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Assessment of Extent of Damage in Patients with idiopathic inflammatory myositis on standard of care treatment
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P160. Myositis-specific and myositis associated autoantibodies in indian patients with inflammatory myositis Puja Srivastava, S. Dwivedi, Able Lawrence, Ramnath Misra; Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Department of Clinical Immunology, India Introduction: Data on Myositis Specific and Associated Autoantibodies (MSA and MAA) from Indian subcontinent is scarce. We studied the prevalence and clinical associations of MSAs and MAAs in patients with IIM. Methods: Clinical data were collected from patients with IIM(fulfilling Bohan and Peter Criteria). Sera were analysed for antibodies against SRP, Mi2, Jo1, PL 7, PL 12, EJ, OJ, Ro-52, Ku, Pm-Scl75 and PM-Scl100, using immunoblot assay (Euroimmune). Results: There were 124 patients with IIM (M:F 1:3.6), dermatomyositis(DM) 55, juvenile dermatomyositis(JDM) 22, polymyositis(PM) 25 and connective tissue disease associated myositis(CTD Myositis) 22. Mean disease duration was 10.9 months. ANA positivity was found in 84(68.9%) and MSA in 54(43.5%) patients. Among MSA, 26(20.9%) had anti-Mi2, 29(23.4%) had Antisynthetase (Jo1, PL-7, PL-12, EJ) and 6(4.8%) patients had anti-SRP antibodies. Distributions of MAAs were as follows: anti-Ro52 in 45(36.3%), anti-Ku and anti-PM-Scl75 in 13(10.5%) each and antiPM-Scl100 in 5(4%) patients. Anti-Mi2 antibodies were associated with adult DM (21/55, 38.2%;p< 0.0001) and increased risk for pharyngeal weakness (13/34,38.2%;p¼0.004). It was also associated with decreased risk for ILD (0/28;p¼0.001). ILD and mechanics hands were associated, both with anti-Jo1and anti-synthetase antibodies (16/28,57% and 14/22,63.6% respectively; p<0.0001). 4/ 6patients with anti-SRP antibody had poor response to multiple drugs. Conclusion: Higher prevalence of anti-Mi2 is probably related to higher proportion of patients with DM. We found absence of ILD in patients with anti-Mi2 antibody suggesting that it may protect against ILD. In Indian population also, anti-synthetase antibodies are associated with ILD and anti-SRP with poor response to treatment.
P161. Assessment of Extent of Damage in Patients with idiopathic inflammatory myositis on standard of care treatment Sukesh Edavalath, Puja Srivastava, Able Lawrence, Vikas Agarwal, Amita Aggarwal, Ramnath Misra; Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India Introduction: Inflammatory myositis is associated with significant long term disability and damage. Data on extent of damage in Indian myositis patients is scarce. Hence we did this study. Methods: Myositis damage scoring was done according to the International Myositis Assessment and Clinical Studies Group Myositis Damage Index (IMACS MDI) -2001. Extent of damage score (MDI Extent Score) is the sum of scores for the eleven individual organ systems with maximum score of 38. All patients who fulfilled the Bohan and Peter criteria of Idiopathic Inflammatory Myositis (IIM) having more than one year follow up were included. Results: Among the 31 subjects (22 females and 9 males), Dermatomyositis (DM), Polymyositis (PM) and Juvenile
Dermatomyositis (JDM) were 22, 6 and 3 respectively. The median age at diagnosis was 37 years (range 5-52) with median disease duration of 3 months (range 1.5-24) at presentation. The median disease duration at damage assessment was 3 years (range 1 e 24). Damage was present in 29/31 patients. The median MDI-Extent score was 3 (range 0-9). Damage occurred most frequently in muscular and endocrine system (15/31; 48% each). Menstrual irregularities were present in (11/15; 73%) of menstruating females. Hypertension and cataract were present in (9/31; 29%) and (7/31; 22%) of the subjects respectively. Age at diagnosis and gender did not correlate with the extent of damage. Conclusion: In our study more than ninety percent of patients with IIM accrue damage by median disease duration of 3 years.
