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