Acute renal failure with polyarteritis nodosa and multiple myeloma

Williams, Andrew; Newland, Adrian C.; Marsh, Frank

doi:10.1136/pgmj.61.715.445

Cited by 10 publications

(5 citation statements)

References 20 publications

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“…Occasional cases of PAN associated with myeloma or paraproteinemia have been described. Williams et a1 (17) reported a patient with acute renal failure who was found to have both PAN and multiple myeloma, and they speculated that these were etiologically related. Bouvet and Delrieu (18) documented the case of a patient with PAN and a biclonal gammopathy who subsequently developed amyloidosis with severe nephrotic syndrome.…”

Section: Discussionmentioning

confidence: 99%

IgA multiple myeloma presenting as Henoch‐Schönlein purpura/polyarteritis nodosa overlap syndrome

Birchmore

Sweeney

Choudhury

et al. 1996

Arthritis & Rheumatism

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We report the unusual case of a man with a 5-year history of relapsing Henoch-Schonlein purpura (HSP) and macroscopic polyarteritis nodosa (PAN) as early manifestations of IgAK multiple myeloma. The glomeruli contained monoclonal IgAK deposits, without other immunoglobulins or A light chains. Glomerular deposits lacked the usual electron density but could be demonstrated by immunoelectron microscopy. Multisystem large vessel vasculitis (antineutrophil cytoplasmic antibody negative) produced aneurysms of renal and hepatic arteries, whereas small vessel vasculitis affected the skin and glomeruli, producing a symptom complex of HSP with dermal and glomerular deposits of IgA. We conclude that HSP/overlap syndrome may be the initial manifestation of IgA myeloma and should be considered within the spectrum of monoclonal IgA deposition disease.Henoch-Schonlein purpura (HSP) is a leukocytoclastic vasculitis of cutaneous venules, capillaries and arterioles, often associated with mesangial glomerulonephritis, and sometimes with a necrotizing intestinal vasculitis. IgA deposits are found in areas of cutaneous vasculitis and are usually present in the renal mesangium (1). HSP with nephritis is common in children, but rare in adults. It sometimes follows respiratory tract infections and in such cases is thought to be due to stimulation of the mucosal immune system with formation of circulating immune complexes (ICs) containing polyclonal IgA (2). HSP can also occur in patients with cirrhosis, and in those cases is thought to be due to defective clearance of IgA-containing ICs (3). Rare cases of HSP associated with monoclonal IgA production have been reported (4).Another IgA-mediated disorder, IgA nephropathy, is the most common primary glomerulonephritis in adults and is a common cause of renal failure. The pattern of IgA deposits and glomerular injury in HSP resembles that seen in IgA nephropathy, and many investigators now consider IgA nephropathy to be a renal-limited form of HSP. We report herein an unusual case of HSP with monoclonal IgAKdomerular deposits associated with antineutrophil cytoplasmic antibody (ANCA)-negative polyarteritis nodosa (PAN) as a complication of IgAK multiple myeloma. CASE REPORTThe patient, a 50-year-old man, developed a purpuric rash on his arms, abdomen, buttocks, and legs, associated with abdominal pain, arthralgias, myalgias, a painful neuropathy, and peripheral edema. Evaluation at another hospital revealed proteinuria and microscopic hematuria. The results of tests for antinuclear antibody (ANA) and cryoglobulins were negative. Biopsy of a purpuric skin lesion showed leukocytoclastic vasculitis, and a diagnosis of HSP was made. He was treated with prednisone 0.5 mg/kg/ day but continued to have arthralgias, myalgias, proteinuria, microscopic hematuria, and flares of purpura. Oral cyclophosphamide was then instituted at a dosage of 1 mg/kg/day, but symptoms and urinary sediment abnormalities continued. A second biopsy of a purpuric skin lesion revealed leukocytoclastic vasculitis with vasc...

