6/14/2013 Two Atypical Cases of Case 1 Temporal Arteritis 62 year old African-American female with no significant PMH presenting with: Persistent dry cough x 6 weeks. Fatigue x 6 weeks. Weight loss of 20 lbs/2 months. Bilal Muzaffar, MD Rheumatology Fellow Emory University School of Medicine Case 1 Case 1 Review of Systems: Physical Exam: No history of headaches, no visual changes or Persistent low grade fevers jaw claudication No temporal artery tenderness No abnormal pulsations of temporal artery No symptoms suggesting Polymyalgia Normal chest exam Rheumatica Rest of the physical exam was unremarkable Rest of the review of systems negative Case 1 Case 1 Labs: Summary: 62 year old female patient with cough, fever, fatigue Hb - 7 g/dl and elevated inflammatory markers Platelet count – 500,000/ul Alkaline phosphates -150 U/L Differential diagnosis: • Infections ESR - 120 mm/hr • Malignancy • Connective tissue disorders and Vasculitis CRP – 220 mg/l 1 6/14/2013 Case 1 Case 1 Labs Continued: Labs Continued: Ferritin – 1000 ng/ml PPD negative HIV negative UA - Normal Blood cultures negative x 4 SPEP - Negative CXR - Normal UPEP - Negative Case 1 Case 1 Further Work-up: PET-CT Scan: CT Chest, Abd and Pelvis - Unrevealing TTE - No vegetations Bone marrow biopsy- Normal Increased FDG avidity along the major vessels and aorta suggesting the possibility of vasculitis CASE 1 CASE 1 Temporal artery biopsy: The vessel lumen is nearly occluded. Inflammation in all three of the vessel layers. Giant cells identified. The IEL is fractured and reduplicated. • The vessel lumen is nearly occluded. • Inflammation in all three layers of the Consistent with the diagnosis of GCA vessel. • Giant cells identified. • The IEL is fractured and reduplicated. 2 6/14/2013 Case 1 DISCUSSION Treatment: Typical manifestations of GCA: Headache Prednisone 60 mg daily Quick Jaw claudication resolution of her symptoms of cough, fatigue Visual changes and fever Polymyalgia symptoms Prednisone tapered to 20 mg with no Abnormalities of Temporal artery recurrence of symptoms Systemic signs of fever, fatigue, weight loss Hellmann DB, Giant cell arteritis, 1998 DISCUSSION DISCUSSION Atypical manifestations of GCA: Dry cough 88 patients with biopsy proven GCA: Arm and Tongue claudication Unexplained anemia Dry cough was a clinical feature in 12 cases. Mesenteric ischemia Sensorinueural hearing loss Dry cough was the only manifestation along with Elevated serum Alkaline phosphatase levels systemic inflammation in 2 cases. TIA/stroke Thierry zenone, et al. Rheumatol int,2012 DISCUSSION DISCUSSION In the same study a correlation was found between Clinical findings in patients with or without Dry inflammatory biomarkers and presence of dry cough cough. The mean CRP: 153.8 mg/l in patients with dry cough 94 mg/l in patients without dry cough Zennone et al. Rheumtol Int, 2012 3 6/14/2013 DISCUSSION DISCUSSION Mechanism of dry cough in GCA not well understood. Conclusion: Various postulates Recognition of dry cough as a clinical feature of Involvement of pulmonary vasculature GCA can avoid diagnostic confusion and prevent Granulomatous inflammation of the bronchial wall extensive pulmonary workup. Vasculitis in the area of cough receptors Early diagnosis and prompt initiation of steroids will prevent the possible serious visual complications. Zenone et al. Rheumtol Int, 2012 Case 2 Case2 73 year old African-American man 4 months prior: with PMH of HTN presenting with: Rheumatoid arthritis diagnosed because of arthritis of his small joints of hands, wrists and Ankles Fatigue x 1 months