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P60 Andersson's lesion in patients with ankylosing spondylitis: a retrospective study
Abstracts of papers presented in IRACON-2010 P57 Exploring the pathogenesis of hypercoagulable state in antiphospholipid syndrome-role of anti-annexin A5 NK Singh, Anurag Gupta, Manoj Godara, Usha Department of General Medicine. Institute of Medical Sciences, Banaras Hindu University, Varanasi Background: Despite several attempts made during the last decade, the exact pathogenesis of exceedingly high thrombotic events and bad obstetric outcome in APS remains eluding. Anti-annexin A5 (aANX IgG) is thought to have a role in pathophysiology of APS. Methods: In this direction, we estimated level of aANX IgG in 82 patients of APS (60 primary and 22 secondary) of which 44 had bad obstetric outcome and 35 patients had thrombosis. We also estimated aANX IgG level in 40 age and sex matched healthy controls and 10 patients of SLE and RA each without any history of thrombosis or pregnancy morbidity. Estimation of aANX IgG was done by quantitative ELISA, using the Zymutest kit (Hyphen Biomed). Results: Scatterogram was plotted for individual values in healthy controls, SLE, RA, primary and secondary APS patients. We used values > mean + 2SD of healthy controls to be considered as positive. Only 3 healthy controls, 2 SLE (p = 0.239) and 1 RA patient (p = 0.794) had marginally elevated values, whereas 31 primary APS (p < 0.001) and 12 secondary APS (p < 0.001) were positive. Based on clinical parameters, 26 cases of bad obstetric outcome and 17 cases of thrombosis were positive (p < 0.05). We also compared aANX IgG level in different groups. Mean ± 2SEM of healthy controls was 3.77 ± 0.49, in SLE patients it was 4.88 ± 1.17 (p = 1.000) and in RA patients it was 4.67 ± 0.97 (p = 1.000). However, it was significantly elevated in primary APS (12.78 ± 1.52, p < 0.001), secondary APS (11.09 ± 1.58, p = 0.001), thrombosis group (12.72 ± 2.29, p < 0.001), bad obstetric outcome group (12.03 ± 1.20, p < 0.001) and total APS patients (12.33 ± 0.77, p < 0.001). Conclusions: From above observations it appears that aANX IgG play a significant role in producing hypercoagulable state in primary and secondary APS.
P58 Study of primary and secondary antiphospholipid syndrome— clinico-immunological profile and therapeutic outcome of 199 patients NK Singh, Anurag Gupta, DP Yadav, Manoj Godara, V Kumar, MN Singh, A Agarwal, M Matah, Usha Department of General Medicine. Institute of Medical Sciences, Banaras Hindu University, Varanasi Background: Antiphospholipid syndrome (APS) is a relatively new disease entity with variable clinical manifestations and controversial treatment approach especially during pregnancy. Methods: The study was conducted from January 2005 to July 2010, with follow-up of 1 to 5 years. A total of 199 APS patients (153 primary and 46 secondary), diagnosed as per updated Sapporo criteria, were recruited. Color-Doppler, CT scan and MRI cranium with venography were performed wherever indicated. Patients with thrombotic events were given heparin and oral anticoagulants whereas pregnant APS patients were given aspirin alone in 81 and along with LMWH in 24 patients from 8 to 34 weeks of gestation. Results: Twenty-six patients had arterial thrombosis, with involvement of intracranial artery in 13, small vessels in 7 (all having digital gangrene), visceral artery in 3 and peripheral medium size artery in 3 patients. Out of 3 patients having visceral artery involvement, 2 had acute MI while the third patient had acute abdomen with extensive gangrenous changes and sloughing of anterior wall of the stomach who recovered on treatment. Forty three patients had venous thrombosis affecting deep veins of lower limb in 21, cortical veins and/or cortical sinuses in 17 of which one patient had progressive vision loss with hydrocephalus, Budd-Chiari syndrome in 2, portal vein thrombosis in 2 patients and splenic vein thrombosis in 1 patient. One patient had fatal catastrophic APS who had extensive DVT of left lower limb, thrombosis in right brachial artery and occlusive stroke. One patient presented as intractable seizure with chronic demyelenating changes in brain and brain stem. Pregnancy morbidity was found in 136 patients which included recurrent early pregnancy loss in 31, late fetal loss (> 10 wk) in 95, still births in 21 and prematurity in 23 patients. One hundred sixty-four patients tested positive for IgG anticardiolipin antibody (> 20 GPL U), 89 for LA and 79 tested for both. Outcome of aspirin and LMWH, although costlier and tried in small number of patients, was superior to aspirin alone in pregnant APS patients. Conclusions: APS patients had varied thrombotic manifestations. Only 10 patients died. Combination of aspirin and LMWH was superior to aspirin alone in pregnant APS patients.
