A rare case of monoclonal gammopathy with autoinflammatory syndrome (schnitzler syndrome): A case report

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<ul><li><p>i n d i a n j o u r n a l o f r h e uma t o l o g y 9 ( 2 0 1 4 ) S 7eS 6 7S62negative Ro, La (ELISA). HRCT chest revealed mediastinal lymph-</p><p>adenopathy with Lymphocytic Interstitial Pneumonia (LIP)</p><p>pattern. Serum IgG4 levels and ACE levels were normal. Lip biopsy</p><p>revealed dense lymphocytic infiltrate with CD8 positivity on IHC.</p><p>HIV (ELISA) was positive with CD4 -176/uL and CD8 - 1769/uL.</p><p>Transbronchial lung biopsy was suggestive of DILS. She was</p><p>managed with steroids and HAART with complete resolution of</p><p>clinical features.</p><p>Conclusion: DILS is a uncommonmanifestation of AIDS with the</p><p>advent of HAART era. It is a Sjogren like syndrome with male</p><p>predominance, absence of arthritis and negative Ro/La serology.</p><p>This case presented as sicca symptoms in a young female with</p><p>predominant generalized lymphadenopathy which is not char-</p><p>acteristic of Primary Sjogren's syndrome (SS). Diligent in-</p><p>vestigations to rule out exclusions of SS are required to clinch</p><p>the diagnosis.P188. Study of Osteoporosis in a mixed (urban) population ofWest Bengal affected by the important precipitating factors</p><p>U.K. Samaddar; SambhunathPandit Hospital, Elgin Road, Kolkata,India</p><p>Introduction: We studied the distribution of Osteoporosis among</p><p>209 people from both urban and rural areas of West Bengal</p><p>affected by the important precipitating factors for the same.</p><p>Methods: The study was done between April 2006 to May 2013 209</p><p>(urban and rural) population, age:49 4 years, Male: Female ratioof 2:7, Hindu: Muslim - 4:3. Lifestyle parameters of the study</p><p>population were noted. The serum calcium and phosphorus level</p><p>were measured. BMD/ DEXA was done.</p><p>Conclusion: Females are mostly affected by osteoporosis. No</p><p>statistically significant difference of osteoporosis in perimeno-</p><p>pausal (405) versus post-menopausal (465) women in ruraland urban areas. Hindu males and females are more commonly</p><p>affected by osteoporosis than Muslim males and females.</p><p>P189. A rare case of monoclonal gammopathy withautoinflammatory syndrome (schnitzler syndrome): A casereport</p><p>V. Vasdeva, A. Bhayanab, S. Piplanic; aArmy Hospital R &amp;R , NewDelhi, India; bInstitute of Aerospace Medicine, Bangalore, India;cCommand Hospital Air Force, Bangalore, India</p><p>Introduction: Schnitzler's syndrome is an extremely rare syn-</p><p>drome characterized by Pyrexia of unknown origin (PUO), urti-</p><p>carial skin lesions, bone pains, raised inflammatory markers and</p><p>monoclonal gammopathy of undetermined significance (MGUS).</p><p>Methods: 46yrs old male presented with 6 months of quotidian</p><p>fever, transient arthritis, transient pruritic urticarial rashes,</p><p>myalgia, lymphadenopathy and splenomegaly in Dec 2012. The</p><p>extensive workup for PUO for infections, malignancy and con-</p><p>nective tissue disorders did not reveal any specific cause. He had</p><p>neutrophilic leukocytosis, high inflammatory markers. The bone</p><p>marrow aspiration showed a reactive marrow. However, the bone</p><p>marrow biopsy revealed plasma cell dyscrasia. Further evaluation</p><p>including Serum protein electrophoresis revealed IgA MGUS. He</p><p>was diagnosed as a case of MGUS with autoinflammatory syn-</p><p>drome. He was initially managed with steroid plus azathioprine</p><p>with which he showed almost 70% subjective improvements. The</p><p>symptoms recurred on reduction of steroid doses. Due to non-</p><p>availability of Anakinra (Anti IL1 Ra), he was started on anti IL-6receptor antibody (Tocilizumab). He has shown good response to</p><p>the treatment and is under regular follow up.