Concluded Case

Young female with gangrene

35f Ho autoimmune haemolytic anaemia 3 y back Direct coombs test,ANA was positive Anti ds dna was negative Presented with rt index finger distal phalanx gangrene with sharp demarcation with severe pain burning at site for 1 months No ho of any raynauds phenomenon Some time mild joint pain Now anti phospholipid screening is normal including anti cardiolipin anti b2 glycoprotein lupus anticoagulant are negative ANCA is negative But centromere protein B is positive in ANA blot rest is negative Now she is on aspirin warfarin Prednisone But in view of persistent pain tadalafil and bosentan was added I came to diagnosis of subclinical limited scleroderma What else can be done here for persistent pain of gangrene distal phalanx and to prevent future complications???? Should we wait for autoamputation?? Thank you in advance!!!!!

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This is undifferentiated connective tissue disease...retake history for raynauds phenomenon... sometimes it can be incomplete meaning only whitish discoloration and not bluish discolouration....check for PAH and ILD....check whether upper limb pulses palpable or not? How did gangrene start...was it sudden onset or slowly progressive?...first we have to ascertain the cause of gangrene to be VASCULITIS or VASCULOPATHY....many times it is very difficult to differentiate between two... sometimes we have to give combined treatment Plz share routine blood reports... especially cbc,LDH,ESR, crp( quantitative), hiv, hbsag, antihcv, urine rm, 2D- echo, cxr.... kindly get arterial doppler of upper limbs Regarding treatment wise, persistent pain indicates ongoing disease activity and I am hoping it's not wet gangrene....has the gangrene become static or is it increasing along with pain?....has pain improved after adding tadalafil ? What is the dose of tadalafil and Prednisone now? I will add hydroxychloroquine, pregabalin...decision to give steroids or not or to add alprostadil depends on ur response to the questions and sharing of reports

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This is undifferentiated connective tissue disease...retake history for raynauds phenomenon... sometimes it can be incomplete meaning only whitish discoloration and not bluish discolouration....check for PAH and ILD....check whether upper limb pulses palpable or not? How did gangrene start...was it sudden onset or slowly progressive?...first we have to ascertain the cause of gangrene to be VASCULITIS or VASCULOPATHY....many times it is very difficult to differentiate between two... sometimes we have to give combined treatment Plz share routine blood reports... especially cbc,LDH,ESR, crp( quantitative), hiv, hbsag, antihcv, urine rm, 2D- echo, cxr.... kindly get arterial doppler of upper limbs Regarding treatment wise, persistent pain indicates ongoing disease activity and I am hoping it's not wet gangrene....has the gangrene become static or is it increasing along with pain?....has pain improved after adding tadalafil ? What is the dose of tadalafil and Prednisone now? I will add hydroxychloroquine, pregabalin...decision to give steroids or not or to add alprostadil depends on ur response to the questions and sharing of reports

Gangrene is dry with demarcation, All pulses are normal It started after pain no ho of raynauds phenomenon CBC normal esr 20 2decho CXR normal Doppler of affected limb normal After tadalafil 10 mg od and bosentan pain have subsided Prednisolone 10 mg od warf 5mg od
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This is systemic sclerosis sine scleroderma, skin usally normal ,anticentromer antibody is usually positive .there is definite raynauds phenomenon,do cold challange test to confirm. Kindly rule out PAH ,ILD by becho and hrct thorax. See increase the dose of tadalafil or bosentan .wait for autoampuation For pain add Gabapentin or pregabalin Continue warfarin for sometime ,donot combine with aspirin as increases risk of bleeding .gradually introduce aspirin and stop warfarin as per your clinical decision. Do PSPT to rule out APS if any chance there. You have done good management Gangrene can be vasculitis related too ,so just do DS DNA C3,c4;ANCA If ild is there , introduce immunosuppression

I think already patient on some immunosuppression with steroid or other steroid sparing agent for AIHA
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Gangrene of B right index finger distal phalanx in a known case of SLE, with autoimmune Hemolytic anemia Digital gangrene is a known complication in autoimmune diseases especially scleroderma Gangrenous portion is nonviable and require removal at some point of time If there is no infection, if it is dry gangrene, one can do wait on watch for some time The purpose of wait and watch approach is 1) To look for etiology 2) To treat underlying pathology 3) To improve vascularity If all three objectives are achieved, one should go for surgery Surgical removal will help in reducing pain, and patient can adapt to new lifestyle and start her life once again. With gangrene present she will need to visit hospital again and again and she will not be able to lead a fruitful social life If objectives are not achieved as specified before, then wait till objectives are achieved.

Thank you doctor
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Any skin tightening, GERD present or not? Blood pressure?

All normal No skin problem no git prob,bp is normal What else can be done here sir??
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