Objective: The aim of this study was to evaluate the role of reticulocyte hemoglobin (Ret He) estimation in subtyping of anemia and to find the correlation of Ret He with the severity of anemia. Methods:Ninety-four patients with rheumatic diseases with anemia were enrolled.Blood samples were taken to determine various parameters. Patients were divided into three groups: iron deficiency anemia, anemia of chronic disease with iron deficiency and anemia of chronic disease depending on the iron status and inflammatory markers. Analysis of variance and Pearson correlation coefficient were used. Receiver operating characteristic analysis was used to evaluate the accuracy of the parameters in differentiating anemia. Results: Statistically significant differences among groups were seen with regard to parameters such as erythrocyte sedimentation rate, C-reactive protein, serum ferritin, total iron binding capacity, transferrin saturation, transferrin receptor protein, soluble transferrin receptor/log ferritin and Ret He. Ret He correlates with the subtype of anemia in patients with rheumatic disorders but it does not correlate with the severity of anemia. Soluble transferrin receptor/log ferritin, Ret He and serum ferritin values were the best parameters to differentiate between various groups. Ret He (pg) values of <24, 24-26.5 and >26.5, while serum ferritin levels (µg/L) of <35, 35-178 and >178 were highly sensitive and specific for iron deficiency anemia, anemia of chronic disease with iron deficiency and anemia of chronic disease groups, respectively. Conclusion: In cost constraints settings, a simple investigation like Ret He alone or with serum ferritin can help us to diagnose and differentiate between the different types of anemia accompanying rheumatological disorders without doing other serum iron studies and expensive tests like transferrin receptor protein which are not readily available. K E Y W O R D S assessment of anemia, log ferritin, Ret He, soluble transferrin receptor
Objectives: The aim of this study was to study the clinical profile of systemic lupus erythematosus (SLE) patients from a tertiary care center in Northwest Punjab. Materials and Methods: It was an observational cross-sectional study conducted at the Rheumatology Clinic, Adesh Hospital, Bathinda. Twenty-five patients classified to be suffering from SLE as per standard classification criteria (Systemic Lupus International Collaborating Clinics [SLICC]) were enrolled after obtaining consent for the same. Socio-demographic data, disease duration, disease activity, and treatment received were recorded. Analysis was performed for the various parameters. Results: The majority of patients (88%) were females with a female to male ratio of 7.3:1 with mean age of 30.5 years. Mucocutaneous involvement (92%) followed by musculoskeletal (84%). Nephritis was seen in 36%, deforming arthritis in 8%, and pleural involvement in 36% while ILD in 12%. Pericarditis was seen in 16%, and myocarditis was seen in 12%. Neurological involvement was seen in 36% patients with two cases of thrombotic CVA, one case of SAH and three cases of seizure disorder. Psychiatric symptoms were observed in 16% cases. AIHA was seen in 12%, leukopenia in 44%, and thrombocytopenia in 68%. Most common antibody was anti-dsDNA being present in 48% cases, followed by Anti Ro-60, Ro-52, Anti Sm, and Anti U1RNP antibody. Conclusion: We found a striking difference in the prevalence of pleuropulmonary features, neuropsychiatric features, Leukopenia, and thrombocytopenia in our subgroup of population as compared to the earlier studies from central and southern parts of India thus further emphasizing the fact that ethnic backgrounds predispose a patient for different phenotype.
