- Email: [email protected]
Study of etiology and outcome of chronic lower extremity ulcer
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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
and immune studies were negative. Chest x-ray showed right hilar enlargement. Serum ACE levels and serum calcium were normal. The biopsy from the purpura yielded a histological diagnosis of leukocytoclastic vasculitis and direct immunofluorescence (DIF) was negative. Computed tomography (CT) disclosed significant mediastinal lymphadenopathy and matted lymph nodes in the lesser sac. Montoux test was highly positive. In view of high ESR, lymphadenopathy and positive montoux test, antitubercular treatment (ATT) was started. After one week of starting ATT, the skin lesions and fever completely resolved. CT chest and abdomen repeated after 2 months showed significant regression of lymphadenopathy. ATT continued for 6 months. Complete resolution of the lymphadenopathy was noted on Magnetic Resonance Imaging of chest and abdomen done 1 year later. Conclusion: Although incidence is rare, tuberculosis should be considered the possible underlying cause of vasculitis.
Introduction: Takayasu's arteritis is a large vessels vasculitis commonly involves the aorta with its branches and the pulmonary arteries. It occurs predominantly in females <40years age, with female to male ratio of 8:1. We hereby present a 50-years male, smoker, with bilateral lower limb gangrene and absent left brachial and radial pulse who on CT angiography had diffuse stenotic involvement of branches of arch of aorta and infrarenal abdominal aorta suggestive of Takayasu's Arteritis. Conclusion: This report intends to highlight that Takayasu's arteritis may rarely have a predominant lower limb involvement, and may be confused with Buerger's disease, especially in males. Clinical distinction of the diagnoses is important, for prediction of long term outcomes and prognosis.
P4. Elderly lady with renal limited vasculitis presenting as pyrexia of unknown origin and progressive renal dysfunction
M.M. Kavithaa, S. Srirama, M. Saravanana, J. Euphrasia Lathab, S. Rajeswaria; aDepartments of Rheumatology; bImmunology, Madras Medical College, Chennai, India
Betsy Ann Josepha, Vimal Abrahama, Anand Zachariaha, Anna T. Valsonb, V. Kavitac; aDepartments of Medicine; b Nephrology; cPathology, Christian Medical College, Vellore, India Introduction: Pauci-immune ANCA vasculitis is when there is absence of immunoglobulins as assessed by immunofluorescence on renal biopsy. It can be renal- limited or can be renal manifestation of systemic vasculitis such as Wegener's granulomatosis, Churg-Strauss vasculitis or microscopic polyangiitis. Methods: A descriptive case report of an elderly lady with pauciimmune renal-limited ANCA positive vasculitis, presenting as pyrexia of unknown origin to a tertiary care hospital in South India Results: A 72 year old lady presented with 6 months history of high grade fever and weight loss of 15 kilograms. Elsewhere she was treated with antibiotics, including anti-tuberculous therapy and oral steroids. Her blood pressure readings were noted to be high since the last 3 months. Clinically she was noted to have pallor and high blood pressure. Investigations revealed anemia, elevated creatinine, and urine analysis showed proteinuria with sediments. Urine protein/urine creatinine ratio was 1.18. Complements were normal, ANA (antinuclear antibody) was negative and p-ANCA (Anti-neutrophil cytoplasmic antibodies) was 227 (normal:<2).Renal biopsy showed focal and segmental proliferative, necrotizing glomerulonephritis with concomitant extensive necrotizing granulomatous interstitial and periglomerular infiltrates. She was initiated on prednisolone at 1 mg/kg and oral cyclophosphamide. She became afebrile and renal functions remained stable. Conclusions: In the setting of progressive renal dysfunction and new onset hypertension it is important to consider the diagnosis of vasculitis in a patient with pyrexia of unknown origin. Elderly age and deteriorating renal function is usually seen with ANCA associated vasculitis and if untreated may result in end stage renal failure and death.
