[MIM 608 710]

Rare: 1-9/100,000.  Granulomatous inflammatory vasculitis of small diameter blood vessels affecting, classically, the upper and lower airways and the kidneys. An autoimmune origin is likely and an E Coli infection is often at the origin of the disease. The average age of onset is 45 years but pediatric cases have been described.

Diagnostic criteria: in addition to systemic signs (fever, weight loss, fatigue), the presence of at least 3 of the following 6 criteria:

-        granulomatous inflammation at the biopsy of a lesion

-        involvement of the upper respiratory tract: hemorrhagic rhinitis, frequent epistaxis, purulent nasal discharge, sometimes septal perforation or destruction of cartilage (saddle deformation of the nasal septum); chronic sinusitis

-        laryngo-tracheo-bronchial involvement: ulcerative lesions in the mouth and tonsils

-        pulmonary involvement: nodules, chronic infiltrates, cavities, hemoptysis; the evolution towards stenotic bronchial lesions is most frequent in children

-        renal impairment: hematuria, proteinuria, focal necrotizing or proliferative  extracapillary glomerulonephritis (crescent-shaped) without immune complexes or Ig deposits

-        presence of antibodies against the cytoplasm of polynuclear neutrophils (ANCAS), in particular against proteinase 3 (PR3)

Additional signs:

-        skin lesions: purpura, papules (10-50%)

-        nerve damage: mononeuritis (10-70%); headache, hemiplegia, seizures (6-13%)

-        ocular lesions

-        heart involvement: rhythm disorders, pericarditis

Treatment: in emergency: repeated plasmapheresis; corticosteroids and cyclophosphamide. Ongoing trials with rituximab. Risk of relapse.

Anesthetic implications:

monitor renal function, heart function; RX thorax; avoid nasal intubation; side effects of the treatment: total blood count, corticosteroid therapy; risk of difficult intubation in case of laryngeal or tracheal lesions (stenosis, hemorrhage); it is wise to opt for a ETT diameter smaller than usual to avoid laryngotracheal lesions and subsequent subglottic stenosis.

References : 

-        Lake CL. 
Anesthesia and Wegener’s granulomatosis: case report and review of the literature. 
Anesth Analg 1978; 57: 353-9

-         Riley RH, Knox GJ, Yuen P. 
Airway crisis during anesthesia in a patient with Wegener’s granulomatosis. 
J Anesth 1997; 11: 234-6

-         Passey J., Walker R.
Wegener's granulomatosis: an unusual case of upper airway obstruction.
Anaesthesia 2000: 55: 682-4

-        Slater RM. 
Anaesthetic airway management of subglottic stenosis in Wegener’s granulomatosis. 
Br J Anaesth 2011 ('blue E-letters' published on May 16, 2011)

-         Cooper A, Rakheja D, Punaro M. 
A 14 year-old boy with sore throat and tea-colored urine. 
Pediatrics 2014; 133: e 1377-80.

-        O’Hear KE, Ingrande J, Brodsky JB et al.
Tracheal stenosis because of Wegener granulomatosis misdiagnosed as asthma.
A &A Case Reports 2016; 6: 311-2.

-        Geng-Ramos G, Nami N, Mener DJ.
Granulomatosis with polyangiitis-associated subglottic stenosis in a 13 year-old : a case report.
Pediatr Anesth 2016 ; 26 : 1112-4

Updated: October 2016