Small Vessel Vasculitis

Vasculitis Highlights Report

Vasculitis Challenge

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Current Challenge

February 22, 2012

38 year old male presents to your Rheumatology clinic for evaluation. His current complaints include ulcerations of his tongue, palate, buccal mucosa, posterior Pharynx, Peri-rectal sores/ulcers, joint pain and skin lesions that were biopsy proven e. nodosum type lesions. He was diagnosed with Behcet’s and started on colchicine 0.6mg twice per day, this did not provide significant relief so 2 months later, he was started on Prednisone 30 mg per day and azathioprine 150/d. He improved, but recurrent mucosal and skin lesions developed each time steroids were lowered to less than 15 mg per day. He would like to know what other options he has so he can “get off steroids.”

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Past Vasculitis Challenges

February 8, 2012

36 year old female presents to her primary care MD for routine physical. On exam the nurse could not obtain a blood pressure in her left arm. Blood pressure in the right arm was 100/60. In further ROS, she states she is “having a hard time blow drying her hair with my left arm” and new onset low-grade fever, fatigue and intermittent jaw pain.

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January 25, 2012

A 50 year old male with GPA since 2008 is having his routine monthly labs done to monitor his methotrexate. He is off steroids and has not has a flare up since his initial diagnosis in 2008, for which he received cyclophosphamide for 6 months with high dose steroids that were tapered off over 6 months. His labs look good with the exception of a mildly elevated sedimentation rate, 45mm/h (normal 0-20) and new 2+ blood in his urine. You call him over the phone and he reports his home urine dipsticks have showed blood for the past 2 days. He also states he has some joint pain but states “I must just be getting old.”

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January 11, 2012

41 year old male with GPA since 2000 presents to your clinic for follow up. He has had multiple relapses almost all of which have been severe enough to require cyclophosphamide induction. His course was complicated by avascular necrosis of the hips following his initial induction but he has overall done well with very little disease or treatment related damage. In 2006 he elected to stop all of his treatment. He remained in remission off all medications for about 3 years.

In 1/09 he had a severe relapse involving his skin, joint, kidney with normal renal function. Treatment options were discussed and we elected to return to cyclophosphamide. He achieved remission and we attempted to transition to mycophenolate mofetil. He did not tolerated this and so azathrioprine was given and the patient had a severe allergic reaction to it. Methotrexate was attempted but he developed leukopenia on it and it was stopped. One month later, the patient again had a severe flare, this flare was treated with Rituximab, which was tolerated well, and 2 months later, remission was achieved and the patient was weaned to 5 mg per day of Prednisone.

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December 28th, 2011

40 year old male comes to your vaculitis clinic with complains of “terrible eye pain”, migratory arthalgias and increased nasal bleeding. He has known GPA since 1996 which has affected his joints, sinuses, nose (crusting, bleeding and saddle-nose deformity), kidneys (hematuria with normal creatinine) and eyes (conjunctivitis), he is C ANCA-PR-3 + at time of diagnosis. Although he was initially treated with high dose steroids and cyclophosphamide in 1996, he has been in remission on prednisone 5mg/d in combination with methotrexate 25 mg/wk SQ. When you examine him you note there is proptosis of the right orbit and diplopia with lateral gaze. An orbital CT scan is done, it shows Right orbital mass, new since 1997 that measures 2.2 x 1.9 cm.

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December 14th, 2011

38 year old female is evaluated by her primary care MD and found to have an asymptomatic aortic insufficiency murmur. She is referred to a cardiology who then finds thickening of the ascending and descending aorta "c/w with vasculitis".

Labs show a sedimentation rate of 82, C - reactive protein of 7.8 and platelet count of 584. Her history is only remarkable for Grave’s disease, for which she had RAI treatment. Further work up reveals addition stenosis of the bilateral renal arteries, right sub-clavian and left carotid. Her infection w/u is negative. You diagnosis her with Takayasu’s arteritis. She has heard “horror stories” about prednisone and does not want to take it. What are the treatment options you discuss?

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November 30th, 2011

A 50 year old male presents to the hospital with rapid progressive kidney failure. On admission his creatinine is 5.6, he has new onset hematuria with adbundant RBC casts and is C ANCA/PR-3 +. Upon further evaluation, he notes recurrent episodes of nasal bleeding, sinusitis and a cough. His imaging studies show bilateral pulmonary infiltrates and mucosal thickening of his maxillary sinuses. He is diagnosed with GPA and given steroids (1mg/kg) and Rituximab therapy (1 gm repeated 2 weeks later) + started on dialysis. After 3 months he is doing well and down to Prednisone 5mg/d but still has a creatinine of 5.0. Would renal transplant be an option for a GPA patient like this?

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November 16th, 2011

30 year old female has known GPA which has involved his nose, sinuses, ears, joint, kidneys (peak creatinine of 2.0) and his was C ANCA/PR-3 positive. He initially received induction therapy of cyclophosphamide for 4 months and steroids. He achieved remission and has been on azathioprine 150mg per day and is off steroids since 2009. Today he is having arthalgia’s, nasal bleeding and new bilateral pulmonary infiltrates on CT. His bronchscopy showed + hemosiderin laden macrophages and no infection, what is a reasonable course of action?

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November 1st, 2011

27 year old woman comes to the office with malaise and rash of 4 weeks duration, She was previously healthy and takes no medications.  The rash is described as erythematous and palpable and non-blanching  (i.e. palpable purpura).  It is located predominately  on the lower extremities. She has no other symptoms and her exam is otherwise normal.

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October 18th, 2011

A 38 year old woman comes in for 12 months of not feeling well. She has diffuse aches and pains mostly in the muscles and fatigue. She has no fever, rash, weight loss. She is referred to you the rheumatologist because she has the following laboratory test.

  • + P ANCA - titer 1/320
  • EIA for Myeloperoxidase - negative i.e <10u

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October 5th, 2011

A 45 year old woman comes in with a 12 week history of numbness and tingling of the right lower extremity. She has been well but has a history of allergic rhinitis and asthma over the past 8 years. On exam she has a sensory defect in a stocking distribution of the right lower leg and weakness of dorsiflexion of the right foot. Her CBC reveals a WBC of 12,000 with 18% eosinophils. Her ESR is 38 mm hr. CMP and UA are normal A CXR is negative.

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September 23rd, 2011

A 58 year old man comes in with a 5 month history of malaise arthralgia, intermittent fever to 101F and weight loss (1o lbs).  He had been previously healthy and on no medications. His exam is unremarkable except some symmetric joint tenderness in the wrists and ankles without synovitis.  Initial laboratory tests revealed a mild anemia of 12.2grms Hb a normal metabolic and renal profile and UA. An ESR is 48mm hr. 

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