P5. Takayasu's arteritis mimicking Burger's disease: diagnostic and management challenges Danveer Bhadu, Meha Sharma, Nilesh Nolkha, Aarti Sharma, Anupam Wakhlu; Department of Rheumatology, King George's Medical College, Lucknow, India
P6. Clinical correlation of cutaneous vasculitis with renal involvement in systemic lupus erythematosus
Aim: To study the clinical correlation of cutaneous vasculitis, a lupus nonspecific skin lesion with renal involvement in patients with lupus. Methods: 47 patients with cutaneous manifestations in lupus who presented to us in the past one year were included in the study. 40 patients with severe acute lupus specific skin lesions and seven patients with cutaneous vasculitis (a single variable of tender, erythematous lesions over the palms and soles) were taken as study groups. Renal biopsy was done in all patients irrespective of urine protein creatinine ratio. Results: Renal involvement was observed in 21 (out of 40) patients with lupus specific skin lesions and in all 7 patients with cutaneous vasculitis (class III -2, class IV -3, and class V-2), p value derived was statistically significant (p value 0.01). All patients with cutaneous vasculitis had low complements, mean C3 ¼ 42.31þ25.2mg/L (p ¼0.02), mean C4 ¼ 7.29þ5.88 mg/L (P ¼0.01) which was statistically significant. Conclusion: We observed that cutaneous vasculitis over palms and soles had 100% renal involvement. Should vasculitis be grouped under lupus specific skin lesion? Based on our observation, we propose that all patients with cutaneous vasculitis should undergo renal biopsy irrespective of urine protein creatinine ratio, though large scale studies are needed.
P7. Study of etiology and outcome of chronic lower extremity ulcer Amol Rauta, Rushabh Kotharia, Lalana Kalekara, Rachana Dhuratb, Yojana Gokhalea; aRheumatology Services, Department of Medicine; b Department of Dermatology, Lokmanya Tilak Municipal Medical College, Mumbai, India Introduction: Ten percent leg ulcers have etiology other than diabetes, trauma or venous insufficiency which is often not investigated leading to prolonged illness and its socioeconomic consequences. This study was conducted to find its etiology and outcome. Methods: Serial recruitment of patients with non-diabetic, nontraumatic chronic ulcer(>1 month) who were thoroughly investigated(CBC, Urine routine, Serum lipids,ESR,CRP,RA,AntiCCP, ANA,ANCA,C3,C4,Cryoglobulins,LA,ACLA,Anti-beta2glycoprotein, HIV, HBsAg, HCV, Doppler, Nerve conduction study and Biopsy of
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
edge of ulcer),treated and followed up for at least 6 months were included. Results: The causes in these 40 patients were: venous in 10, vasculitis in 18, arterial in 4 pyoderma gangrenosum in RA in 2,malignancy in 1,tuberculosis in 2 and idiopathic in 3.Of 18 vasculitis patients studied, etiology found were PAN(2),cutaneous PAN(1),WG(1),MPA(1),leukocytoclastic(5)(primary),SLE(2),RA(2),liv edoidvasculopathy(1),SSc(1),secondary APLA syndrome(SLE)(1), Dermatomyositis(1). Most patients (28) had vasculitic or venous ulcers which can be differentiated by clinical and laboratory parameters viz.site (gaiter area in venous), surrounding skin (hyperpigmented in venous), raised acute phase reactants in vasculitic, urine being active in vasculitic and biopsy. Of the 33 skin biopsies done 21(63.63%) were diagnostic giving high yield. Etiology diagnosed on biopsy being vasculitis (18), tuberculosis (2), malignancy (1) and others (12) being nonspecific. Of 18 vasculitic patients, disease got diagnosed due to extensive investigation in 13 viz.5 leucocytoclastic vasculitis,2 PAN,1 cutaneousPAN,1 WG,1 MPA,1 RA,1 secondary APLA(SLE)and 2 SLE. Conclusions: Aggressive investigations including biopsy are recommended in all chronic ulcers. Prognostically it is important to differentiate between vasculitic and venous cause.