weight loss- 40 lbs/3-4 months Started on Prednisone 10 mg bid which was Headache x 3 weeks tapered to 10 mg. His arthritic symptoms were under control at the time of presentation. Case 2 Case2 One month prior to presentation: Physical Exam: Afebrile Admitted with acute ischemic posterior No scalp tenderness and normal temporal circulation stroke artery pulsations He had persistent fevers, bi-basilar lung b/l basal crepitations on lung exam infiltrates and was treated with Abx Normal cardiac exam Bilateral lower extremity edema 2+ Fevers persisted despite Abx and no infectious etiology was established No skin rashes, No synovitis, Normal neurological exam 4 6/14/2013 Case 2 Case 2 Labs: Labs continued: CBC Hgb - 9 g/dl Liver enzymes-normal WBC -12,000/ul Eosinophil count -40% Hepatitis studies (B and C)- negative Chemistry Creatinine -1.1 -1.5 mg/dl Thyroid functions- normal Ferritin - 680 ng/ml ESR - 100 mm/hr Albumin - 1.8 mg/dl UA -trace protein Case 2 CASE 2 Summary: Differential diagnosis: EGPA (Chrug strauss) 72 M with h/o RA presenting with headache, fatigue, Rheumatoid vasculitis eosinophilia, elevated inflammatory markers and Parasitic infection. Pulmonary infiltrates on Imaging Hematologic malignancy. Hypereosinophilic syndrome. Giant cell arteritis. Case 2 Case 2 Labs Continued: Labs Continued: RF - 1000 U/dl ANA and ANCA - negative Anti CCP-Ab >250 U/dl Parasitic studies - negative ESR - 100 m/hr HIV - negative CRP - 120 mg/l Blood cultures - persistently negative BNP - normal SPEP and UPEP - normal 5 6/14/2013 Case 2 Case 2 Further Work-up: Temporal artery biopsy: TEE- EF of 35%. No vegetations Extensive transmural granulomatous Bone marrow biopsy -Normal infiltrate with abundance of eosinophils and fragmentation of the elastica Upper and lower GI endoscopy -Normal Giant cells were not identified CT Chest – Bi-basilar ground glass opacities CASE 2 CASE 2 Biopsy Biopsy findings findings Transmural inflammation with abundance of eosinophils and fragmentation of the elastica •Giant cells not identified Case 2 Case 2 Diagnosis: Treatment : Eosinophilic temporal artery vasculitis Started on high dose steroids: Secondary to RA vs Evolving EGPA Eosinophil count rapidly decreased to normal and Cardiac involvement and stroke secondary to marked improvement of his systemic symptoms of eosinophilic vasculitis ? fatigue and headaches 6 6/14/2013 Case 2 Case 2 Discussion: Discussion: Histological features of this case not consistent Some of these cases fulfill the criterion for EGPA with diagnosis of GCA either at presentation or on follow up. Have to consider other systemic vasculitis known Most of these cases remain uncharacterized as to Cause TA vasculitis eosinophilic temporal and systemic arteritis Eosinophilic TA vasculitis usually characterized by In our patient with RA with high RF and anti-ccp peripheral eosinophilia and by non– giant cell antibody titres, Rheumatoid vasculitis is the most granulomatous inflammation with eosinophilic likely underling diagnosis infiltrate of the temporal artery Grisham E,et al Human pathol 1995 Case 2 Case 2 Vasculitis of temporal artery: Conclusion: Polyarteritis nodosa All temporal arteritis is not GCA Amyloidosis EGPA (Churg-Strauss syndrome) Temporal artery vasculitis may be an indicator of Microscopic polyangiitis underlying systemic vasculitis. GPA (Wegener’s granulomatosis) Cryoglobulinemic vasculitis Distinction between TAB-localized Systemic vasculitis and GCA can guide the choice of therapy. Rheumatoid vasculitis Thierry ge´ne´reau,et al Arthritis and Rheumatism ,December 1999 THANK YOU 7.