Poster presentations
S27
clinical features arthralgia, parotid enlargement, purpura were present in 1 (12.5%), 4 (50%), 2 (25%) of patients. All had ANA positivity with antibodies to SSA and SSB. None had low complements or cryoglobulinemia. Hyperglobulinemia was present in 4 (50%) patients. Minor salivary gland biopsy confirmed glandular inflammation with lymphocytic infiltrates in all. All of them had renal tubular acidosis with hypokalemia. One patient had nephrocalcinosis. None had proteinuria, hematuria. Biochemical osteomalacia was documented in 3 out of 4 (75%) patients. All of them improved with alkali and K + supplementation. Discussion: Renal manifestations of primary Sjogren’s syndrome may vary from 2% to 67% in various series which includes interstitial nephritis manifesting as dRTA and NDI, and less frequently as nephrocalcinosis, urolithiasis, and hypokalemia. Hypokalemic paralysis as initial presentation is extremely rare. Conclusion: All female patients with hypokalemic paralysis should be carefully screened for primary Sjogren’s syndrome and vice versa.
P60 Andersson’s lesion in patients with ankylosing spondylitis: a retrospective study Vishnu Sharma1, S Nagaraj1, S Sunil2, C Balakrishnan1 1
Department of Rheumatology, PD Hinduja National Hospital and MRC Mumbai, Department of Rheumatology, Kokilaben Dhirubhai Ambani hospital, Mumbai
2
Background: Andersson’s lesion (AL) is an important complication of patients with ankylosing spondylitis. AL may result from inflammation or (stress-) fractures of the complete ankylosed spine. There is paucity of Indian literature regarding this lesion especially the management aspect. We present the experience at our centre with patients with AS and AL. Methods: Retrospective analysis of the case-files of patients with AS and AL was done. The diagnosis of AS was based on ACR criteria. The demographic data of the patients including the age, sex, duration of disease, presenting features of discitis were noted. The modality of treatment adopted in each patient was also recorded. The outcomes of the treatment were analysed. Over a ten year period 20 patients were identified. Results: The mean age of patients was 42.35 years. There were 16 males and 4 females. The mean duration of disease was 16.9 years. 6 patients presented with sudden onset of sharp pain at thoracic region, 2 had diffuse back pain, other patients had insidious onset back pain and one patient had pain radiating to lower limbs with weakness. X-ray was the initial imaging modality in all patients and was diagnostic in 4 patient, other patients required MRI for diagnosis. 6 patients underwent surgery. Surgical intervention (stabilization and fixation) produced better results than conservative treatment in terms of symptom relief and function. Conclusion: AL is a rare but important complication of AS. It requires high degree of suspicion for diagnosis. Appropriate and timely intervention (including surgery) produces satisfactory results in terms of symptom relief and function.
P61 Pachydermoperiostosis with mitral valve prolapse—an unusual association M Mahesh, R Sunil Verrappa Department of Medicine and Radiology. JSS Hospital and Medical College, Mysore Case Report: Pachydermoperiostosis is a rare hereditary disorder characterized by digital clubbing, pachydermia (thickening of facial skin and scalp) and periostosis (swelling of periarticular tissue and subperiosteal new bone formation). Well known and common associations are polyarthritis, seborrhoea, eyelid ptosis and hyperhidrosis. Unusual associations reported in literature are bone marrow failure, cranial suture defects, gastric ulcer and female esutcheon. The association of Pachydermoperiostosis with heart failure has been reported recently. A case of pachydermoperiostosis with typical presentation and clinical features is being reported herewith in view of the presence of mitral valve prolapse which has not been reported previously as an association of Pachydermoperiostosis. Keywords: Pachydermoperiostosis, mitral valve prolapse
P62 Mono-ostotic paget’s disease: a very rare occurrence in India C Bammigatti, Karthik, W Mukta, AK Das Department of Medicine, JIPMER Puducherry
P59 Hypokalemic paralysis as initial presentation in female patients with primary Sjogren’s syndrome—a case series Paul T Antony, VS Negi, P Padhan, CB Mithun Division of Clinical Immunology, Department of Medicine. Jawaharlal Institute of Post Graduate Medical Education and Research, Puducherry Introduction: Primary Sjogren’s syndrome is a systemic autoimmune disease with varied clinical manifestations. Here we present a case series with hypokalemic paralysis as initial presentation in primary Sjogren’s syndrome which is a relatively uncommon manifestation. Patients Out of 8 patients, all were females with a mean age of 29.87 years. The mean: duration of disease was 1.25 years. All of them had history of sicca symptoms. Among the
