A 35 y.o. woman presented with cough, myalgias and hearing loss

this was her cxr

Our patient had granulomatous polyangiitis(GPA) .  This was formerly known at Wegener’s and is a small-medium sized necrotizing vasculitis.  It is part of a large group of disorders called anti-neutrophil-cytoplamic antibody (ANCA) associated disease. These diseases have  lung, kidney and otorhinolargic manifestations. A useful way to remember these findings is the classification by DeRemee  ELK( E standing for ears and otorhinologic, L for lung and K for kidney).  

saddle nose deformity can occur in granulomatous polyangiitis.

In 1936, a German pathologist Friedrich Wegener described three cases of this distinctive form of vasculitis.  It was named after him but when his Nazi ties came to light in 2000, the name was changed to granulomatosis with polyangiitis. Antineutrophil antibodies have been shown to react with proteinase 3 (PR3) which is an enzyme present in  neutrophils  causing them to de-granlulate and damage endothelial cells.

 ANCA is present is 80-90% of patients with disease. C-ANCA is directed against cytoplasmic antibodies and p-ANCA is directed against myeloperoxidase antibodies. The reason for production of antineutrophil antibodies is not known. There have been many postulated associations with GPA including genetic abnormalities like alpha 1 antitrypsin or HLA-DP  and infectious causes like hep C.

While nasal involvement makes GPA most likely in the case of our patient and ANCA confirms it; there are many pulmonary renal syndromes which can present with CXR abnormalities .

While our patient presented with deafness, she developed hemoptysis two months later and this is when her  CXR was taken. She had hematuria at that time as well. She was positive for c-ANCA 1:160 and PR3/+ as well.  She was treated with immunosuppression but returned with worsening renal failure. She was treated with steroids and is receiving rituximab infusions.  She is on dapsone for PCP prophylaxis.

The expected life span with treatment is 8-9 years.

A pt with Goodpasture’s presenting with a similar CXR

DeRemee R, McDonald T, Harrison E, et al. Wegener’s granulomatosis. Anatomic correlates, a proposed classification. Mayo Clin Proc. 1976 Dec;51(12):777-81.

Qasum A, Patel J. ANCA positive vasculitis StatPearls https://www.ncbi.nlm.nih.gov/books/NBK554372/

Jones R, Tervaert J, Hauser T,, et al. European Vasculitis Study Group. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. NEJM. 2010 Jul 15;363(3):211-20.