{"title":"Patterns of Pulmonary Involvement in an Egyptian Cohort with Autoimmune Diseases: A Descriptive Study","authors":"R. Mohammed","doi":"10.37532/1758-4272.2021.16(6).191","DOIUrl":"https://doi.org/10.37532/1758-4272.2021.16(6).191","url":null,"abstract":"Pulmonary involvement has been reported as a major contributor to significant morbidity and poor disease outcome in a myriad of autoimmune collagen vascular diseases. Aim of the work: To screen and to describe the different patterns of pulmonary involvement in a cohort of Egyptian patients with autoimmune diseases. Patients and methods: A cross sectional study conducted on patients attending the outpatient clinic as well as inpatient ward of rheumatology and rehabilitation and chest departments in Kasr Al Ainy teaching hospital- School of Medicine Cairo University in the period from January 2017 to January 2019. Approval and registration of the research by the institutional review board for research ethics was fulfilled prior to initiation of the study. HRCT chest was done to all patients using 16 channels MSCT in Kasr Al Ainy. Reconstructed axial, coronal and sagittal images were done to all patients; complementary mediastinal images were additionally taken. Results: Parenchymal lung disease in the form of interstitial pneumonitis with variable degrees of fibrosis (IPF) were encountered in 48% of the study cohort. Features suggestive of pulmonary hypertension with prominent PA diameter was found in 33% of cases. Mediastinal lymph node enlargement was noted in 34% of cases. Conclusion: Pulmonary involvement is not uncommon in patients with autoimmune diseases. High resolution CT chest remains the most sensitive technique for early detection of parenchymal lung disease in these patients.","PeriodicalId":13740,"journal":{"name":"International Journal of Clinical Rheumatology","volume":"33 1","pages":"191"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77691384","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Abida Hasan Do, S. Sharif, V. Capric, Madina Ray Aleks, E. Feoktistov
{"title":"Hyperviscosity Syndrome in Undifferentiated Connective Tissue Disease: A Case Report","authors":"Abida Hasan Do, S. Sharif, V. Capric, Madina Ray Aleks, E. Feoktistov","doi":"10.37532/1758-4272.2021.16(8).221","DOIUrl":"https://doi.org/10.37532/1758-4272.2021.16(8).221","url":null,"abstract":"Hyperviscosity Syndrome (HVS) is an oncologic emergency characterized by a triad of bleeding, visual disturbances and neurological deficits. Given its potentially fatal complications, the initial response in HVS is symptomatic treatment with a focus on decreasing blood viscosity to limit its serious clinical consequences. After symptomatic management with apheresis, the underlying cause of HVS must then be addressed. In this report, we present a case of a 45-year-old woman with extensive rheumatologic history admitted with syncope following several months of decreased oral intake. The patient had clinical signs of HVS and an M spike with elevated gamma globulins. She was treated with pulse dose steroids following plasma exchange and continued on a prednisone taper along with biologic therapy. This case is unique in that a patient with undifferentiated connective tissue disease, not previously documented, presented with hyper-viscosity syndrome secondary to increased IgG gamma globulins with a monoclonal spike, a presentation, to our knowledge, not yet described in the literature. The treatment regimen designed for this case was plasmapheresis with pulse dose steroids followed by rituximab-based therapy for induction with Mycophenolate mofetil and Hydroxychloroquine for maintenance therapy. While the use of rituximab has been associated with a clinically significant IgM flare, which would theoretically lead to worsening of hyperviscosity, in this case rituximab showed clinical effectiveness in the treatment of HVS.","PeriodicalId":13740,"journal":{"name":"International Journal of Clinical Rheumatology","volume":"50 1","pages":"221"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90272493","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Kimura disease and IgG4-related disease: Same disease spectrum or separate co-existing conditions?","authors":"T. Malley, A. G. Patel","doi":"10.37532/1758-4272.2021.16(1).028","DOIUrl":"https://doi.org/10.37532/1758-4272.2021.16(1).028","url":null,"abstract":"Here we report the case of a previously healthy 42-year old gentleman presenting with lacrimal, parotid