</p><p>Conclusion: This association of MGUS and Autoinflammatory</p><p>syndrome (Schnitzler syndrome) is an extremely rare entity with</p><p>only a few case reports world over and the first being reported</p><p>from India. The long term prognosis and treatment modalities are</p><p>not well defined owing to rarity of this clinical condition and our</p><p>experiment with non-conventional therapy showed good results.</p><p>This case report would provide a good opportunity to discuss the</p><p>clinical profile and treatment modalities for this syndrome.</p><p>P190. Peripheral Ischemic lesions in rheumatology:Presentation and outcomes</p><p>U. Dhakada, Das S.K.a, Nolkha N.a, Sharma M.a, Bhadu D.a,</p><p>Wakhlu A.a, Kumar M.b, Srivastav D.a, Sharma A.a, Dhaon P.a,</p><p>Wakhlu A.a, Kumar P.a; aDepartments of Rheumatology andbRadiodiagnosis, King George's Medical University, Lucknow, India</p><p>Introduction: As Rheumatic diseases involve multiple organ sys-</p><p>tems, they can present with various extra-articular manifesta-</p><p>tions including peripheral ischemic lesions and gangrene.</p><p>Aims and objectives: To analyses incidence, presentation and</p><p>outcomes of peripheral ischemic lesions in rheumatology</p><p>practice.</p><p>Methods: All patients attending the Rheumatology department of</p><p>a tertiary teaching institution between March 2013 and February</p><p>2014 were assessed for any for peripheral ischemic lesions. Data</p><p>and outcomes were prospectively analysed.</p><p>Results: Total 34 patients presented with peripheral ischemic le-</p><p>sions, of which 29 (85%) had rheumatic diseases and 5 (15%) had</p><p>non - rheumatic cause for the ischemic lesion. In the group with</p><p>rheumatic diseases, mean age of presentation was 34 years, 24</p><p>were adults (with 83% females), 5 were children (with 60% fe-</p><p>males) and 82% presented in winter season. Early ischemic /</p><p>pregangrenous lesions were present in 47% cases while gangre-</p><p>nous lesions were present in 53% cases. Most common rheuma-</p><p>tological cause was vasculitis 13 (45%) either primary or</p><p>secondary, followed by systemic sclerosis 5 (17%). Other causes</p><p>were SLE with APS (14%), primary APS (7%), MCTD (7%) and un-</p><p>differentiated vasculitis (10%). Amputation (either surgical or</p><p>auto-amputation) was the outcome in 56% cases, while 44% le-</p><p>sions recovered completely with medical treatment.</p><p>Conclusions: Peripheral ischemic lesions may present either as</p><p>initial or predominant manifestation of a rheumatic disease. Fe-</p><p>males are affected more and most cases present in winters.</p><p>Vasculitis was the most common cause. Early diagnosis and</p><p>aggressive multidisciplinary management should be warranted</p><p>for optimal outcomes in these cases.</p><p>P191. A study on the role of ECG, echo and dipyridamolethallium scan in identifying early myocardial damage insystemic sclerosis</p><p>Sriram S.a, Madeshwaran M.a, S. Balameenaa,</p><p>Latha.J. Eupharasiaa, S. Rajeswarib; aDepartments of Rheumatologyand bImmunology, Madras Medical College, Chennai, India</p><p>Aim: To compare the effectiveness of electrocardiogram (ECG),</p><p>echo and thallium (Tl-201) scan in early detection of myocardial</p><p>ischemia and fibrosis in systemic sclerosis.</p><p>Methods: Tl-201 scan, ECG and echocardiogramwas performed on</p><p>30 systemic sclerosis patients of age 20-40 years (22 diffuse and 8</p><p>limited type) over a period of 1 year. The reports were compared to</p><p>http://dx.doi.org/10.1016/j.injr.2014.10.193http://dx.doi.org/10.1016/j.injr.2014.10.193</p><p>Study of Osteoporosis in a mixed (urban) population of West Bengal affected by the important precipitating factorsA rare case of monoclonal gammopathy with autoinflammatory syndrome (schnitzler syndrome): A case reportPeripheral Ischemic lesions in rheumatology: Presentation and outcomesA study on the role of ECG, echo and dipyridamole thallium scan in identifying early myocardial damage in systemic sclerosis</p></li></ul>


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