Case Report: An Uncommon Presenting Complaint of GPA

Click to enlarge Table 1.

In light of the positive ANCA serologies with pulmonary and renal manifestations, a diagnosis of granulomatosis with polyangiitis with digital limb ischemia was confirmed.

Plasmapheresis and pulse-dose methylprednisolone were initiated for five concurrent days. With plasmapheresis, her digital ischemia stabilized without further progression. After completion of this therapy, the patient was started on a combination of prednisone and rituximab, with improvement in symptoms over the treatment period (see Figure 2).

Upon discharge she was referred to hand surgery, hyperbaric oxygen therapy and acute rehabilitation. She underwent amputation after four months.

FIGURE 2: Progression of digital limb ischemia over two-week hospital course. Gangrenous progression occurred in digits 1–4 on the right hand and digits 2–5 on the left hand. (Click to enlarge.)

Discussion

Digital limb ischemia and gangrene are rare clinical manifestations of GPA.^2-5

Lau et al. conducted a literature review and found 16 other case reports of GPA associated with digital limb ischemia.² Since that case report publication in 2017, at least two other cases have been reported.^3,4 According to previous studies, most cases of digital ischemia associated with GPA have presented with mainly upper extremity involvement, as seen in our patient.²

The most reported presenting signs and symptoms of GPA usually involve the ear, nose and throat (~73%), lungs (~45%), kidney (~18%) and eyes (~15%).⁶ Onset of common manifestations of GPA, such as hematuria and hemoptysis, did not present for at least one week into our patient’s hospital course. However, our patient did have an episode of sinusitis one month prior to onset of digital limb ischemia, consistent with GPA.

Digital ischemia and gangrene are more commonly associated with thromboangiitis obliterans, a medium vessel vasculitis.⁷ The pathophysiology of ischemia in vasculitis has been hypothesized to originate from the destruction of these vessels from active inflammation; however, arterial thrombi on biopsies have also been reported.²

Because digital limb ischemia is a rare presentation of small vessel vasculitis, the pathophysiology is not well studied; however, it has been presumed to be similar to the proposed pathophysiology of medium vessel vasculitis.²

Other case reports with digital limb ischemia associated with GPA have also reported mononeuropathy, as seen in our patient.^3,8 This is a common clinical finding in vasculitis, resulting from ischemia of a focal nerve trunk.⁹ This finding further supported the diagnosis of GPA.