Background: Paget’s disease of bone is a rare form of metabolic bone disease characterized by overactive osteoclastic bone resorption followed by a compensatory increase in disorganized osteoblastic new bone formation. Mono-ostotic form is seen only in 20% of Paget’s disease in western world. There are only 11 cases of mono-ostotic Paget’s disease described in literature from India. Case Report: A 47 years old labourer presented with insidious onset, progressive, mechanical left low back pain associated with difficulty in squatting & getting up for 6 months. Examination revealed antalgic gait, reduced range of movements in left hip and ill defined tender swelling over left posterior superior iliac spine region. Blood investigations revealed features consistent with chronic kidney disease and significantly elevated alkaline phosphatase level (1142 U/L). X-ray (Figure 1) and computed tomography (Figure 2) revealed a destructive lesion affecting both cortical and spongy bone of whole of left pelvis with diffuse lucent areas and linear densities in it, thinned out cortex, reduction of left hip joint
P58 Study of primary and secondary antiphospholipid syndrome— clinico-immunological profile and therapeutic outcome of 199 patients NK Singh, Anurag Gupta, DP Yadav, Manoj Godara, V Kumar, MN Singh, A Agarwal, M Matah, Usha Department of General Medicine. Institute of Medical Sciences, Banaras Hindu University, Varanasi Background: Antiphospholipid syndrome (APS) is a relatively new disease entity with variable clinical manifestations and controversial treatment approach especially during pregnancy. Methods: The study was conducted from January 2005 to July 2010, with follow-up of 1 to 5 years. A total of 199 APS patients (153 primary and 46 secondary), diagnosed as per updated Sapporo criteria, were recruited. Color-Doppler, CT scan and MRI cranium with venography were performed wherever indicated. Patients with thrombotic events were given heparin and oral anticoagulants whereas pregnant APS patients were given aspirin alone in 81 and along with LMWH in 24 patients from 8 to 34 weeks of gestation. Results: Twenty-six patients had arterial thrombosis, with involvement of intracranial artery in 13, small vessels in 7 (all having digital gangrene), visceral artery in 3 and peripheral medium size artery in 3 patients. Out of 3 patients having visceral artery involvement, 2 had acute MI while the third patient had acute abdomen with extensive gangrenous changes and sloughing of anterior wall of the stomach who recovered on treatment. Forty three patients had venous thrombosis affecting deep veins of lower limb in 21, cortical veins and/or cortical sinuses in 17 of which one patient had progressive vision loss with hydrocephalus, Budd-Chiari syndrome in 2, portal vein thrombosis in 2 patients and splenic vein thrombosis in 1 patient. One patient had fatal catastrophic APS who had extensive DVT of left lower limb, thrombosis in right brachial artery and occlusive stroke. One patient presented as intractable seizure with chronic demyelenating changes in brain and brain stem. Pregnancy morbidity was found in 136 patients which included recurrent early pregnancy loss in 31, late fetal loss (> 10 wk) in 95, still births in 21 and prematurity in 23 patients. One hundred sixty-four patients tested positive for IgG anticardiolipin antibody (> 20 GPL U), 89 for LA and 79 tested for both. Outcome of aspirin and LMWH, although costlier and tried in small number of patients, was superior to aspirin alone in pregnant APS patients. Conclusions: APS patients had varied thrombotic manifestations. Only 10 patients died. Combination of aspirin and LMWH was superior to aspirin alone in pregnant APS patients.
Poster presentations
S27
clinical features arthralgia, parotid enlargement, purpura were present in 1 (12.5%), 4 (50%), 2 (25%) of patients. All had ANA positivity with antibodies to SSA and SSB. None had low complements or cryoglobulinemia. Hyperglobulinemia was present in 4 (50%) patients. Minor salivary gland biopsy confirmed glandular inflammation with lymphocytic infiltrates in all. All of them had renal tubular acidosis with hypokalemia. One patient had nephrocalcinosis. None had proteinuria, hematuria. Biochemical osteomalacia was documented in 3 out of 4 (75%) patients. All of them improved with alkali and K + supplementation. Discussion: Renal manifestations of primary Sjogren’s syndrome may vary from 2% to 67% in various series which includes interstitial nephritis manifesting as dRTA and NDI, and less frequently as nephrocalcinosis, urolithiasis, and hypokalemia. Hypokalemic paralysis as initial presentation is extremely rare. Conclusion: All female patients with hypokalemic paralysis should be carefully screened for primary Sjogren’s syndrome and vice versa.