P162. Distal RTA, chronic interstitial nephritis, hypokalemic periodic paresis, pancreatitis and endocrinopathy in pSS. Vikas Garg, Manish Kumar, B.K. Kundu, A.K. Gadpayle; Department of Medicine, PGIMER Dr. RML Hospital, New Delhi, India Abstract: A 32 year old married female presented with loose stools, vomiting & pain abdomen since 4 days, progressive weakness of all four limbs since 2 days with no history of fever, bowel/bladder dysfunction, sensory symptoms, back pain, trauma, and urine discoloration. She is known hypothyroid since 8 years, suffered 4 similar episode in last 3 years for which she was advised Kþ supplements & had B/L renal calculi since 2 years. On examination she had hypotonia, power was 0/5 all four limbs, all reflex were diminished. On investigations Hb 8.5gm%, TLC 9,000, urea 42 mg/dl, creatinine 1.4 mg/dl, Na 144, K 2.7, Cl 130, Ca 7.6, PO4 1.3, amylase 523, lipase 798. ABG showed metabolic acidosis with normal anion gap. Urine pH 7.5 with normal anion gap, albumin þþ, 30-40 RBC. USG showed bulky pancreas with increased echogenicity, GB sludge & multiple calculi in B/L kidneys with pelvicalyceal dilatation. Patient history was reviewed and she gave history of dryness of eyes, mouth & gritty sensations in eyes since 1 year. On investigation RF, ANA, SSA/SSB, CRP & anti-TPO were positive. IgA/IgG/IgM were raised. Serum PTH, Estradiol & C3/C4 were low. DEXA scan showed osteoporosis, IVP was suggestive of medullary sponge kidney. Schirmer's test was normal. Renal biopsy showed features of chronic tubulointerstitial nephritis & Lip biopsy showed lymphoepithelial sialadenitis consistent with Sjogren's syndrome. Conclusions: Renal involvement in Sjogren's uncommonly present as hypokalemic paralysis in absence of significant sicca symptoms. Multiple endocrinopathy including hypothyroidism & hypoparathyroidism can also occur.
P163. Clinical profile of primary sjogren's syndrome with hypokalemic periodic paralysis Nachiket Kulkarni, Naisar Nahar, Anuradha Venugopalan, Arvind Chopra; Centre for Rheumatic Diseases (CRD), Pune, India Introduction: Primary Sjogren's syndrome(pSS) with Hypokalemic Periodic Paralysis(HPP) is increasingly reported. Whether it's a protean manifestation or a different clinical subset is unraveled. Present study evaluates this clinical phenotype. Methods: Subjects of Primary Sjogren's Syndrome with Hypokalemic Periodic Paralysis (HPP) were identified retrospectively from database maintained at Centre for Rheumatic Diseases, Pune since 1996 with records of over 35000 patients. Clinical and investigations data was extracted pertaining to initial examination
i n d i a n j o u r n a l o f r h e u m a t o l o g y 9 ( 2 0 1 4 ) S 7 eS 6 7
P160. Myositis-specific and myositis associated autoantibodies in indian patients with inflammatory myositis Puja Srivastava, S. Dwivedi, Able Lawrence, Ramnath Misra; Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Department of Clinical Immunology, India Introduction: Data on Myositis Specific and Associated Autoantibodies (MSA and MAA) from Indian subcontinent is scarce. We studied the prevalence and clinical associations of MSAs and MAAs in patients with IIM. Methods: Clinical data were collected from patients with IIM(fulfilling Bohan and Peter Criteria). Sera were analysed for antibodies against SRP, Mi2, Jo1, PL 7, PL 12, EJ, OJ, Ro-52, Ku, Pm-Scl75 and PM-Scl100, using immunoblot assay (Euroimmune). Results: There were 124 patients with IIM (M:F 1:3.6), dermatomyositis(DM) 55, juvenile dermatomyositis(JDM) 22, polymyositis(PM) 25 and connective tissue disease associated myositis(CTD Myositis) 22. Mean disease duration was 10.9 months. ANA positivity was found in 84(68.9%) and MSA in 54(43.5%) patients. Among MSA, 26(20.9%) had anti-Mi2, 29(23.4%) had Antisynthetase (Jo1, PL-7, PL-12, EJ) and 6(4.8%) patients had anti-SRP antibodies. Distributions of MAAs were as follows: anti-Ro52 in 45(36.3%), anti-Ku and anti-PM-Scl75 in 13(10.5%) each and antiPM-Scl100 in 5(4%) patients. Anti-Mi2 antibodies were associated with adult DM (21/55, 38.2%;p< 0.0001) and increased risk for pharyngeal weakness (13/34,38.2%;p¼0.004). It was also associated with decreased risk for ILD (0/28;p¼0.001). ILD and mechanics hands were associated, both with anti-Jo1and anti-synthetase antibodies (16/28,57% and 14/22,63.6% respectively; p<0.0001). 4/ 6patients with anti-SRP antibody had poor response to multiple drugs. Conclusion: Higher prevalence of anti-Mi2 is probably related to higher proportion of patients with DM. We found absence of ILD in patients with anti-Mi2 antibody suggesting that it may protect against ILD. In Indian population also, anti-synthetase antibodies are associated with ILD and anti-SRP with poor response to treatment.