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Section: Discussionmentioning

confidence: 99%

IgA multiple myeloma presenting as Henoch‐Schönlein purpura/polyarteritis nodosa overlap syndrome

Birchmore

Sweeney

Choudhury

et al. 1996

Arthritis & Rheumatism

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“…Vasculitis is rarely accompanied by MM. To the best of our knowledge, nine cases of PAN with a complication of MM, including our case, have been reported (7)(8)(9)(10)(11)(12)(13)(14) (Table). The reports show that the mean age of these patients was 54.3 years (range, 37-78 years).…”

Section: Discussionmentioning

confidence: 78%

Acute Kidney Injury in a Patient with Polyarteritis Nodosa and Multiple Myeloma

et al. 2014

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We herein report the case of a Japanese man with polyarteritis nodosa (PAN) accompanied by multiple myeloma (MM). The patient was diagnosed with PAN. Concurrently, IgG kappa paraprotein was detected, and bone marrow changes indicative of MM were observed. Prednisolone (PSL) administered at a dose of 30 mg/day was initiated; however, the serum creatinine level increased. In spite of increasing the dose of PSL to 45 mg/day and initiating treatment with double filtration plasmapheresis, the patient's renal dysfunction continued to progress and haemodialysis was introduced. He died from pneumonia 12 months after admission. We conclude that renal failure is an important risk factor in the prognosis of PAN accompanied by MM.

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“…Acute renal failure is a very rare manifestation in PAN; moreover, Samarkos et al [3] stated that most of the reported cases of acute renal failure complicated with PAN were associated with concomitant use of nephrotoxic agents such as contrast media [4,5] or other aggravating conditions such as multiple myeloma [6]. As compared with MPA, in which more active urine sediments are observed because of necrotizing crescentic glomerulonephritis, renal insufficiency in PAN is characterized by mildly active urine sediments, since it is primarily caused by renal ischemia because of luminal narrowing of the mediumsized arteries in the kidney [7] as observed in our case.…”

Section: Discussionmentioning

confidence: 99%

An autopsy-proven case of myeloperoxidase-antineutrophil cytoplasmic antibody-positive polyarteritis nodosa with acute renal failure and alveolar hemorrhage

et al. 2010

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An 80-year-old woman positive for myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) was admitted with a 3-month history of fever, general malaise, and weight loss, after unsuccessful treatment with antibiotics. Upon admission, her fever persisted, and there was concomitant deterioration of renal function without active urine sediments. Furthermore, she developed hemoptysis, and chest computed tomography (CT) scan revealed bilateral diffuse alveolar hemorrhage. Although a renal biopsy was not performed because of her dementia, we initially suspected microscopic polyangiitis (MPA) on the basis of her clinical course. Because of her poor general condition, she was administered a low dose of prednisolone. Although her fever subsided, she suffered from intractable alveolar hemorrhage and eventually died from respiratory failure. During the autopsy, fibrinoid necrosis was restricted to medium-sized arteries, including the arcuate arteries of the kidneys and the bronchial arteries, without necrotizing crescentic glomerulonephritis and alveolar capillaritis. Therefore, polyarteritis nodosa (PAN) was diagnosed. It is important to distinguish between MPA and PAN because they can lead to life-threatening complications, and their treatment strategies and prognosis are different. When a patient presents with MPO-ANCA, alveolar hemorrhage, and acute renal failure with little evidence of glomerulonephritis, a differential diagnosis of PAN should be made; however, it is difficult to do so without pathological findings. Therefore, pathological examination should be carried out whenever possible.

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Acute renal failure with polyarteritis nodosa and multiple myeloma

Cited by 10 publications

References 20 publications

IgA multiple myeloma presenting as Henoch‐Schönlein purpura/polyarteritis nodosa overlap syndrome

IgA multiple myeloma presenting as Henoch‐Schönlein purpura/polyarteritis nodosa overlap syndrome

Acute Kidney Injury in a Patient with Polyarteritis Nodosa and Multiple Myeloma

An autopsy-proven case of myeloperoxidase-antineutrophil cytoplasmic antibody-positive polyarteritis nodosa with acute renal failure and alveolar hemorrhage

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