a rare case of monoclonal gammopathy with autoinflammatory syndrome (schnitzler syndrome): a case...

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negative Ro, La (ELISA). HRCT chest revealed mediastinal lymph- adenopathy with Lymphocytic Interstitial Pneumonia (LIP) pattern. Serum IgG4 levels and ACE levels were normal. Lip biopsy revealed dense lymphocytic infiltrate with CD8 positivity on IHC. HIV (ELISA) was positive with CD4 -176/uL and CD8 - 1769/uL. Transbronchial lung biopsy was suggestive of DILS. She was managed with steroids and HAART with complete resolution of clinical features. Conclusion: DILS is a uncommon manifestation of AIDS with the advent of HAART era. It is a Sjogren like syndrome with male predominance, absence of arthritis and negative Ro/La serology. This case presented as sicca symptoms in a young female with predominant generalized lymphadenopathy which is not char- acteristic of Primary Sjogren's syndrome (SS). Diligent in- vestigations to rule out exclusions of SS are required to clinch the diagnosis. P188. Study of Osteoporosis in a mixed (urban) population of West Bengal affected by the important precipitating factors U.K. Samaddar; SambhunathPandit Hospital, Elgin Road, Kolkata, India Introduction: We studied the distribution of Osteoporosis among 209 people from both urban and rural areas of West Bengal affected by the important precipitating factors for the same. Methods: The study was done between April 2006 to May 2013 209 (urban and rural) population, age:49 þ 4 years, Male: Female ratio of 2:7, Hindu: Muslim - 4:3. Lifestyle parameters of the study population were noted. The serum calcium and phosphorus level were measured. BMD/ DEXA was done. Conclusion: Females are mostly affected by osteoporosis. No statistically significant difference of osteoporosis in perimeno- pausal (40 þ5) versus post-menopausal (46 þ5) women in rural and urban areas. Hindu males and females are more commonly affected by osteoporosis than Muslim males and females. P189. A rare case of monoclonal gammopathy with autoinflammatory syndrome (schnitzler syndrome): A case report V. Vasdev a , A. Bhayana b , S. Piplani c ; a Army Hospital R &R , New Delhi, India; b Institute of Aerospace Medicine, Bangalore, India; c Command Hospital Air Force, Bangalore, India Introduction: Schnitzler's syndrome is an extremely rare syn- drome characterized by Pyrexia of unknown origin (PUO), urti- carial skin lesions, bone pains, raised inflammatory markers and monoclonal gammopathy of undetermined significance (MGUS). Methods: 46yrs old male presented with 6 months of quotidian fever, transient arthritis, transient pruritic urticarial rashes, myalgia, lymphadenopathy and splenomegaly in Dec 2012. The extensive workup for PUO for infections, malignancy and con- nective tissue disorders did not reveal any specific cause. He had neutrophilic leukocytosis, high inflammatory markers. The bone marrow aspiration showed a reactive marrow. However, the bone marrow biopsy revealed plasma cell dyscrasia. Further evaluation including Serum protein electrophoresis revealed IgA MGUS. He was diagnosed as a case of MGUS with autoinflammatory syn- drome. He was initially managed with steroid plus azathioprine with which he showed almost 70% subjective improvements. The symptoms recurred on reduction of steroid doses. Due to non- availability of Anakinra (Anti IL1 Ra), he was started on anti IL-6 receptor antibody (Tocilizumab). He has shown good response to the treatment and is under regular follow up. Conclusion: This association of MGUS and Autoinflammatory syndrome (Schnitzler syndrome) is an extremely rare entity with only a few case reports world over and the first being reported from India. The long term prognosis and treatment modalities are not well defined owing to rarity of this clinical condition and our experiment with non-conventional therapy showed good results. This case report would provide a good opportunity to discuss the clinical profile and treatment modalities for this syndrome. P190. Peripheral Ischemic lesions in rheumatology: Presentation and outcomes U. Dhakad a , Das S.K. a , Nolkha N. a , Sharma M. a , Bhadu D. a , Wakhlu A. a , Kumar M. b , Srivastav D. a , Sharma A. a , Dhaon P. a , Wakhlu A. a , Kumar P. a ; a Departments of Rheumatology and b Radiodiagnosis, King George's Medical University, Lucknow, India Introduction: As Rheumatic diseases involve multiple organ sys- tems, they can present with various extra-articular manifesta- tions including peripheral ischemic lesions and gangrene. Aims and objectives: To analyses incidence, presentation and outcomes of peripheral ischemic lesions in rheumatology practice. Methods: All patients attending the Rheumatology department of a tertiary teaching institution between March 2013 and February 2014 were assessed for any for peripheral ischemic lesions. Data and outcomes were prospectively analysed. Results: Total 34 patients presented with peripheral ischemic le- sions, of which 29 (85%) had rheumatic diseases and 5 (15%) had non - rheumatic cause for the ischemic lesion. In the group with rheumatic diseases, mean age of presentation was 34 years, 24 were adults (with 83% females), 5 were children (with 60% fe- males) and 82% presented in winter season. Early ischemic / pregangrenous lesions were present in 47% cases while gangre- nous lesions were present in 53% cases. Most common rheuma- tological cause was vasculitis 13 (45%) either primary or secondary, followed by systemic sclerosis 5 (17%). Other causes were SLE with APS (14%), primary APS (7%), MCTD (7%) and un- differentiated vasculitis (10%). Amputation (either surgical or auto-amputation) was the outcome in 56% cases, while 44% le- sions recovered completely with medical treatment. Conclusions: Peripheral ischemic lesions may present either as initial or predominant manifestation of a rheumatic disease. Fe- males are affected more and most cases present in winters. Vasculitis was the most common cause. Early diagnosis and aggressive multidisciplinary management should be warranted for optimal outcomes in these cases. P191. A study on the role of ECG, echo and dipyridamole thallium scan in identifying early myocardial damage in systemic sclerosis Sriram S. a , Madeshwaran M. a , S. Balameena a , Latha.J. Eupharasia a , S. Rajeswari b ; a Departments of Rheumatology and b Immunology, Madras Medical College, Chennai, India Aim: To compare the effectiveness of electrocardiogram (ECG), echo and thallium (Tl-201) scan in early detection of myocardial ischemia and fibrosis in systemic sclerosis. Methods: Tl-201 scan, ECG and echocardiogram was performed on 30 systemic sclerosis patients of age 20-40 years (22 diffuse and 8 limited type) over a period of 1 year. The reports were compared to indian journal of rheumatology 9 (2014) S7 eS67 S62