P161. Assessment of Extent of Damage in Patients with idiopathic inflammatory myositis on standard of care treatment Sukesh Edavalath, Puja Srivastava, Able Lawrence, Vikas Agarwal, Amita Aggarwal, Ramnath Misra; Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India Introduction: Inflammatory myositis is associated with significant long term disability and damage. Data on extent of damage in Indian myositis patients is scarce. Hence we did this study. Methods: Myositis damage scoring was done according to the International Myositis Assessment and Clinical Studies Group Myositis Damage Index (IMACS MDI) -2001. Extent of damage score (MDI Extent Score) is the sum of scores for the eleven individual organ systems with maximum score of 38. All patients who fulfilled the Bohan and Peter criteria of Idiopathic Inflammatory Myositis (IIM) having more than one year follow up were included. Results: Among the 31 subjects (22 females and 9 males), Dermatomyositis (DM), Polymyositis (PM) and Juvenile
Dermatomyositis (JDM) were 22, 6 and 3 respectively. The median age at diagnosis was 37 years (range 5-52) with median disease duration of 3 months (range 1.5-24) at presentation. The median disease duration at damage assessment was 3 years (range 1 e 24). Damage was present in 29/31 patients. The median MDI-Extent score was 3 (range 0-9). Damage occurred most frequently in muscular and endocrine system (15/31; 48% each). Menstrual irregularities were present in (11/15; 73%) of menstruating females. Hypertension and cataract were present in (9/31; 29%) and (7/31; 22%) of the subjects respectively. Age at diagnosis and gender did not correlate with the extent of damage. Conclusion: In our study more than ninety percent of patients with IIM accrue damage by median disease duration of 3 years.
P162. Distal RTA, chronic interstitial nephritis, hypokalemic periodic paresis, pancreatitis and endocrinopathy in pSS. Vikas Garg, Manish Kumar, B.K. Kundu, A.K. Gadpayle; Department of Medicine, PGIMER Dr. RML Hospital, New Delhi, India Abstract: A 32 year old married female presented with loose stools, vomiting & pain abdomen since 4 days, progressive weakness of all four limbs since 2 days with no history of fever, bowel/bladder dysfunction, sensory symptoms, back pain, trauma, and urine discoloration. She is known hypothyroid since 8 years, suffered 4 similar episode in last 3 years for which she was advised Kþ supplements & had B/L renal calculi since 2 years. On examination she had hypotonia, power was 0/5 all four limbs, all reflex were diminished. On investigations Hb 8.5gm%, TLC 9,000, urea 42 mg/dl, creatinine 1.4 mg/dl, Na 144, K 2.7, Cl 130, Ca 7.6, PO4 1.3, amylase 523, lipase 798. ABG showed metabolic acidosis with normal anion gap. Urine pH 7.5 with normal anion gap, albumin þþ, 30-40 RBC. USG showed bulky pancreas with increased echogenicity, GB sludge & multiple calculi in B/L kidneys with pelvicalyceal dilatation. Patient history was reviewed and she gave history of dryness of eyes, mouth & gritty sensations in eyes since 1 year. On investigation RF, ANA, SSA/SSB, CRP & anti-TPO were positive. IgA/IgG/IgM were raised. Serum PTH, Estradiol & C3/C4 were low. DEXA scan showed osteoporosis, IVP was suggestive of medullary sponge kidney. Schirmer's test was normal. Renal biopsy showed features of chronic tubulointerstitial nephritis & Lip biopsy showed lymphoepithelial sialadenitis consistent with Sjogren's syndrome. Conclusions: Renal involvement in Sjogren's uncommonly present as hypokalemic paralysis in absence of significant sicca symptoms. Multiple endocrinopathy including hypothyroidism & hypoparathyroidism can also occur.
P163. Clinical profile of primary sjogren's syndrome with hypokalemic periodic paralysis Nachiket Kulkarni, Naisar Nahar, Anuradha Venugopalan, Arvind Chopra; Centre for Rheumatic Diseases (CRD), Pune, India Introduction: Primary Sjogren's syndrome(pSS) with Hypokalemic Periodic Paralysis(HPP) is increasingly reported. Whether it's a protean manifestation or a different clinical subset is unraveled. Present study evaluates this clinical phenotype. Methods: Subjects of Primary Sjogren's Syndrome with Hypokalemic Periodic Paralysis (HPP) were identified retrospectively from database maintained at Centre for Rheumatic Diseases, Pune since 1996 with records of over 35000 patients. Clinical and investigations data was extracted pertaining to initial examination