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   Table of Contents - Current issue
December 2019
Volume 14 | Issue 4
Page Nos. 259-330

Online since Tuesday, December 31, 2019

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Pulmonary sarcoidosis – Predictors of relapse: Are we there yet? p. 259
Vineeta Shobha
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Lipidomics in psoriatic disease: The new kid on the omics block p. 261
Ashish J Mathew, Vinod Chandran
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Rheumatology in India: Yesterday, today, and tomorrow? p. 263
Robert J Moots
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Experience of sarcoidosis and factors predicting relapse at a tertiary care institute in North India p. 265
Alok Nath, Zia Hashim, Ajmal Khan, Mansi Gupta, Zafar Neyaz, Durga Prasanna Misra, Ravi Mishra, Shivani Srivastava
Background: Sarcoidosis is a multisystem, chronic granulomatous disease of unknown etiology. The clinical course of sarcoidosis is punctuated with remission and relapses, which is unpredictable. Materials and Methods: This was a retrospective observational study in which case records of all patients with a confirmed diagnosis of sarcoidosis attending the outpatient department of pulmonary medicine department between January 2014 and December 2018 were evaluated. The diagnosis of sarcoidosis was confirmed by demonstration of noncaseating granuloma in cytopathology or histopathology along with a compatible clinical picture. Mantoux test, serum angiotensin-converting enzyme (ACE) levels, serum calcium, and 24-h urinary calcium were done. Results: Sarcoidosis was found to be more common in females. Cough, breathlessness, fever, and fatigue were the predominant symptoms. Mantoux was negative in 95.83%, raised serum ACE was seen in 65.83%, hypercalcemia in 10.83%, and hypercalciuria in 30.0% of patients, respectively. Chest radiograph revealed that 14.17% of patients presented with Stage 1, 52.50% of patients with Stage 2, 15.83% of patients with Stage 3, and 17.50% of patients with Stage 4. Mediastinal lymphadenopathy was seen in contrast-enhanced computed tomography chest in 93.33%, septal thickening with nodularity in 61.67% and fibrosis in 25.83%. Corticosteroids were started in 97.5% patients and 24.17% patients relapsed after stopping or tapering corticosteroids. Conclusion: Sarcoidosis is a common cause of bilateral hilar lymphadenopathy and interstitial lung diseases. Relapses are frequent after stopping corticosteroids, so patients should be followed up actively after stopping treatment.
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Factors predicting early discontinuation of methotrexate as a first-line treatment for rheumatoid arthritis in Italy: Results from the GISEA registry p. 271
Andreina Manfredi, Marco Sebastiani, Florenzo Iannone, Elisa Gremese, Alessandra Bortoluzzi, Ennio Favalli, Roberto Gorla, Fausto Salaffi, Enrico Fusaro, Rosario Foti, Luca Cantarini, Roberto Caporali, Alberto Cauli, Stefano Alivernini, Francesco Paolo Cantatore, Antonio Carletto, Fabrizio Conti, Salvatore D'Angelo1, Oscar Epis, Roberta Ramonda, Antonio Marchesoni, Gianfranco Ferraccioli, Giovanni Lapadula
Objective: Despite the well-established efficacy of methotrexate (MTX) in rheumatoid arthritis (RA), monotherapy is not sufficient in almost half of patients. The aim of this registry-based study was to detect possible predictive factors for the early failure of MTX as a first-line treatment in early RA patients. Materials and Methods: Five-hundred and ninety RA patients beginning MTX as the first-line treatment were included. Persistence on therapy was re-evaluated after 12 months. Baseline features of disease were evaluated by means of univariate Cox regression, and parameters significantly associated to the outcome were included in multivariate model. Results: One hundred and forty-nine patients (25.3%) failed MTX during the 1st year, for inefficacy in 43.6% and adverse events in 37.5% of cases, respectively. At univariate analysis, patients who discontinued or failed treatment showed lower mean age, higher prevalence of anti-citrullinated peptide antibodies (ACPAs), and higher number of tender/swollen joints. The dose of MTX was correlated with the efficacy and the tolerance of the drug. In particular, patients treated with 7.5 mg of MTX weekly showed a higher rate of discontinuation for inefficacy than adverse events, and the contrary was detected for higher doses. On multivariate analysis, age, ACPA, and number of tender joints were directly associated with MTX discontinuation or failure. Conclusions: More than 25% of RA patients treated with MTX as a first-line therapy failed treatment at 12 months. ACPA positivity, age, and number of tender joints were associated with early withdrawal of MTX in RA patients, while the dose of MTX was correlated to the efficacy and safety of the drug.