and submandibular gland enlargement with associated pulmonary nodules, a pancreatic cyst and radiographic features of pancreatitis. Lacrimal gland biopsy indicated features in keeping with Kimura disease; however the clinical picture was not entirely typical. Prior to commencing treatment a further biopsy was sought from an alternative site, left submandibular gland, which confirmed features of IgG4-related disease. Although Kimura disease and IgG4-related disease share various clinical, serological and histopathological features; they are recognised as two distinct clinical entities. We discuss the possibility that the two conditions are on the same disease spectrum.","PeriodicalId":13740,"journal":{"name":"International Journal of Clinical Rheumatology","volume":"11 1","pages":"028"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87098764","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Nasereldin Hamednalla Elasha Hamednalla, El Agib, S. Ali, M. Essa
{"title":"Clinical presentation of psoriatic arthritis among sudanese: hospital based study","authors":"Nasereldin Hamednalla Elasha Hamednalla, El Agib, S. Ali, M. Essa","doi":"10.37532/1758-4272.2021.16(2).104","DOIUrl":"https://doi.org/10.37532/1758-4272.2021.16(2).104","url":null,"abstract":"Back ground: Psoriasis is a common papulo-squamous disorder affecting 2% of the population and is characterized by well demarcated red scaly plaques. Psoriatic Arthritis (PsA) may be defined as an inflammatory arthritis which is seronegative for Rheumatoid Factor (RF) and enthesitis that is associated with psoriasis. The aims of our study is to identify different clinical presentations and types of psoriatic arthritis. Methods: This is a descriptive cross sectional hospital based study conducted at Omdurman military hospital, Khartoum, Sudan, for a period of six months. The study included 96 patients confirm diagnosed by dermatologist as psoriasis, the data obtain with designed questionnaire contains the clinical presentations of the disease, lab result and radiological imaging, the study analyzed by SPSS v.20 by using one way a nova method to determine the P. value. Results: From the total number of patients with Psoriasis, more than one third was psoriatic arthritis, 6% of patients with psoriatic arthritis were oligo-arthritis joint involvements, 14% were polyarthritis, 9% were distal inter phalangeal joint, 9% were spondylitis/sacroillitis and the remaining 5% were arthritis mutilants. In conclusion: the study found out most common pattern of joint involvement in psoriatic arthritis is oligoarthritis, which is not known to be common in psoriatic arthritis previously.","PeriodicalId":13740,"journal":{"name":"International Journal of Clinical Rheumatology","volume":"204 1","pages":"104"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76199694","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Rare cause of pigmenturia from anti-hydroxy-3-methylglutarylcoenzyme; A reductase (HMGCR) induced muscle necrosis treated withimmunosuppression: A Case Report and literature review","authors":"E. John, P. Kelly","doi":"10.37532/1758-4272.2021.16(7).207","DOIUrl":"https://doi.org/10.37532/1758-4272.2021.16(7).207","url":null,"abstract":"Myositis and muscle necrosis can be associated with anti-HMGCR antibody in patients with prior HMG-CoA reductase exposure. There are isolated reports of muscle necrosis in patients with no prior history of statin exposure. We percent a case of severe muscle necrosis with no predisposing factors. Our patient had high anti-HMGCR antibody titers causing rhabdomyolysis, muscle necrosis requiring long-term immunosuppressive agents","PeriodicalId":13740,"journal":{"name":"International Journal of Clinical Rheumatology","volume":"83 1","pages":"207"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89850199","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
M. N. Drehmer, Gabriel Vaisam Castro, I. Pereira, Iliada Rainha de Souza Sara Emelie Lofgren