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Page 1: A rare case of monoclonal gammopathy with autoinflammatory syndrome (schnitzler syndrome): A case report

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 7S62

negative Ro, La (ELISA). HRCT chest revealed mediastinal lymph-

adenopathy with Lymphocytic Interstitial Pneumonia (LIP)

pattern. Serum IgG4 levels and ACE levels were normal. Lip biopsy

revealed dense lymphocytic infiltrate with CD8 positivity on IHC.

HIV (ELISA) was positive with CD4 -176/uL and CD8 - 1769/uL.

Transbronchial lung biopsy was suggestive of DILS. She was

managed with steroids and HAART with complete resolution of

clinical features.

Conclusion: DILS is a uncommonmanifestation of AIDS with the

advent of HAART era. It is a Sjogren like syndrome with male

predominance, absence of arthritis and negative Ro/La serology.

This case presented as sicca symptoms in a young female with

predominant generalized lymphadenopathy which is not char-

acteristic of Primary Sjogren's syndrome (SS). Diligent in-

vestigations to rule out exclusions of SS are required to clinch

the diagnosis.

P188. Study of Osteoporosis in a mixed (urban) population ofWest Bengal affected by the important precipitating factors

U.K. Samaddar; SambhunathPandit Hospital, Elgin Road, Kolkata,India

Introduction: We studied the distribution of Osteoporosis among

209 people from both urban and rural areas of West Bengal

affected by the important precipitating factors for the same.

Methods: The study was done between April 2006 to May 2013 209

(urban and rural) population, age:49 þ 4 years, Male: Female ratio

of 2:7, Hindu: Muslim - 4:3. Lifestyle parameters of the study

population were noted. The serum calcium and phosphorus level

were measured. BMD/ DEXA was done.

Conclusion: Females are mostly affected by osteoporosis. No

statistically significant difference of osteoporosis in perimeno-

pausal (40þ�5) versus post-menopausal (46þ�5) women in rural

and urban areas. Hindu males and females are more commonly

affected by osteoporosis than Muslim males and females.

P189. A rare case of monoclonal gammopathy withautoinflammatory syndrome (schnitzler syndrome): A casereport

V. Vasdeva, A. Bhayanab, S. Piplanic; aArmy Hospital R &R , NewDelhi, India; bInstitute of Aerospace Medicine, Bangalore, India;cCommand Hospital Air Force, Bangalore, India

Introduction: Schnitzler's syndrome is an extremely rare syn-

drome characterized by Pyrexia of unknown origin (PUO), urti-

carial skin lesions, bone pains, raised inflammatory markers and

monoclonal gammopathy of undetermined significance (MGUS).

