IgG4-Related Disease: A Clinical Case Series From a Tertiary Care Center in India.

AIM: Immunoglobulin G4-related disease (IgG4-RD) is often an unrecognized, rare fibroinflammatory condition that can involve various organ systems. This study aimed to identify the different clinical patterns of this disease in a single center in North India. METHODS: Patients were diagnosed on the basis of published diagnostic criteria for IgG4-RD. Patients' presenting complaints; epidemiologic profiles; and laboratory, radiologic, and histologic findings along with the treatment and outcomes were collected and analyzed. RESULTS: In total, 70 patients were diagnosed with the disease. The female-to-male ratio was 0.94:1, and it increased with multiorgan involvement. The mean age of patients was 41.4 years, and the majority of the patients (65.7%) were younger than 50 years. Patients were diagnosed as possible (38.57%), probable (32.85%), and definite (28.57%) IgG4-RD. The incidence of the involvement of orbital and periorbital tissues was the highest (52.9%); however, 13% of the patients had multiple organ involvement. Patients with involvement of the retroperitoneal tissues and the lymph nodes were 8.5% and 5.7%, respectively. Increased serum IgG4 levels were found in 74.3% of the patients with single-organ involvement, whereas all patients with multiorgan involvement had increased IgG4 levels. The majority of patients (94.3%) required immunosuppressive medications along with corticosteroids. Azathioprine was the most commonly used (72.8%) immunosuppressive medication. Rituximab was used in 17.1% of the patients, of whom only one had multisystem involvement. CONCLUSIONS: This study depicts the most common patterns of organ involvement, along with the epidemiologic, laboratory, histologic, and radiologic data and response to treatment, in IgG4-RD, with a definite ophthalmology referral bias.

Ascending aorta aneurysm in scleroderma.

Ascending aorta aneurysm in scleroderma can be ascribed to its macrovascular involvement which is very less elucidated. We here describe a 56-year-old female with rapidly progressive diffuse cutaneous scleroderma. She presented with skin thickening involving all four limbs, thorax and abdomen. She had other features like arthritis, Raynaud's phenomena, dyspnoea, heaviness of chest, and dysphagia. On investigation, she was strongly positive for antinuclear antibody and Scl 70. Imaging revealed interstitial lung disease (nonspecific interstitial pneumonia pattern) and a fusiform dilatation of ascending aorta of 6.5 cm. Patient was offered surgical correction for aneurysm, for which she refused. To the best of our knowledge, our case report adds up to the few cases of ascending aorta aneurysm in scleroderma available in world literature.

Evaluation of reticulocyte hemoglobin for assessment of anemia in rheumatological disorders.

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.

Cogan syndrome: An autoimmune eye and ear disease with systemic manifestations.

Cogan syndrome (CS) is a rare vasculitis seen primarily in young adults. It predominantly affects eyes, ears and the heart with characteristic findings of interstitial keratitis, sensorineural hearing loss and vestibular dysfunction. A high index of suspicion is required to diagnose this rare disorder. It is one of the few vasculitis which can involve vessels of all sizes: small, medium and large. Coexistence of inflammatory bowel disease (IBD) in Cogan syndrome has been described in the literature. Immunosuppressive agents such as corticosteroids with or without steroid sparing agents are the standard of care. Early diagnosis and treatment are the cornerstone of treatment to prevent permanent damage to the ears and eyes. We describe a patient with Cogan syndrome with large vessel vasculitis and IBD. Our patient was treated with glucocorticoids and methotrexate.

Validity of Simplified Ankylosing Spondylitis Disease Activity Scores (SASDAS) in Indian Ankylosing Spondylitis Patients.

INTRODUCTION: Ankylosing Spondylitis Disease Activity Score (ASDAS) is a complex score for monitoring disease activity in Ankylosing Spondylitis (AS). AIM: To develop a simplified version of the ASDAS. MATERIALS AND METHODS: Consenting 254 AS patients (modified New York and/or Assessment in Ankylosing Spondylitis 2009 criteria) were recruited. Sociodemographic data and disease characteristics such as Bath AS Disease Activity Index (BASDAI), Bath AS Functional Index (BASFI), and AS Quality of Life (ASQoL), Erythrocyte Sedimentation Rate (ESR), and C-Reactive Protein (CRP) were collected. Simplified ASDAS (SASDAS) was calculated as the simple sum of patient global assessment, back pain (BASDAI question no.2), peripheral pain and swelling (BASDAI question no.3), morning stiffness (BASDAI question no.6), and either ESR in mm/hour (for SASDAS-ESR) or CRP in mg/L (for SASDAS-CRP); this sum was divided by 10 to obtain the final score. RESULTS: Most patients (224/254; 88.19%) were males with the median age of 30 years. SASDAS-ESR and SASDAS-CRP showed good correlation with ASDAS-ESR and ASDAS-CRP respectively (r2=0.78 and 0.58 respectively; p-value<0.0001). SASDAS-ESR showed good correlation with CRP (r=0.50) and is fairly correlated with backpain (r=0.19), morning stiffness (r=0.21) and peripheral pain (r=0.21); SASDAS-CRP showed good correlation with BASFI (r=0.32), and ESR (r=0.55) (all p-value<0.0001). Using established ASDAS cut-off values, corresponding cut-off points between 'inactive', 'moderate', 'high', and 'very high' disease activities (with optimum sensitivity and specificity) were 1.83, 2.45 and 4.45 for SASDAS-ESR, and 0.79, 1.50, and 3.26 for SASDAS-CRP. Both the SASDAS scores showed good correlation with BASDAI. CONCLUSION: SASDAS-ESR and SASDAS-CRP are reliable, easy-to-calculate scores for disease activity assessment in Asian Indian AS patients; which can be used in daily clinical practice.