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Charlson Comorbidity Index in patients with systemic lupus erythematosus in Egypt and its relation with disease characteristics p. 277
Samah M Alian, Sahar S Khalil, Rabab S Zaghlol
Background: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder associated with various comorbid conditions. Here, we assessed the burden of comorbidity in patients with SLE in Egypt using the Charlson Comorbidity Index (CCI) and determined its association with the disease characteristics. Patients and Methods: In this retrospective study, data of patients with SLE in Egypt were collected from the medical records. This included demographic data, clinical characteristics, comorbidities, and baseline SLE disease activity index (SLEDAI) and the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) scores at the last visit. The CCI score was calculated for each patient. The data of deceased patients were also collected. Results: Overall, 152 patients were included in this study. The most common comorbidities excluded from the CCI were serious infections (27%) and hypertension (23%); the most common comorbidities included in the CCI, apart from the connective tissue disease, were moderate or severe renal disease (12.5%), peptic ulcer (9.9%), and diabetes mellitus (9.9%) without end-organ damage. The mean CCI score was 1.9 and was significantly correlated with the disease duration, SLEDAI, and SDI. The death rate was 7.9%; the most common cause of death was infection (50%). Death was significantly associated with a high score of SLEDAI, SDI, and CCI. The mortality rate was higher in patients with SLE along with hypertension, renal affection, hyperlipidemia, infection, and tumors (P < 0.05). Conclusion: Patients with SLE in Egypt experienced comorbidities and high CCI scores that affected their survival.
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The increased erythrocyte membrane n-6/n-3 fatty acids ratio and inflammatory markers in patients with psoriasis p. 283
Serap Ozer Yaman, Asım Orem, Fulya Balaban Yucesan, Savas Yayli, Sadık Ozturk, Sevgi Bahadir
Background: Psoriasis is a common chronic, recurrent inflammatory skin disorder. Omega-3 fatty acids exhibit low inflammatory effects, while n-6 fatty acids display high inflammatory effects. The purpose of this preliminary study was to determine erythrocyte membrane n-6/n-3 fatty acid ratio values and their relationship with plasma inflammatory mediators in patients with psoriasis. Methods: The study groups consisted of thirty patients with psoriasis and 36 healthy controls. Analyses of fatty acids in the erythrocyte membrane were performed using gas chromatography and a flame ionization detector. Serum sample inflammation parameters (C-reactive protein [CRP]), erythrocyte sedimentation rate (ESR), and interleukin-6 (IL-6) were also analyzed. Results: The erythrocyte membrane n-6/n-3 fatty acid ratio was significantly higher in the psoriatic group compared to the healthy control group (P = 0.004). CRP, ESR, and IL-6 levels were also higher (P < 0.05) in the psoriatic group. The n-6/n-3 ratio was positively correlated with CRP and IL-6 levels in the psoriatic group (rho = 0.687, P= 0.0001 and rho = 0.542, P= 0.002, respectively). ROC curve analyses of CRP, ESR, IL6, and n6/n3 ratio values in patients with psoriasis performed similarly. Conclusion: This preliminary study indicates that erythrocyte membrane n6/n3 fatty acid ratio was increased and showed correlations with plasma CRP and IL-6 in patients with psoriasis. Receiver operating characteristic analysis indicates that the n6/n3 ratio (area underthe curve = 0.699) may be a suitable inflammatory marker for further exploration in psoriasis. Erythrocyte membrane n-6/n-3 fatty acid ratio may be used as one of the pro-inflammatory markers in psoriasis.