P8. Genetics of ANCA associated vasculitides in north Indian population R.W. Minza, J. Singha, N. Kaura, S. Ananda, L. Rania, D. Guptab, N.K. Pandac, A. Sharmad; aDepartments of Immunopathology; b Pulmonary Medicine; cENT and dInternal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India Introduction: ANCA (anti neutrophilic cytoplasmic antibodies) associated vasculitides (AAV) comprises of a group of autoimmune diseases that are characterized by necrotizing small vessel vasculitis and frequent presence of ANCA. AAVs include granulomatosis with polyangiitis, microscopic polyangiitis that are characterized by ANCA mounted to specific antigens like proteinase 3 (PR3) and myeloperoxidase (MPO). This study was planned to explore the genetic basis of AAV in a north Indian population. Methods: 100 patients and 100 age and sex matched controls were enrolled in a study from north Indian population. ANCA were detected in the sera of the patients by indirect immunofluorescence and specific pattern i.e. cANCA and pANCA were ascribed. ELISAs were done for PR3 and MPO detection. HLA typing for DRB1-DQB1 loci was done by PCR-SSP and SNP detection was done for 5 SNPs in CTLA-4 gene and 3 SNPs in PTPN22 gene by real time PCR, using Simple probes. Results: 54% patients were cANCA positive and 36% patients were pANCA positive and 10% patients were negative for ANCA by immunofluorescence. Out of these patients, 62% were positive for anti PR3 antibodies and 35% were positive for anti MPO antibodies. DQB1*05 and CTLA-4 -1661 AA were significantly associated with the AAV disease group. DRB1*03 was significantly associated with the patients with PR3 positivity. Conclusion: The results of present study indicate that DQB1*05 allele and CTLA4 1661 SNP are associated with AAV. DRB1*03 is associated with GPA.
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P9. Takayasu's arteritis e Case report Joseph V.F. Vijin, Ahmed B. Mukhtar; Internal Medicine, Yenepoya Medical College, Yenepoya University, Deralakatte, Mangalore, India Introduction: Takayasu's arteritis also known as pulseless disease or occlusive thromboaortopathy is a form of vasculitis of unknown cause that chiefly affects the aorta and its major branches, most frequently in young women. The disease is named for the Japanese ophthalmologist who in 1908 described a young woman with peculiar retinal arteriovenous anastomoses caused by retinal ischemia from large vessel vasculitis. Methods: This is an interesting case report of 20 year old female presented with fever on and off, multiple joint pains since 2 years and generalized weakness since 1 year, with history of claudication of upper limbs. All the symptoms were progressive in nature. On examination she had a BMI of 18.35kg/m2, swelling over the left side of neck which was pulsatile and non-tender, BP right arm e 120/64, BP left arm e 100/68. Feeble pulse over the left radial and brachial artery, bilateral aortic bruit. Investigations shows anemia (9gm/dl), high ESR (122mm/1hr), CRP of 32.8mg/l, iron profile study shows anemia of chronic disease (ferritin e 660, iron e 29, TIBC e 334), USG neck showed aneurysmal dilation from the root of aorta involving bilateral common carotid artery with wall thickening. CT angiogram was suggestive of aorto arteritis (Takayasu's arteritis type 1), hence came to a diagnosis and started on oral steroids 40 mg/day (1mg/kg/day). Conclusion: vasculitis disorders present in different ways, but it is important not to miss the diagnosis.
P10. Relapsing polychondritis: Clinical presentations, disease activity and outcomes Aman Sharmaa, Arjun D. Lawa, Pradeep Bamberyb, Vinay Sagara, Varun Dhira, Rajesh Vijayvergiyad, Ajay Wanchuc, e f f Kusum Sharma , Ashok Gupta , Naresh K. Panda , Surjit Singha; a Department of Internal Medicine PGIMER, Chandigarh; bBundaberg Base Hospital Bundaberg, Australia; cDivision of Arthritis and Rheumatic Diseases, Oregon Health & Science University, Portland, Oregon, USA; dDepartment of Cardiology; eDepartment of Medical Microbiology; fDepartment of Otorhinolaryngology, Postgraduate Institute of Medical Education and Research, Chandigarh, India Background: Relapsing polychondritis is a rare disease characterized by inflammation of cartilaginous and proteoglycan rich structures. There are very few published single centre case series from all across the world. Clinical characteristics and outcomes in 26 patients seen at a tertiary care centre of North India are being described. Methods: This was a retrospective study involving all patients diagnosed to have relapsing polychondritis according to Damiani and Levine's modification of McAdam's criteria .Details of clinical presentations, investigation reports, disease activity assessment as measured by relapsing polychondritis disease activity index(RPDAI), treatment instituted and outcomes were recorded. Results: e 14-65 years) met the diagnostic criteria. The clinical manifestations were auricular chondritis (96.1%), arthritis (53.8%), hearing impairment (42.3%),eye involvement(42%), skin involvement(26%), Cardiovascular(11.5%) and laryngotracheal involvement(11.5%).The median RPDAI was 31(range 9-66). Two patients died on follow up. Overall survival was 92.3% and median survival was 13.5 years.
