Wegener’s Granulomatosis with Antiproteinase-3 Antibodies Occurring after Hodgkin’s Disease

Abstract

We describe the first association between Hodgkin’s lymphoma and Wegener’s granulomatosis, heralded by renal involvement. A 43-year-old man developed rapidly progressive glomerulonephritis requiring chronic hemodialysis 8 months after remission of Hodgkin’s lymphoma. At that moment, no extrarenal involvement was found, despite extensive investigation. Antineutrophil cytoplasm antibodies were positive, without specificity for proteinase-3 or myeloperoxydase. Six months after beginning hemodialysis, multiple pulmonary nodules appeared, along with rapid clinical worsening. A surgical biopsy was performed which disclosed a giant cell granuloma. Antimyeloperoxydase antibodies remained negative, whereas proteinase-3 antibodies became positive. Wegener’s granulomatosis was diagnosed and treatment with cyclophosphamide and steroids was started. Clinical and radiological improvement occurred promptly. Eleven months after treatment, both Wegener’s disease and Hodgkin’s lymphoma remained in remission.

Rapidly progressive diffuse large B‐cell lymphoma with initial clinical presentation mimicking seronegative Wegener's granulomatosis

“…A 4–11‐fold increased risk for developing lymphomas has been observed in patients with Wegener's granulomatosis (6, 7), while an increased risk for lymphoma has also been reported in other rheumatic conditions (8, 9). An interesting case of Wegener's granulomatosis developing in a patient in remission for Hodgkin's lymphoma has also been described (10). Altered immune tolerance associated with these lymphoproliferative diseases or with the immunosuppressive therapy used to treat these diseases probably also plays a role.…”

Intravascular Large B-Cell Lymphoma Complicated by Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis that was Successfully Treated with Rituximab-Containing Chemotherapy

“…[10][11][12][13][14] In the present case, the patient had sinusitis and the titer of PR3-ANCA was high, but the histology demonstrated small vessel vasculitis without granu- There are only five such cases with a definitive diagnosis of vasculitis confirmed by biopsy reported in the literature (Table 1). 6,[16][17][18] Two cases were positive for MPO-ANCA or p-ANCA, and three cases were positive for PR3-ANCA or c-ANCA. Four cases were B-cell malignancies and one case was a T-cell malignancy.…”

“…6 In the remaining case, AAV was followed by lymphoma, suggesting that an immunosuppressive state or the use of cytotoxic drugs, such as cyclophosphamide, might cause lymphoproliferative disorders. 16,18 In the pathogenesis of AAV, ANCA activates neutrophils and vascular endothelial cells. 19 Activated endothelial cells up-regulate adhesion molecules and we could speculate that lymphoma cells can easily aggregate in the microvasculature.…”

Monoclonal Gammopathy Associated with Crescentic Glomerulonephritis and Antimyeloperoxidase Antibodies

“…More recently, a patient with crescentic glo merulonephritis due to Wegener's granulomatosis with c-ANCA. after remission of Hodgkin's lymphoma, has been reported [19], However, the association of monoclonal gammopathy with crescentic glomerulonephritis is very unusual [2][3][4], Experimental studies [20] have demonstrated that a proliferative glomerulonephritis may be induced by plasmocytomas secreting monoclonal immunoglobulins. In addition, the observation that a reduction of the mono- Martin/Martinez Ara/Picazo/PascualSalcedo/Garda Puig/Torre-Carballada/Gil clonal component with plasma exchange was associated with an improvement of glomerular lesions and renal function, strongly favors the hypothesis that plasma cell dyscrasia may trigger a glomerulopathy [3,21].…”