1. Photo or Graphic
Continue’d presentation and hospital course
• Initially patient was hypoxemic but responsive to face mask so no intubation was done,solu-Medrol 60 q6h
was started in addition o the ABX.
• Labs on presentation: WBC: 17000 with 15% eosinophils no bands. ABG revealed PaO2 of 68 on room
air and 85 on 5L face mask
• Basic metabolic panel, liver function test, HIV, sputum culture, blood culture, urine strep, legionella,
influenza, hepatitis panel were all negative
• C-ANCA was positive and ESR>50,CRP:12 with normal RF, ANA, P-ANCA C3 C4, cryoglobulins, EKG
(no signs of atrial enlargement)
• AFB, histoplasmosis, fungus culture, aspergillus, blastomycosis, strongyloides, cryptococcal antigens
were negative
• CT scan chest revealed: bilateral infiltrates with no cavitary lesions and no PE
• WBC count continued to rise: 20000 to 33000, respiratory status was stable so a CT guided biopsy of the
lung was done which was inconclusive.
• An open lung biopsy was done and showed: focal acute angitis, giant cell granulomatosis, acute and
chronic compatible with Wegener’s granulomatosis
• Anti GBM was also as part of the workup for hemoptysis and came back positive
Discussion
• This case is unusual for 3 key reasons:
• 1. While it is reported in 30 % of Good-
Pastures disease, it is extremely unusual to
find a dual positive Wegener’s disease with +
Anti-GBM and C-ANCA
• 2.The mean age for diagnosing Wegener’s
disease is 40, while in this case it is 22 years
• 3.Completely normal kidney function and UO,
with full field of hematuria on UA, and it stayed
normal after discharge
• It is very important to differentiate between the
causes of hematuria especially in young
patient, as it most commonly includes
vasculitis, autoimmune, infection in that age
group.
• Treatment differs in all these cases from ABX,
immunosuppressive medication to plasma
exchange therefore a detailed history, with a
full autoimmue, vascultitic, and infectious
workup should be done with the biopsy being
the only definite way of diagnosing the
different types as they may also overlap.
References
1.Fauci AS, Haynes BS, Katz P, Wolff SM. Wegener's granulomatosis:
prospective clinical and therapeutic experience with 85 patients for 21 years.
Ann Intern Med. January 1983;98(1):76-85.
2.Manganelli P, Fietta P, Carotti M, Pesci A, Salaffi F. Respiratory system
involvement in systemic vasculitis. Clin Exp Rheumatol. March-April
2006;24:S48-S59. [Medline].
3.Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: an analysis
of 158 patients. Ann Intern Med. Mar 15 1992;116(6):488-98.
4.Holle JU, Gross WL. Neurological involvement in Wegener's granulomatosis.
Curr Opin Rheumatol. Jan 2011;23(1):7-11.
5.Mukhtyar C, Guillevin L, Cid MC, et al. EULAR recommendations for the
Imaging, Microscope and Diagnosis
Introduction
• Double positive good-pastures syndrome for
both C-ANCA, anti –GBM is reported in 30 %
of cases, however a double positive wegner
disease with normal kidney function in a
young adult is extremely uncommon
• This is a case of a lung biopsy proven wegner
disease with + anti Gbm and C-ANCA. Full
field RBC with no casts on UA and normal
kidney function in a 22 year old male
Case presentation
• 22 years old hispanic male, Jehovah's witness
with no past medical history in his usual state
of health before presentation to the
emergency department for 3 days of fever,
shortness of breath, cough, mild hemoptysis,
fatigue, pain and soreness in the back and
both legs
• A Doppler U/S of lower extremities did not
reveal any DVT; a CT angiogram of the chest
was negative for pulmonary embolism but it
did show multiple bilateral, peripheral, and
peribronchial consolidations with generalized
groundglass, patchy opacities particularly in
the lower lobes likely representing organizing
pneumonia or acute interstitial pneumonia
with no pathological adenopathy
• Patient was initially started on Azithromycin
and Ceftriaxone in the ED to cover CAP since
he had no hx of any immunodeficiencies,
hospitalizations, or travel.
• To note patient was transferred from a
different hospital to our ED after an initial
bronchoscopy was inconclusive with no
improvement in his repiratory status and for
the EHMC bloodless program
A Case of Wegner’s Disease with Double Positive
C-ANCA and ANTI-GBM in a 22 year old
Youssef Yaacoub MD, Hanaa Benchekroun MD, Pierre El Hachem MD, Craigh Bryan MD