286. Clinical characteristics and outcomes of paediatric patients presenting with digital gangrene to a Rheumatology clinic.

Background: Digital gangrene involving hands and feet is an uncommon presentation in paediatric patients and in many cases an etiological factor is not identified. Paediatric vasculitides are multisystemic diseases which can have digital gangrene as one of the myriad manifestations that need to be diagnosed and treated early to improve outcomes. This study was carried out to evaluate the clinical features, treatment modalities and outcomes of paediatric patients presenting with digital gangrene/pre-gangrene to a Rheumatology clinic over 2 year follow up.

Methods: This prospective observational cohort study was conducted in the Clinical Immunology and Rheumatology department of a tertiary care teaching and referral hospital in Northern India over a period of 24 months (January 2019-December 2020). Clinical details of paediatric patients (<18 years) with digital gangrene were recorded at 1^st visit, 3 months ,6 months and 2 years follow up. Patients with frank sepsis or septic shock/DIC were excluded.

Results: 10 pediatric patients with mean age 14.6 +/- 1.48 years were enrolled. The mean duration of illness was 13.95 +/- 20.4 months. 5 patients had both gangrene and pre-gangrene while 5/10 had only gangrene. Presentation was B/L in 6/10 patients with both hands and feet involved in 3/10. Median no of digits with gangrenous changes were 5(IQR 1-10) while median no of digits with pre-gangrene were 5(IQR 0-12). 5 children showed concomitant bacterial infection of digits requiring antibiotic. Raynauld’s preceded gangrene in 3/10. The most frequent organ involvement at presentation apart from gangrene was cutaneous (6/10), musculoskeletal (4/10), PNS (3/10) and GI (3/10). 6 patients had leukocytosis (13220 +/- 6972.9 /mm³) while thrombocytosis (222+/-174.6 x 10⁹/L) was present in 3/10. All 10 patients had elevated ESR (69.1+/-33.9 mm) with elevated CRP (20.923.2 mg/dl) in 8/10 patients. Mean ESR (17.6+/-13.7) and CRP (3.84+/-4.22) decreased by >50 % at 3 months in 8/10 patients. At 6 months follow up all patients had normal inflammatory markers except raised ESR in 2 and raised CRP in 1. Persistent APLA positivity (> 40 U) at 12 weeks was documented in 3 patients with LAC being most common. Hypocomplementemia was present in 5 patients with 4 showing low serum C3(normal 90-180) and low C4(10-40) in 5/10. 2/10 patients had low IgA levels while one patient with elevated IgG levels was diagnosed with Juvenile SSc with Autoimmune hepatitis at follow up. Arterial doppler showed vasculitis in 5, ischemic changes in 2, both vasculitis and thrombosis in one patient and normal for 3 patients. Gangrene was attributed to Childhood PAN in 3/10, SLE-APS in 2, SLE/Lupus vasculitis in 1, SLE lupus vasculitis and APS in 1, Juvenile SSc in 2 while 1 patient had Scrub typhus associated vasculitis. 8 patients received oral glucocorticoids as treatment including pulse methylprednisolone for 2 patients and concomitant anticoagulation in 5/10. I.V cyclophosphamide was the preferred steroid sparing drug (7/10). 1 patient of Juvenile SSc received methotrexate while 2 patients didn’t receive 2^nd line therapy. Child with SLE/APS/small vessel vasculitis required IVIG for myocarditis. Azathioprine was used as maintenance in 8/10 children. The mean dose of prednisolone at 2 years follow up was 4.3756.022 mg/d. The median pain score on VAS scale of 0-10 was 8(IQR 5-10). Median no of autoamputated digits at 6 months were 2(IQR 0-6). Surgical amputation was done in 4/10 patients. At 2 years clinical remission was seen in 4/10,4/10 had improvement in symptoms without clinical remission,1 patient had active disease while 1 patient with SLE expired due to cardiogenic shock.

Conclusions: In our cohort of pediatric patients presenting with digital gangrene SLE with APS was most common etiology. Childhood PAN, SLE small vessel vasculitis, juvenile SSc and infection associated vasculitis remain the other important causes. In tropical countries infections are important vasculitis mimics and must be ruled out prior to attributing gangrene to autoimmune disease. Long term clinical outcomes of such patients are good provided early diagnosis and initiation of treatment.