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
and immune studies were negative. Chest x-ray showed right hilar enlargement. Serum ACE levels and serum calcium were normal. The biopsy from the purpura yielded a histological diagnosis of leukocytoclastic vasculitis and direct immunofluorescence (DIF) was negative. Computed tomography (CT) disclosed significant mediastinal lymphadenopathy and matted lymph nodes in the lesser sac. Montoux test was highly positive. In view of high ESR, lymphadenopathy and positive montoux test, antitubercular treatment (ATT) was started. After one week of starting ATT, the skin lesions and fever completely resolved. CT chest and abdomen repeated after 2 months showed significant regression of lymphadenopathy. ATT continued for 6 months. Complete resolution of the lymphadenopathy was noted on Magnetic Resonance Imaging of chest and abdomen done 1 year later. Conclusion: Although incidence is rare, tuberculosis should be considered the possible underlying cause of vasculitis.
Introduction: Takayasu's arteritis is a large vessels vasculitis commonly involves the aorta with its branches and the pulmonary arteries. It occurs predominantly in females <40years age, with female to male ratio of 8:1. We hereby present a 50-years male, smoker, with bilateral lower limb gangrene and absent left brachial and radial pulse who on CT angiography had diffuse stenotic involvement of branches of arch of aorta and infrarenal abdominal aorta suggestive of Takayasu's Arteritis. Conclusion: This report intends to highlight that Takayasu's arteritis may rarely have a predominant lower limb involvement, and may be confused with Buerger's disease, especially in males. Clinical distinction of the diagnoses is important, for prediction of long term outcomes and prognosis.
P4. Elderly lady with renal limited vasculitis presenting as pyrexia of unknown origin and progressive renal dysfunction
M.M. Kavithaa, S. Srirama, M. Saravanana, J. Euphrasia Lathab, S. Rajeswaria; aDepartments of Rheumatology; bImmunology, Madras Medical College, Chennai, India
Betsy Ann Josepha, Vimal Abrahama, Anand Zachariaha, Anna T. Valsonb, V. Kavitac; aDepartments of Medicine; b Nephrology; cPathology, Christian Medical College, Vellore, India Introduction: Pauci-immune ANCA vasculitis is when there is absence of immunoglobulins as assessed by immunofluorescence on renal biopsy. It can be renal- limited or can be renal manifestation of systemic vasculitis such as Wegener's granulomatosis, Churg-Strauss vasculitis or microscopic polyangiitis. Methods: A descriptive case report of an elderly lady with pauciimmune renal-limited ANCA positive vasculitis, presenting as pyrexia of unknown origin to a tertiary care hospital in South India Results: A 72 year old lady presented with 6 months history of high grade fever and weight loss of 15 kilograms. Elsewhere she was treated with antibiotics, including anti-tuberculous therapy and oral steroids. Her blood pressure readings were noted to be high since the last 3 months. Clinically she was noted to have pallor and high blood pressure. Investigations revealed anemia, elevated creatinine, and urine analysis showed proteinuria with sediments. Urine protein/urine creatinine ratio was 1.18. Complements were normal, ANA (antinuclear antibody) was negative and p-ANCA (Anti-neutrophil cytoplasmic antibodies) was 227 (normal:<2).Renal biopsy showed focal and segmental proliferative, necrotizing glomerulonephritis with concomitant extensive necrotizing granulomatous interstitial and periglomerular infiltrates. She was initiated on prednisolone at 1 mg/kg and oral cyclophosphamide. She became afebrile and renal functions remained stable. Conclusions: In the setting of progressive renal dysfunction and new onset hypertension it is important to consider the diagnosis of vasculitis in a patient with pyrexia of unknown origin. Elderly age and deteriorating renal function is usually seen with ANCA associated vasculitis and if untreated may result in end stage renal failure and death.