{"title":"Estrogen receptor alpha gene (ESR1)variant is associated with rheumatoidarthritis susceptibility and to particularclinical manifestations of the disease","authors":"M. N. Drehmer, Gabriel Vaisam Castro, I. Pereira, Iliada Rainha de Souza Sara Emelie Lofgren","doi":"10.37532/1758-4272.2021.16(5).139","DOIUrl":"https://doi.org/10.37532/1758-4272.2021.16(5).139","url":null,"abstract":"Objective: Several lines of evidence suggest a pivotal role of the estrogen pathway in several autoimmune-related responses and disease. The aim of this study was to investigate the association of a variant in ESR1 gene with RA susceptibility and expand the analysis to specific clinical manifestations of RA in a sample of Brazilian patients. Methods: 449 individuals (268 healthy controls and 181 RA patients) were genotyped for the polymorphism rs2234693 in the ESR1 gene with Taqman SNP probes. The patient’s group was composed by 153 female and 28 men. Association analysis were performed by logistic regression using SPSS software. Results: The rs2234693 variant is associated with RA in males and females (rs2234693-CC genotype OR = 2.21, p = 0.0032). The rs2234693-CC genotype was also associated with the presence of cardiopathy (OR = 2.40, p = 0.025), high blood pressure (OR = 2.50, p = 0.019) and osteoporosis (OR = 1.94, p = 0.031), and also with the presence of anti-CCP antibodies (rs2234693-C allele OR= 1.91, p = 0.024). Conclusion: ESR1 is associated with RA, as well as with the presence of anti-CCP antibodies and particularRA-associated clinical manifestations (cardiopathy, high blood pressure and osteoporosis). Our study presents the first evidence of association between ESR1 variant and RA.","PeriodicalId":13740,"journal":{"name":"International Journal of Clinical Rheumatology","volume":"10 1","pages":"139"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85138586","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Multiple exostosis disease","authors":"K. Janani","doi":"10.37532/1758-4272.2021.16(1).033","DOIUrl":"https://doi.org/10.37532/1758-4272.2021.16(1).033","url":null,"abstract":"Multiple exostosisdisease is a rare condition. It is characterized by the proliferation and the development of numerous bone swellings in the metaphysis of long bones with a possible abnormality in their shape and length. It preferentially affects the knees, shoulders, ankles and wrists. These lesions remain clinically asymptomatic, but the pain is the main complaint of patients. The phenotype of this disease varies greatly between patients. All bones that develop through endochondral ossification can be affected like long bones. Some localizations are rare but potentially serious such as the ribs, the spine or the pelvis due to the proximity to important structures. The most feared, although rare, complication is the transformation into chondrosarcoma, requiring a close monitoring. The treatment can be conservative or surgical. The treatment and excisional surgery may be indicated in the event of functional disorders or deformities.The greater knowledge of the pathophysiology of the disease makes it possible to consider potential therapeutic targets. We report a rare case with iliac bone localization, discovered in adolescence rather than childhood.","PeriodicalId":13740,"journal":{"name":"International Journal of Clinical Rheumatology","volume":"17 1","pages":"033"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78201744","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Z. Abay, NebiyuBekele, Fikadu Alemye, Endalkachew Belayneh, AbebeWorku Abilo Tadesse
{"title":"Diagnosis of sclerodermatomyositis in Ethiopian woman","authors":"Z. Abay, NebiyuBekele, Fikadu Alemye, Endalkachew Belayneh, AbebeWorku Abilo Tadesse","doi":"10.37532/1758-4272.2021.16(7).210","DOIUrl":"https://doi.org/10.37532/1758-4272.2021.16(7).210","url":null,"abstract":"Background: Sclerodermatomyosits is an overlap syndrome diagnosed when systemic sclerosis and dermatomyositis are observed in one patient, which develops simultaneously or sequentially. The American College of Rheumatology - European League against Rheumatism (ACR-EULAR) classification Criteria was implemented to define both diseases. Case presentation: A 36-year-old female patient was presented with excessive clumsiness and sleepiness of one year duration. She was diagnosed with hypothyroidism and was started on levothyroxine. She noticed associated white-blue-red discoloration of digits upon exposure to cold, diffuse skin thickening and tightness, dysphagia to solid foods, constipation, and difficulty of standing up from a toilet seat, holding objects, and combing hair. She was tired all the time, had muscle aches, and had lost her appetite and weight. On physical examination, vital signs were within normal limits. She had diffuse scleroderma-like skin changes with a salt-and-pepper appearance over extremities, neck and trunk. She had erythematic skin eruptions over the palpebrae (heliotrope rash), and depigmented skin lesions