Peripheral Neuropathy / Vasculitis patient Recovers Using Rituximab


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Nothing was working in vasculitis and peripheral neuropathy patient when they turned to Rituximab:

"An interesting observation from the present case is the dramatic response of peripheral neuropathy to B-cell depletion with a full recovery of motor strength following initial paralysis," Fanouriakis and colleagues noted.
A patient with refractory eosinophilic granulomatosis and polyangiitis (EGPA) who had severe neuropathy and necrotizing glomerulonephritis and received two cycles of rituximab had a full resolution of his neuropathic symptoms and normal renal function by 20 months, according to Antonis Fanouriakis, MD, of University Hospital of Heraklion in Greece, and colleagues.

In addition, among cases identified in the literature, rituximab was effective for a broad spectrum of disease manifestations, including involvement of the kidney, lung, skin, liver, and heart as well as the peripheral nervous system, the researchers reported online inSeminars in Arthritis and Rheumatism.

Eosinophilic granulomatosis and polyangiitis is the rarest of the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides, a group that also includes granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA).

It shares certain characteristics with GPA and MPA, but also is distinct in featuring high levels of eosinophils in the circulation and tissue, an association with asthma, and cardiac involvement.

The B-cell depleting agent rituximab has been approved for the treatment of GPA and MPA, but thus far has not been evaluated in clinical trials for EGPA. Experience has been limited to case reports and series.

The Case

The patient was a 51-year-old man who reported a 10-day history of arthralgias and numbness in his arms and legs. He had a history of nasal polyps without asthma, and had received treatment with antibiotics and inhaled beta-agonists for a presumed respiratory tract infection 2 weeks before the extremity symptoms began.

At the time of admission, he was wheezing, hypoxic, tachycardic, and had polyarthritis of the hand joints. Muscle strength was reduced in the extremities.

Laboratory tests revealed eosinophilia, with a level of 10,300/mcL (the upper limit of normal is 700/mcL), a serum creatinine of 1.1 mg/dL, and glomerular hematuria with cellular casts.

He was positive for ANCA and had high titers of anti-myeloperoxidase antigen, at 89.8 U/mL, but was negative for rheumatoid factor and anti-cyclic citrullinated peptide antibody.

Nerve conduction studies confirmed the presence of sensorimotor axonal mononeuritis, and renal biopsy showed segmental necrotizing glomerulonephritis with cellular crescents, and numerous eosinophils within the renal infiltrates.

Other potential causes of the eosinophilia, such as parasitic infection, were ruled out, and he was diagnosed with EGPA. His Birmingham Vasculitis Activity Score (BVAS) was 22.

His initial treatment plan was intended to be pulsed methylprednisolone followed by oral prednisone in dosages of 0.5 mg/kg/day and monthly pulses of intravenous cyclophosphamide in dosages of 0.75 mg/m2.

However, his neurologic symptoms worsened, and by day 10 following the first dose of cyclophosphamide he had lost all motor function in one hand and was having gait difficulties. The eosinophilia persisted, and additional nerve conduction studies showed ongoing axonal degeneration.

He then was given rescue therapy with rituximab, with two doses of 1,000 in a 2-week period, and two additional pulses of methylprednisolone.

By 6 months, his neurologic symptoms had improved and laboratory values had normalized. He was given another course of rituximab, followed by maintenance therapy with methotrexate, 25 mg/week, and 20 months after symptom onset his extremity strength and renal function were normal. His BVAS was zero, indicating complete remission.

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