P5. Takayasu's arteritis mimicking Burger's disease: diagnostic and management challenges Danveer Bhadu, Meha Sharma, Nilesh Nolkha, Aarti Sharma, Anupam Wakhlu; Department of Rheumatology, King George's Medical College, Lucknow, India
P6. Clinical correlation of cutaneous vasculitis with renal involvement in systemic lupus erythematosus
Aim: To study the clinical correlation of cutaneous vasculitis, a lupus nonspecific skin lesion with renal involvement in patients with lupus. Methods: 47 patients with cutaneous manifestations in lupus who presented to us in the past one year were included in the study. 40 patients with severe acute lupus specific skin lesions and seven patients with cutaneous vasculitis (a single variable of tender, erythematous lesions over the palms and soles) were taken as study groups. Renal biopsy was done in all patients irrespective of urine protein creatinine ratio. Results: Renal involvement was observed in 21 (out of 40) patients with lupus specific skin lesions and in all 7 patients with cutaneous vasculitis (class III -2, class IV -3, and class V-2), p value derived was statistically significant (p value 0.01). All patients with cutaneous vasculitis had low complements, mean C3 ¼ 42.31þ25.2mg/L (p ¼0.02), mean C4 ¼ 7.29þ5.88 mg/L (P ¼0.01) which was statistically significant. Conclusion: We observed that cutaneous vasculitis over palms and soles had 100% renal involvement. Should vasculitis be grouped under lupus specific skin lesion? Based on our observation, we propose that all patients with cutaneous vasculitis should undergo renal biopsy irrespective of urine protein creatinine ratio, though large scale studies are needed.
P7. Study of etiology and outcome of chronic lower extremity ulcer Amol Rauta, Rushabh Kotharia, Lalana Kalekara, Rachana Dhuratb, Yojana Gokhalea; aRheumatology Services, Department of Medicine; b Department of Dermatology, Lokmanya Tilak Municipal Medical College, Mumbai, India Introduction: Ten percent leg ulcers have etiology other than diabetes, trauma or venous insufficiency which is often not investigated leading to prolonged illness and its socioeconomic consequences. This study was conducted to find its etiology and outcome. Methods: Serial recruitment of patients with non-diabetic, nontraumatic chronic ulcer(>1 month) who were thoroughly investigated(CBC, Urine routine, Serum lipids,ESR,CRP,RA,AntiCCP, ANA,ANCA,C3,C4,Cryoglobulins,LA,ACLA,Anti-beta2glycoprotein, HIV, HBsAg, HCV, Doppler, Nerve conduction study and Biopsy of
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
edge of ulcer),treated and followed up for at least 6 months were included. Results: The causes in these 40 patients were: venous in 10, vasculitis in 18, arterial in 4 pyoderma gangrenosum in RA in 2,malignancy in 1,tuberculosis in 2 and idiopathic in 3.Of 18 vasculitis patients studied, etiology found were PAN(2),cutaneous PAN(1),WG(1),MPA(1),leukocytoclastic(5)(primary),SLE(2),RA(2),liv edoidvasculopathy(1),SSc(1),secondary APLA syndrome(SLE)(1), Dermatomyositis(1). Most patients (28) had vasculitic or venous ulcers which can be differentiated by clinical and laboratory parameters viz.site (gaiter area in venous), surrounding skin (hyperpigmented in venous), raised acute phase reactants in vasculitic, urine being active in vasculitic and biopsy. Of the 33 skin biopsies done 21(63.63%) were diagnostic giving high yield. Etiology diagnosed on biopsy being vasculitis (18), tuberculosis (2), malignancy (1) and others (12) being nonspecific. Of 18 vasculitic patients, disease got diagnosed due to extensive investigation in 13 viz.5 leucocytoclastic vasculitis,2 PAN,1 cutaneousPAN,1 WG,1 MPA,1 RA,1 secondary APLA(SLE)and 2 SLE. Conclusions: Aggressive investigations including biopsy are recommended in all chronic ulcers. Prognostically it is important to differentiate between vasculitic and venous cause.
