Polyarteritis nodosa complicating multiple myeloma - a case report and review of the literature.
BACKGROUND
Polyarteritis nodosa (PAN) is a necrotizing vasculitis of small to-medium-sized vessels, rarely associated with hematologic neoplasms.


METHODS
We report a 44-year-old man with a history of monoclonal gammopathy of undetermined significance (MGUS) who presented with rapidly progressing sensorimotor peripheral neuropathy. Two weeks after onset the patient developed severe acute acral and retinal ischemia. MR-angiography and nerve biopsy revealed a systemic necrotizing vasculitis (PAN type). At this time, bone marrow biopsy identified a smoldering multiple myeloma. Immediate immunosuppressive and anti-neoplastic treatment (steroids, immunoglobulins, bortezomib combined with cyclophosphamide followed by lenalidomide maintenance) resulted in a favorable clinical outcome. After 4 years, the patient is in good clinical condition with sustained partial remission from myeloma and without evidence of relapse of PAN.


CONCLUSIONS
This is a remarkable case of a histologically confirmed peripheral neuropathy due to polyarteritis nodosa associated with progression of MGUS to multiple myeloma. Immediate diagnosis and combined immunosuppressive and anti-neoplastic treatment may improve the outcome of this potentially life-threatening clinical condition.
Markus
M
Hutterer
Michael
M
Steurer
Romana
R
Höftberger
Johannes A
JA
Hainfellner
Andreas
A
Pircher
Martha
M
Nowosielski
Peter
P
Hau
Wolfgang
W
Löscher
Eberhard
E
Gunsilius
Thomas
T
Berger
Guenther
G
Stockhammer
D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D004487:Edema / Q000209:etiology; D004576:Electromyography; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D009101:Multiple Myeloma / Q000150:complications* / Q000188:drug therapy / Q000473:pathology*; D009431:Neural Conduction; D009460:Neurologic Examination; D010523:Peripheral Nervous System Diseases / Q000209:etiology / Q000473:pathology; D010488:Polyarteritis Nodosa / Q000150:complications* / Q000188:drug therapy / Q000473:pathology*
D000893:Anti-Inflammatory Agents; D000970:Antineoplastic Agents; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D008775:Methylprednisolone