over the knuckles (Guttron’s sign), neck and chest (V-sign), and back (Shawl sign), rough and cracked hands (mechanic’s hands) and pitted fingertip scars. She had difficulty of getting out of a chair and lifting objects. Skeletal muscle enzyme was elevated and muscle biopsy revealed muscle fiber atrophy with collagenous fibrosis. Diagnoses of systemic sclerosis and dermatomyositis were settled by ACR-EULAR classification criteria. Conclusion: The patient described had sclerodermatomyositis using ACR-EULAR Classification Criteria. A high index of clinical suspicion is required to diagnose this rare disease in resource-limited settings to prevent adverse outcomes.","PeriodicalId":13740,"journal":{"name":"International Journal of Clinical Rheumatology","volume":"25 1","pages":"210"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77829543","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Successful switching to Infliximabafter systemic allergic reaction toAdalimumab drug: a case report","authors":"A. Sadeghi","doi":"10.37532/1758-4272.2021.16(5).158","DOIUrl":"https://doi.org/10.37532/1758-4272.2021.16(5).158","url":null,"abstract":"Subjective: To describe a case of systemic allergic reaction to Adalimumab drug in an Ankylosing Spondylitis (AS) patient and switching to Infliximab. Case report: A 43-year-old middleeastern man with a history of AS who was initiated with the treatment of Adalimumab. After the second injection of the drug immediate systemic allergic reaction with the symptoms of dizziness, shortness of the breath, angioedema of the limps and generalized rashes and itching happened. Discussion: Systemic reactions to Adalimumab although rare have been reported before. In case of allergic reaction to this drug desensitization and switching to another anti-TNFα have been successfully tried.","PeriodicalId":13740,"journal":{"name":"International Journal of Clinical Rheumatology","volume":"44 5 1","pages":"158"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86199259","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Metabolic syndrome,hematological markers of inflammation and disease activity in rheumatoid arthritis","authors":"A. Gaafar, H. Amer","doi":"10.37532/1758-4272.2021.16(2).052","DOIUrl":"https://doi.org/10.37532/1758-4272.2021.16(2).052","url":null,"abstract":"Background: The coexistence of Rheumatoid Arthritis (RA) and Metabolic Syndrome (MetS) is common. Aim: The present study assessed the effect of MetS on disease activity and hematological markers of inflammation to Platelets/Lymphocyte Ratio (PLR) and Neutrophil/Lymphocyte Ratio (NLR) in RA patients. Study design: A cross-sectional case control study using STARD reporting guideline. Methods: The present study included 5 groups: treatment-naive RA patients with MetS (GI, n=50), treated RA patients with MetS (GII, n=50), treatment-naive RA patients without MetS (GIII, n=50), treated RA patients without MetS (GIV, n=50) and healthy age and sex-matched controls (GV, n=50). RA was diagnosed based on the 2010 ACR/EULAR criteria. Disease activity of RA patients was calculated using the DAS-28 score. For the diagnosis of MetS, we adopted the Harmonized Joint Scientific Statement (HJSS) on metabolic syndrome recommendations. Results: Patients in GI had significantly higher DAS28 when compared with other groups. GII and GIII patients had significantly higher DAS28 when compared with GIV. It was also shown that GI patients had significantly higher PLR and NLR when compared with GIII. Similarly, GII patients had significantly higher PLR and NLR when compared with GIV. Logistic regression analysis identified presence only MetS [OR (95%CI): 8.66 (1.34-56.1), p=0.024] and increased PLR (OR (95%CI): 0.98 (0.96-0.99), p=0.002) as independent predictors of high disease activity in treatment-naive patients while increased PLR was the only independent predictor of disease activity in treatmentexperienced patients. Conclusion: Metabolic syndrome is associated with elevated hematological markers of inflammation and disease activity in treatment-naive RA patients. Both PLR and NLR are risk factors for RA activity in treatment-naive patients with PLR being more strongly correlated with disease activity.","PeriodicalId":13740,"journal":{"name":"International Journal of Clinical Rheumatology","volume":"PP 1","pages":"052"},"PeriodicalIF":0.0,"publicationDate":"2021-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84361544","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}