A 57 y.o. presents with sob and hemoptysis

her xray is shown below

What do you think is wrong?

Our patient had ANCA-associated small-vessel vasculitis which includes microscopic polyangiitis, Wegnener’s granulomatosis, Churg-Strauss syndrome and drug-induced vasculitis. Her ANCA titer was 1:2560. The key to recognizing small vessel vasculitis is the involvement of multiple organ systems.  In this case, the lungs and kidneys were involved and the patient presented with a creatinine of 11.  The type of ANCA ( anti neutrophil cytoplasmic antibodies)  can also be tested.  (myeloperoxicase-ANCA or proteinase 3-ANCA now replace C and P ANCA). 

ANCA are specific antibodies for antigens found on neutrophils and monocyte lysomes. ANCA was first  reported in 1982 to cause pulmonary/renal syndromes. Small and medium sized blood vessels become inflamed leading to hemorrhage in the lungs and deposition of antibodies in the kidneys. The most common skin lesion found with this vasculitis is palpable purpura which can be found in Henoch Schonlein pupura.

Henoch Schonlein small vessel vasculitis is caused by IgA

When someone presents with multi organ system disease the differential can also include: atheroembolic disease, endocarditis, antiphospholipid syndrome atrial myxoma and sepsis. Malignancy, such as lymphoma or leukemia can present with dermal vasculitis.

Pt with lymphoma causing small vessel vasculitis

The treatment of small-vessel associated vasculitis is immune suppression;  steroids followed by methotrexate or rituximab. Our patient is still in the hospital pending dialysis.

Niles JL, Bottinger EP, Saurina GR, et al. The syndrome of lung hemorrhage and nephritis is usually an ANCA-associated condition. Arch Intern med 1996;156:440-5.

Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med 1997;337:1512-23.

vanRossum AP,PasHH, Fazzini F, et al. Abundance of the long pentraxin PTX3 at sites of leukocytoclastic lesions in patients with small-vessel vasculitis. Arthritis Pheum 2006;54:986.

Loricera J, Blanco R, Ortiz-Sanjuan F, et al. Single-organ cutaneous small-vessel vasculitis according to the 2012 revised international chapel Hill consensus Conference nomenclature of Vasculitides: a study of 60 patients from  a series of 766 cutaneous vasculitis cases. Rheumatology(Oxford) 2015:54:77.