P8. Genetics of ANCA associated vasculitides in north Indian population R.W. Minza, J. Singha, N. Kaura, S. Ananda, L. Rania, D. Guptab, N.K. Pandac, A. Sharmad; aDepartments of Immunopathology; b Pulmonary Medicine; cENT and dInternal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India Introduction: ANCA (anti neutrophilic cytoplasmic antibodies) associated vasculitides (AAV) comprises of a group of autoimmune diseases that are characterized by necrotizing small vessel vasculitis and frequent presence of ANCA. AAVs include granulomatosis with polyangiitis, microscopic polyangiitis that are characterized by ANCA mounted to specific antigens like proteinase 3 (PR3) and myeloperoxidase (MPO). This study was planned to explore the genetic basis of AAV in a north Indian population. Methods: 100 patients and 100 age and sex matched controls were enrolled in a study from north Indian population. ANCA were detected in the sera of the patients by indirect immunofluorescence and specific pattern i.e. cANCA and pANCA were ascribed. ELISAs were done for PR3 and MPO detection. HLA typing for DRB1-DQB1 loci was done by PCR-SSP and SNP detection was done for 5 SNPs in CTLA-4 gene and 3 SNPs in PTPN22 gene by real time PCR, using Simple probes. Results: 54% patients were cANCA positive and 36% patients were pANCA positive and 10% patients were negative for ANCA by immunofluorescence. Out of these patients, 62% were positive for anti PR3 antibodies and 35% were positive for anti MPO antibodies. DQB1*05 and CTLA-4 -1661 AA were significantly associated with the AAV disease group. DRB1*03 was significantly associated with the patients with PR3 positivity. Conclusion: The results of present study indicate that DQB1*05 allele and CTLA4 1661 SNP are associated with AAV. DRB1*03 is associated with GPA.
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P9. Takayasu's arteritis e Case report Joseph V.F. Vijin, Ahmed B. Mukhtar; Internal Medicine, Yenepoya Medical College, Yenepoya University, Deralakatte, Mangalore, India Introduction: Takayasu's arteritis also known as pulseless disease or occlusive thromboaortopathy is a form of vasculitis of unknown cause that chiefly affects the aorta and its major branches, most frequently in young women. The disease is named for the Japanese ophthalmologist who in 1908 described a young woman with peculiar retinal arteriovenous anastomoses caused by retinal ischemia from large vessel vasculitis. Methods: This is an interesting case report of 20 year old female presented with fever on and off, multiple joint pains since 2 years and generalized weakness since 1 year, with history of claudication of upper limbs. All the symptoms were progressive in nature. On examination she had a BMI of 18.35kg/m2, swelling over the left side of neck which was pulsatile and non-tender, BP right arm e 120/64, BP left arm e 100/68. Feeble pulse over the left radial and brachial artery, bilateral aortic bruit. Investigations shows anemia (9gm/dl), high ESR (122mm/1hr), CRP of 32.8mg/l, iron profile study shows anemia of chronic disease (ferritin e 660, iron e 29, TIBC e 334), USG neck showed aneurysmal dilation from the root of aorta involving bilateral common carotid artery with wall thickening. CT angiogram was suggestive of aorto arteritis (Takayasu's arteritis type 1), hence came to a diagnosis and started on oral steroids 40 mg/day (1mg/kg/day). Conclusion: vasculitis disorders present in different ways, but it is important not to miss the diagnosis.
P10. Relapsing polychondritis: Clinical presentations, disease activity and outcomes Aman Sharmaa, Arjun D. Lawa, Pradeep Bamberyb, Vinay Sagara, Varun Dhira, Rajesh Vijayvergiyad, Ajay Wanchuc, e f f Kusum Sharma , Ashok Gupta , Naresh K. Panda , Surjit Singha; a Department of Internal Medicine PGIMER, Chandigarh; bBundaberg Base Hospital Bundaberg, Australia; cDivision of Arthritis and Rheumatic Diseases, Oregon Health & Science University, Portland, Oregon, USA; dDepartment of Cardiology; eDepartment of Medical Microbiology; fDepartment of Otorhinolaryngology, Postgraduate Institute of Medical Education and Research, Chandigarh, India Background: Relapsing polychondritis is a rare disease characterized by inflammation of cartilaginous and proteoglycan rich structures. There are very few published single centre case series from all across the world. Clinical characteristics and outcomes in 26 patients seen at a tertiary care centre of North India are being described. Methods: This was a retrospective study involving all patients diagnosed to have relapsing polychondritis according to Damiani and Levine's modification of McAdam's criteria .Details of clinical presentations, investigation reports, disease activity assessment as measured by relapsing polychondritis disease activity index(RPDAI), treatment instituted and outcomes were recorded. Results: e 14-65 years) met the diagnostic criteria. The clinical manifestations were auricular chondritis (96.1%), arthritis (53.8%), hearing impairment (42.3%),eye involvement(42%), skin involvement(26%), Cardiovascular(11.5%) and laryngotracheal involvement(11.5%).The median RPDAI was 31(range 9-66). Two patients died on follow up. Overall survival was 92.3% and median survival was 13.5 years.