Disclosures: None

Background: Digital gangrene involving hands and feet is an uncommon presentation in paediatric patients and in many cases an etiological factor is not identified. Paediatric vasculitides are multisystemic diseases which can have digital gangrene as one of the myriad manifestations that need to be diagnosed and treated early to improve outcomes. This study was carried out to evaluate the clinical features, treatment modalities and outcomes of paediatric patients presenting with digital gangrene/pre-gangrene to a Rheumatology clinic over 2 year follow up.

Methods: This prospective observational cohort study was conducted in the Clinical Immunology and Rheumatology department of a tertiary care teaching and referral hospital in Northern India over a period of 24 months (January 2019-December 2020). Clinical details of paediatric patients (<18 years) with digital gangrene were recorded at 1^st visit, 3 months ,6 months and 2 years follow up. Patients with frank sepsis or septic shock/DIC were excluded.

Results: 10 pediatric patients with mean age 14.6 +/- 1.48 years were enrolled. The mean duration of illness was 13.95 +/- 20.4 months. 5 patients had both gangrene and pre-gangrene while 5/10 had only gangrene. Presentation was B/L in 6/10 patients with both hands and feet involved in 3/10. Median no of digits with gangrenous changes were 5(IQR 1-10) while median no of digits with pre-gangrene were 5(IQR 0-12). 5 children showed concomitant bacterial infection of digits requiring antibiotic. Raynauld’s preceded gangrene in 3/10. The most frequent organ involvement at presentation apart from gangrene was cutaneous (6/10), musculoskeletal (4/10), PNS (3/10) and GI (3/10). 6 patients had leukocytosis (13220 +/- 6972.9 /mm³) while thrombocytosis (222+/-174.6 x 10⁹/L) was present in 3/10. All 10 patients had elevated ESR (69.1+/-33.9 mm) with elevated CRP (20.923.2 mg/dl) in 8/10 patients. Mean ESR (17.6+/-13.7) and CRP (3.84+/-4.22) decreased by >50 % at 3 months in 8/10 patients. At 6 months follow up all patients had normal inflammatory markers except raised ESR in 2 and raised CRP in 1. Persistent APLA positivity (> 40 U) at 12 weeks was documented in 3 patients with LAC being most common. Hypocomplementemia was present in 5 patients with 4 showing low serum C3(normal 90-180) and low C4(10-40) in 5/10. 2/10 patients had low IgA levels while one patient with elevated IgG levels was diagnosed with Juvenile SSc with Autoimmune hepatitis at follow up. Arterial doppler showed vasculitis in 5, ischemic changes in 2, both vasculitis and thrombosis in one patient and normal for 3 patients. Gangrene was attributed to Childhood PAN in 3/10, SLE-APS in 2, SLE/Lupus vasculitis in 1, SLE lupus vasculitis and APS in 1, Juvenile SSc in 2 while 1 patient had Scrub typhus associated vasculitis. 8 patients received oral glucocorticoids as treatment including pulse methylprednisolone for 2 patients and concomitant anticoagulation in 5/10. I.V cyclophosphamide was the preferred steroid sparing drug (7/10). 1 patient of Juvenile SSc received methotrexate while 2 patients didn’t receive 2^nd line therapy. Child with SLE/APS/small vessel vasculitis required IVIG for myocarditis. Azathioprine was used as maintenance in 8/10 children. The mean dose of prednisolone at 2 years follow up was 4.3756.022 mg/d. The median pain score on VAS scale of 0-10 was 8(IQR 5-10). Median no of autoamputated digits at 6 months were 2(IQR 0-6). Surgical amputation was done in 4/10 patients. At 2 years clinical remission was seen in 4/10,4/10 had improvement in symptoms without clinical remission,1 patient had active disease while 1 patient with SLE expired due to cardiogenic shock.

Conclusions: In our cohort of pediatric patients presenting with digital gangrene SLE with APS was most common etiology. Childhood PAN, SLE small vessel vasculitis, juvenile SSc and infection associated vasculitis remain the other important causes. In tropical countries infections are important vasculitis mimics and must be ruled out prior to attributing gangrene to autoimmune disease. Long term clinical outcomes of such patients are good provided early diagnosis and initiation of treatment.

Disclosures: None