoantibodies have also been reported in cancer patients without symptoms of rheumatologic disease. The rise of oncoimmunotherapy stimulates inquiry into how and why anti-CENP-B autoantibodies are formed. In this review, we describe the clinical correlations between anti-CENP-B autoantibodies, rheumatologic disease, and cancer; the molecular features of CENP-B; possible explanations for autoantigenicity; and, finally, a possible mechanism for induction of autoantibody formation.

Erasmus syndrome presented as CREST syndrome with Scl-70 positivity: a case report

Erasmus syndrome is a rare pathology defined as a systemic sclerosis secondary to contact with silica, associated or not with silicosis. More recent studies have related silica as an environmental factor stimulating different immune responses of the body. This report was made with the objective of presenting a rare case of systemic sclerosis, in a localized cutaneous form, also called CREST syndrome. A patient with a 20-year history of mining developed silicosis, cutaneous calcinosis, Raynauld's phenomenon, esophagopathy, sclerodactyly, telangiectasias and high positivity for the antitopoisomerase I antibody, the most common antibody in the systemic form.

A rare cause of dysphagia: CREST syndrome

<p class="abstract">Reporting a case of a 29-year-old female patient who came to the outpatient department with features of CREST syndrome. This case report illustrates the ENT presentation of CREST syndrome. A 29 year female with features of CREST syndrome (Calcinosis+, Raynaud’s phenomena +, oesophageal dysmotility+ sclerodactyly+ and Telangiectasias+, with no pulmonary hypertension) with ANA titres positive. Complete blood count, serum electrolytes, renal function tests, liver function tests, chest X-ray ,barium swallow ,ECG was done. Rigid oesophagoscope was used for the bougie dilatation of the stricture of oesophagus and was conservatively treated for her symptoms and being followed up at present. GI disturbances such as heartburn, dysphagia or respiratory complaints. Example: dyspnoea are occasionally the first manifestations of the disease. Dysphagia, manifested by various abnormal swallowing sensation, is intially caused by impaired oesphageal motility but later can result from gastroesophageal reflux disease and secondary stricture formation. Hereby concluding that any patient coming in OPD with similar features as mentioned above we should have a differential diagnosis of CREST syndrome and limited cutaneous scleroderma, yet it is a rare case.</p>

Addressing Stroke Risk in a Patient with CREST Syndrome and Atrial Fibrillation with Left Atrial appendage Occluder Device (WATCHMAN)

CREST syndrome is rare autoimmune disease causing calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly and telangiectasias. We present a case of an eighty-two year old female with CREST syndrome who presented to our clinic with atrial fibrillation and prohibitive bleeding risk. Managing stroke risk in atrial fibrillation is essential to minimize the morbidity and mortality of the condition. Those with CREST syndrome presenting with recurrent gastrointestinal bleeding may require alternatives to anticoagulation. Recently, the left atrial appendage occluder device became widely used to manage patients at increased risk for bleeding. The device provides a safe and efficacious alternative in lowering atrial fibrillation associated stroke risk. Our patient underwent uncomplicated implantation of the left atrial appendage occluder device. She was closely monitored for one year where she remained stroke free and had one minor episode of gastrointestinal hemorrhage.

Multiple Autoimmune Syndrome: An Unusual Combination of Autoimmune Disorders

Background: Autoimmune diseases are multifactorial with environmental and heritable factors. Autoimmunity reflects an altered immune status, therefore the presence of more than one disorder is not uncommon. The coexistence of three or more autoimmune diseases in a patient constitutes multiple autoimmune syndrome (MAS). This is an interesting case of a middle-aged female who had celiac disease, primary biliary cholangitis, autoimmune hepatitis and evolving CREST (Calcinosis, Rhaynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly and Telangiectasia) syndrome. Case Report: Fifty years old female patient presented with generalized fatigue, fever, weight loss, vertigo and constipation. She was a diagnosed case of celiac disease, and responded well to glutenfree diet. Family history was unremarkable for any autoimmune disorder. Laboratory workup showed normal complete blood counts, markedly elevated transaminases and alkaline phosphates. Her antinuclear antibodies (ANA) test was strongly positive (>1:320) and showed an anti-centromere pattern. Anti-extractable nuclear antibody(ENA) assay showed anti-mitochondrial and anti- CENP B antibodies. Liver biopsy revealed overlap syndrome (primary biliary cholangitis and autoimmune hepatitis). : This patient had celiac disease, primary biliary cholangitis and autoimmune hepatitis. Extensive immunological workup unexpectedly revealed the presence of anti-centromere protein B (anti-CENP B) antibodies which are strongly associated with CREST syndrome. Clinical re-evaluation of the patient gave clues of the evolving CREST syndrome. This case report highlights the importance of adequate immunological investigations in conjunction with clinical information for adequate patient management to achieve favorable consequences in the future. Conclusion: Patients suffering from an autoimmune disease need special attention as multiple immune- mediated disorders may be present simultaneously or sequentially during the course of the disease process. MAS patients are at a higher risk of acquiring infections and tumor development due to prolonged use of immunosuppressants. These patients need close surveillance for the development of another autoimmune disease, so as to control the current disease and to prevent future complications. This case report emphasizes the importance of a multidisciplinary team approach including an immunologist who may facilitate a better understanding of disorders related to the breakdown of immune tolerance.