Background:IgG4-related disease (IgG4-RD) is an often unrecognized, rare fibro-inflammatory condition that can involve various organ systems.Objectives:The aim of this study was to identify the different clinical patterns of this disease in a single centre in North India.Methods:70 patients were diagnosed on the basis of published diagnostic criteria for IgG4-RD. Patients’ presenting complaints, epidemiological profiles, laboratory, radiological and histological findings along with the treatment and outcomes were collated and analyzed from 2011 – 2019.Results:In a total of 70 patients who were diagnosed with the disease, the ratio of female to male ratio was 1:1. The mean age of patients was 41.4 years. Involvement of orbits and peri-orbital tissues was highest (52.9%) due to large number of referrals from ophthalmology services. 13% of patients had multiple organ involvement. Patients with involvement of retroperitoneal tissues and lymph nodes were 8.5% and 5.7%, respectively. Increased serum IgG4 levels were found in 74.3% of the patients. Histopathological examinations of the affected organs were performed in 44 (62.85%) patients and a diagnosis of possible (38.57), probable (32.85%) and definite (28.57%) IgG4-RD was made. Majority of the patients (94.3%) required immunosuppressive medications along with corticosteroids. Azathioprine was the most commonly used (72.8%) immunosuppressive medication. Rituximab was used in17.1% of the patients, of whom one had multisystem involvement.Conclusion:This study depicts the most common patterns of organ involvement along with the epidemiological, laboratory, histological, radiological data and response to treatment, in IgG4-RD, with a definite ophthalmology referral bias, in a tertiary care centre in North India.References:[1]Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med 2012; 366(6):539-51.[2]Khosroshahi A, CarruthersMN, Deshpande V, Unizony S, Bloch DB, Stone JH. Rituximab for the treatment of IgG4-related disease: lessons from 10 consecutive patients. Medicine 2012; 91(1):57-66.Table 1.Clinical and laboratory characteristicsCharacteristicsNumber of patients (N=70)Age (years)41.4Age group in years, n (%) >5024 (34.28) <5046 (65.71)Sex, n (%) Male36 (51.42) Female34 (47.22) ESR (mm/hr)24.37 CRP (mg/dL)12.31 Serum IgG (mg/dL)1214.2 Serum IgE (IU/L)587.2Type of IgG4 related disease, n (%) Definite20 (28.57) Probable23 (32.85) Possible27 (38.57)Data represented as mean, unless otherwise specified.CRP, C-reactive protein; ESR, erythrocyte sedimentation rate; Ig, immunoglobulin.Table 2.Distribution of patients according to organ involvement.Organ involvementNumber (%)Orbital and periorbital37(52.9)Retroperitoneum6 (8.5)Laryngotracheal tissue4 (5.7)Aorta and branches4 (5.7)Lymph nodes3(4.2)Paranasal sinus2 (2.8)Paravertebral tissue2 (2.8)Pancreas2 (2.8)Lung and bronchus2 (2.8)Eye (Scleritis)1 (1.4)Prevesical Mass1 (1.4)Ear polyp1 (1.4)Small Bowel1 (1.4)Primary sclerosing cholangitis1 (1.4)Musculoskeletal1(1.4)Submandibular gland1(1.4)Central nervous system1 (1.4)Multisystem involvement9 (12.86%)Figure 1.Right eye proptosisFigure 2.CT abdomen showing hydronephrosis due to retroperitoneal fibrosisDisclosure of Interests:None declared
IgG4-related disease (IgG4-RD) is a recently discovered (2003) complex disease, manifesting in various organs with symptoms mimicking other diseases. Progression of the disease leads to organ failure and hence early diagnosis is an urgent requirement in these patients. There is scarcity in reporting of IgG4-RD globally and in India. The aim of the study was to generate awareness on the epidemiology, diagnosis, and practice trends for IgG4-RD in India and globally, and to aid Indian physicians in early diagnosis of IgG4-RD in patients. Additionally, the evidence currently available in the Indian subpopulation has been evaluated. A preliminary literature search was performed using the PubMed database with the keywords including ‘IgG4-related disease’ in the title and abstract to obtain the relevant data. In total, PubMed identified 2071 publications comprising world-wide studies published in the English language before 30 April 2021. Studies were filtered region-wise by adding ‘India’ to the search strategy and total 60 publications were identified. The relative newness of IgG4-RD and the ensuing paucity in literature limits diagnosis by clinicians. Awareness of the disease among Indian clinicians would improve understanding of the disease and development of a country-specific consensus-based management guideline might lead to better prognosis in Indian patients with IgG4-RD.
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