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The relationship between US7 ultrasound joint scoring system and disease activity score DAS28 in rheumatoid arthritis: A study in Indian population p. 290
Saurabh Maheshwari, Samar Chatterjee, Amar T Atal, DS Grewal, Vibhuti Maria
Introduction: US7 (7 joint ultrasound score) is an emerging ultrasound (US) joint scoring system for the assessment of disease activity in patients with Rheumatoid Arthritis (RA). Methods and Material: The study was conducted at a tertiary care center in Southern India. Sixty-two patients of RA were recruited for this cross-sectional observational study. Patients' clinical & laboratory parameters were used to calculate the disease activity score DAS28. Grayscale and Power Doppler Ultrasound (GSUS & PDUS) were performed to calculate US7 score.Statistical analysis used: Spearman's rank-order correlation, Pearson interclass correlation and ROC (receiver operating characteristic) curve analysis were performed. Results: Median DAS28 value was 4.04 with one patient in clinical remission. There was a statistically significant positive correlation (r=0.262) between synovitis by GSUS score & DAS 28 (p-value = 0.040) and synovitis by PDUS score and DAS 28 (r=0.340, p-value = 0.004). No significant correlation was found between DAS28 and tenosynovitis or erosions score. ROC curve analysis yielded the p values of 0.04 and 0.004 for synovitis by GSUS and synovitis by PDUS respectively in differentiating between the patients with low (DAS groups 1 & 2) and high disease activity (3 & 4). Conclusions: There is good correlation of the synovitis component of US7 score with DAS28. However, relationship of tenosynovitis and erosions with disease activity is questionable according to our results. An 'abbreviated US7' score with detection of only synovitis may be more suitable. This requires further validation.
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The effects of epigenetic regulation on phenotypic expressivity in Turkish patients with familial Mediterranean fever p. 297
Eser Dogan, Semra Gursoy, Giray Bozkaya, Secil Arslansoyu Camlar, Ozge Aksel Kilicarslan, Alper Soylu, Ayfer Ulgenalp, Salih Kavukcu, Ozlem Giray Bozkaya
Introduction: In this study, we aimed to characterize the effect of methylation on clinical diversity and gene expression levels in familial Mediterranean fever. Materials and Methods: Forty children, who were diagnosed with FMF according to the Tel-Hashomer criteria, were included in the study. The control group consisted of 32 healthy children. Demographic data, results of molecular studies, physical examination findings, number of attacks, response to treatment were recorded. To test the possibility that methylation is responsible for different phenotypic reflections of mutations, we classified FMF patients with respect to MEFV mutations and studied the differences of MEFV mRNA, pyrin and methylation levels between different subgroups. Results: MEFV mRNA expression levels were significantly lower in FMF patients, compared to control subjects (P = 0.049). Consistent with this observation, pyrin levels were significantly lower in FMF patients (P = 0.037). In addition, we found that MEFV methylation level was higher in FMF patients compared to control subjects (P = 0.376). Conclusions: There was no evidence that differences in methylation levels could be responsible for variable effects of different mutations and variable clinics in the MEFV gene. Further studies on larger patient groups are necessary to identify the effects of different factors.