P60 Andersson’s lesion in patients with ankylosing spondylitis: a retrospective study Vishnu Sharma1, S Nagaraj1, S Sunil2, C Balakrishnan1 1
Department of Rheumatology, PD Hinduja National Hospital and MRC Mumbai, Department of Rheumatology, Kokilaben Dhirubhai Ambani hospital, Mumbai
2
Background: Andersson’s lesion (AL) is an important complication of patients with ankylosing spondylitis. AL may result from inflammation or (stress-) fractures of the complete ankylosed spine. There is paucity of Indian literature regarding this lesion especially the management aspect. We present the experience at our centre with patients with AS and AL. Methods: Retrospective analysis of the case-files of patients with AS and AL was done. The diagnosis of AS was based on ACR criteria. The demographic data of the patients including the age, sex, duration of disease, presenting features of discitis were noted. The modality of treatment adopted in each patient was also recorded. The outcomes of the treatment were analysed. Over a ten year period 20 patients were identified. Results: The mean age of patients was 42.35 years. There were 16 males and 4 females. The mean duration of disease was 16.9 years. 6 patients presented with sudden onset of sharp pain at thoracic region, 2 had diffuse back pain, other patients had insidious onset back pain and one patient had pain radiating to lower limbs with weakness. X-ray was the initial imaging modality in all patients and was diagnostic in 4 patient, other patients required MRI for diagnosis. 6 patients underwent surgery. Surgical intervention (stabilization and fixation) produced better results than conservative treatment in terms of symptom relief and function. Conclusion: AL is a rare but important complication of AS. It requires high degree of suspicion for diagnosis. Appropriate and timely intervention (including surgery) produces satisfactory results in terms of symptom relief and function.
P61 Pachydermoperiostosis with mitral valve prolapse—an unusual association M Mahesh, R Sunil Verrappa Department of Medicine and Radiology. JSS Hospital and Medical College, Mysore Case Report: Pachydermoperiostosis is a rare hereditary disorder characterized by digital clubbing, pachydermia (thickening of facial skin and scalp) and periostosis (swelling of periarticular tissue and subperiosteal new bone formation). Well known and common associations are polyarthritis, seborrhoea, eyelid ptosis and hyperhidrosis. Unusual associations reported in literature are bone marrow failure, cranial suture defects, gastric ulcer and female esutcheon. The association of Pachydermoperiostosis with heart failure has been reported recently. A case of pachydermoperiostosis with typical presentation and clinical features is being reported herewith in view of the presence of mitral valve prolapse which has not been reported previously as an association of Pachydermoperiostosis. Keywords: Pachydermoperiostosis, mitral valve prolapse
P62 Mono-ostotic paget’s disease: a very rare occurrence in India C Bammigatti, Karthik, W Mukta, AK Das Department of Medicine, JIPMER Puducherry
P59 Hypokalemic paralysis as initial presentation in female patients with primary Sjogren’s syndrome—a case series Paul T Antony, VS Negi, P Padhan, CB Mithun Division of Clinical Immunology, Department of Medicine. Jawaharlal Institute of Post Graduate Medical Education and Research, Puducherry Introduction: Primary Sjogren’s syndrome is a systemic autoimmune disease with varied clinical manifestations. Here we present a case series with hypokalemic paralysis as initial presentation in primary Sjogren’s syndrome which is a relatively uncommon manifestation. Patients Out of 8 patients, all were females with a mean age of 29.87 years. The mean: duration of disease was 1.25 years. All of them had history of sicca symptoms. Among the
Background: Paget’s disease of bone is a rare form of metabolic bone disease characterized by overactive osteoclastic bone resorption followed by a compensatory increase in disorganized osteoblastic new bone formation. Mono-ostotic form is seen only in 20% of Paget’s disease in western world. There are only 11 cases of mono-ostotic Paget’s disease described in literature from India. Case Report: A 47 years old labourer presented with insidious onset, progressive, mechanical left low back pain associated with difficulty in squatting & getting up for 6 months. Examination revealed antalgic gait, reduced range of movements in left hip and ill defined tender swelling over left posterior superior iliac spine region. Blood investigations revealed features consistent with chronic kidney disease and significantly elevated alkaline phosphatase level (1142 U/L). X-ray (Figure 1) and computed tomography (Figure 2) revealed a destructive lesion affecting both cortical and spongy bone of whole of left pelvis with diffuse lucent areas and linear densities in it, thinned out cortex, reduction of left hip joint