Methods: 46yrs old male presented with 6 months of quotidian

fever, transient arthritis, transient pruritic urticarial rashes,

myalgia, lymphadenopathy and splenomegaly in Dec 2012. The

extensive workup for PUO for infections, malignancy and con-

nective tissue disorders did not reveal any specific cause. He had

neutrophilic leukocytosis, high inflammatory markers. The bone

marrow aspiration showed a reactive marrow. However, the bone

marrow biopsy revealed plasma cell dyscrasia. Further evaluation

including Serum protein electrophoresis revealed IgA MGUS. He

was diagnosed as a case of MGUS with autoinflammatory syn-

drome. He was initially managed with steroid plus azathioprine

with which he showed almost 70% subjective improvements. The

symptoms recurred on reduction of steroid doses. Due to non-

availability of Anakinra (Anti IL1 Ra), he was started on anti IL-6

receptor antibody (Tocilizumab). He has shown good response to

the treatment and is under regular follow up.

Conclusion: This association of MGUS and Autoinflammatory

syndrome (Schnitzler syndrome) is an extremely rare entity with

only a few case reports world over and the first being reported

from India. The long term prognosis and treatment modalities are

not well defined owing to rarity of this clinical condition and our

experiment with non-conventional therapy showed good results.

This case report would provide a good opportunity to discuss the

clinical profile and treatment modalities for this syndrome.

P190. Peripheral Ischemic lesions in rheumatology:Presentation and outcomes

U. Dhakada, Das S.K.a, Nolkha N.a, Sharma M.a, Bhadu D.a,

Wakhlu A.a, Kumar M.b, Srivastav D.a, Sharma A.a, Dhaon P.a,

Wakhlu A.a, Kumar P.a; aDepartments of Rheumatology andbRadiodiagnosis, King George's Medical University, Lucknow, India

Introduction: As Rheumatic diseases involve multiple organ sys-

tems, they can present with various extra-articular manifesta-

tions including peripheral ischemic lesions and gangrene.

Aims and objectives: To analyses incidence, presentation and

outcomes of peripheral ischemic lesions in rheumatology

practice.

Methods: All patients attending the Rheumatology department of

a tertiary teaching institution between March 2013 and February

2014 were assessed for any for peripheral ischemic lesions. Data

and outcomes were prospectively analysed.

Results: Total 34 patients presented with peripheral ischemic le-

sions, of which 29 (85%) had rheumatic diseases and 5 (15%) had

non - rheumatic cause for the ischemic lesion. In the group with

rheumatic diseases, mean age of presentation was 34 years, 24

were adults (with 83% females), 5 were children (with 60% fe-

males) and 82% presented in winter season. Early ischemic /

pregangrenous lesions were present in 47% cases while gangre-

nous lesions were present in 53% cases. Most common rheuma-

tological cause was vasculitis 13 (45%) either primary or

secondary, followed by systemic sclerosis 5 (17%). Other causes

were SLE with APS (14%), primary APS (7%), MCTD (7%) and un-

differentiated vasculitis (10%). Amputation (either surgical or

auto-amputation) was the outcome in 56% cases, while 44% le-

sions recovered completely with medical treatment.

Conclusions: Peripheral ischemic lesions may present either as

initial or predominant manifestation of a rheumatic disease. Fe-

males are affected more and most cases present in winters.

Vasculitis was the most common cause. Early diagnosis and

aggressive multidisciplinary management should be warranted

for optimal outcomes in these cases.

P191. A study on the role of ECG, echo and dipyridamolethallium scan in identifying early myocardial damage insystemic sclerosis

Sriram S.a, Madeshwaran M.a, S. Balameenaa,

Latha.J. Eupharasiaa, S. Rajeswarib; aDepartments of Rheumatologyand bImmunology, Madras Medical College, Chennai, India

Aim: To compare the effectiveness of electrocardiogram (ECG),

echo and thallium (Tl-201) scan in early detection of myocardial

ischemia and fibrosis in systemic sclerosis.

Methods: Tl-201 scan, ECG and echocardiogramwas performed on

30 systemic sclerosis patients of age 20-40 years (22 diffuse and 8

limited type) over a period of 1 year. The reports were compared to