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Sustained remission in large-Vessel vasculitis: Do they ever burn out? p. 304
Sakir Ahmed, Padmanabha Shenoy, Debashish Danda, Ramnath Misra
Drug-free remission is the ideal end point for any chronic disease. Although there are data on drug-free remission in rheumatoid arthritis and lupus, such data are limited for most vasculitis. Notably, there is less evidence for disease-modifying agents in large-vessel vasculitis (LVV). Thus, we explored the literature about sustained remission in LVV. MEDLINE and SCOPUS were searched for outcomes in LVV, and the results were manually screened for studies with at least 1-year follow-up. Isolated polymyalgia rheumatica was not included. In giant cell arteritis (GCA), histological and clinical remissions were discordant. Histology could not predict relapse rate. Various imaging techniques exhibit vessel wall inflammation in clinically quiescent disease. Relapse rate seems to correlate with the rate of steroid reduction. Relapse was rare when on higher steroid doses. Emerging evidence suggests that tocilizumab and methotrexate may prevent relapse. In Takayasu arteritis (TA), histology specimens are difficult to obtain. Remission on imaging does not mirror clinical remission. While magnetic resonance imaging and positron emission tomography are sensitive tools, these cannot differentiate smouldering disease from vascular repair. The best predictor of relapse is the extent of disease. Approximately half of the TA patients relapsed by 5 years. In patients undergoing intravascular procedures, restenosis occurred in around a third. Even for patients on anti- Tumour necrosis factor necrosis factor, sustained remission was in 20% only. LVV seems to be steroid dependent, and the efficacy of various steroid-sparing agents cannot be established unless the natural history of the disease is known. Both TA and GCA can have grumbling courses with relapse rates increasing over time.
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“Spontaneous pneumomediastinum in primary Sjogren's syndrome: An uncommon manifestation” p. 312
Viswesvaran Balasubramanian, Shibdas Chakrabarti, Abhijeet Singh, Nitesh Gupta
Sjogren's syndrome (SS) is the second most common autoimmune disorder, after rheumatoid arthritis that affects predominantly middle-aged women. Pulmonary involvement in SS can manifest as airway abnormalities, interstitial lung disease (ILD), and lymphoproliferative disorders. Spontaneous pneumomediastinum has been reported in ILD associated with various connective tissue diseases, most frequently inflammatory myopathies (dermatomyositis or polymyositis). However, the occurrence of spontaneous pneumomediastinum in SS is uncommon. We report a rare occurrence of spontaneous pneumomediastinum in a patient with primary SS.
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Hypocomplementemic urticarial vasculitis syndrome: A disease with many faces p. 317
Ankit Agrawal, Sangeetha Pabolu, Shirisha Ale, Yesenia Galan
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disease process where the exact pathogenesis is not known. It is an independent immunological condition affecting multiple organ systems. Major manifestations include chronic urticarial vasculitis (UV) with complement deficiency and demonstration of serum C1q antibodies. We describe a case of a 60-year-old male who presented with a generalized rash and was eventually diagnosed with HUVS. Usually, HUVS and systemic lupus erythematosus (SLE) have overlapping features, and our case was unique as our patient did not have any SLE manifestations. This article emphasizes the importance of comprehensive review of systemic manifestations accompanying UV. Although rare, it raises the awareness among the physicians about its clinical presentation and diagnostic approach and also predicts the prognosis of the same.
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Single-organ sarcoidosis involving hepatic, lacrimal, and Cardiac p. 321
BN Shiva Prasad, Ravi Puranik, Venugopal Anandaswamy, P Jayakumar
Sarcoidosis is a multisystem idiopathic granulomatous disease. Lung is the most common organ involved but can involve any organ. The skin, eyes, joints, and heart are affected in a significant fraction of patients. Extrapulmonary sarcoidosis may be the major manifestation of the disease. Extrapulmonary disease may manifest before, concurrent with, or after the development of pulmonary disease or as isolated independent disease. We present three cases of isolated organ sarcoidosis involving the liver, lacrimal, and heart. Isolated organ involvement is difficult to diagnose and the treatment of Isolated organ is not defined.
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Bone mineral density is decreased in patients with systemic sclerosis and correlates with serum intact parathormone levels: A cross-sectional study p. 325
Mahmood Dhahir Al-Mendalawi
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Response to comments on “bone mineral density is decreased in patients with systemic sclerosis and correlates with serum intact parathormone levels: A cross-sectional study” p. 326
Shefali Sharma, Aadhaar Dhooria, Tulika Singh
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Postmalignancy sarcoid-like reaction p. 327
Shiva Prasad, Nairutya Shivathirthan, Keyur Yogesh Patel
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Adiponectin and rheumatoid arthritis p. 330
Beuy Joob, Viroj Wiwanitkit
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