Paraneoplastic rheumatologic syndromes

Azar, Lama; Khasnis, Atul

doi:10.1097/bor.0b013e328359e780

Cited by 61 publications

(82 citation statements)

References 45 publications

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“…A range of autoimmune phenomena may occur related to malignancy. Regardless the pathogenesis is still unclear, some mechanisms have already been proposed to elucidate this connection, as the release of cytokines and antigens by malignant cells [1,3]. These phenomena are better described on hematological cancers, although a relation with solid tumors, like prostate and ovarian cancers, has already been found [9,13,14].…”

Section: Discussionmentioning

confidence: 99%

“…Cancers are found on 5% of patients with vasculitis [4], and the most common form is leukocitoclastic cutaneous vasculitis [3,14,15], with strongest association to hematological disorders, lung, bowel and renal cancers [4,13,14]. Concerning AAV, the association with cancer is rare, and most cases described are granulomatosis with polyangiitis [1,5,11].…”

Section: Discussionmentioning

confidence: 99%

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confidence: 99%

“…Among these syndromes, paraneoplastic vasculitis are very rare, especially necrotizing systemic vasculitis. It is estimated that 2-5% of all causes of vasculitis are paraneoplastic, with the most common presentation being leukocitoclastic cutaneous vasculitis related to hematological malignancies [1,3,4], and fewer cases related to solid organ tumors as an underlying cause.There are some reports of paraneoplastic ANCA-associated vasculitis (AAV), especially granulomatosis with polyangiitis associated with hematological malignancies and with solid organ tumors such as lung and renal carcinomas [5]. We report a case of eosinophilic granulomatosis with polyangiitis (EGPA) associated to chromophobe renal cell carcinoma (chCRR), an unusual form of malignant renal neoplasm, with complete remission of vasculitis after tumor resection.…”

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confidence: 99%

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confidence: 99%

See 4 more Smart Citations

Eosinophilic Granulomatosis with Polyangiitis Associated to Chromophobe Renal Cell Carcinoma: A Case Report

Oliveira¹,

Simaan²,

Neto³

et al. 2017

J Vasc

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Paraneoplastic syndromes are characterized by the set of signs and symptoms of an underlying cancer, frequently occult, due to a variety of remote tumor effects unrelated to the mechanical impact of the tumor mass or distant metastasis [1,2]. Among these syndromes, paraneoplastic vasculitis are very rare, especially necrotizing systemic vasculitis. It is estimated that 2-5% of all causes of vasculitis are paraneoplastic, with the most common presentation being leukocitoclastic cutaneous vasculitis related to hematological malignancies [1,3,4], and fewer cases related to solid organ tumors as an underlying cause.There are some reports of paraneoplastic ANCA-associated vasculitis (AAV), especially granulomatosis with polyangiitis associated with hematological malignancies and with solid organ tumors such as lung and renal carcinomas [5]. We report a case of eosinophilic granulomatosis with polyangiitis (EGPA) associated to chromophobe renal cell carcinoma (chCRR), an unusual form of malignant renal neoplasm, with complete remission of vasculitis after tumor resection.It is important to be aware of the rare association between vasculitis and cancer, since early recognition could provide proper treatment and better prognosis. A 62 year-old man presented with painful palpable purpura on lower limbs and palms, dry cough, dyspnea and fever four weeks prior to admission. He had a long medical history of hypertension and diabetes, and lifetime tobacco exposure of 35 packyears.There was also a severe persistent asthma starting three years before. Weight loss, abdominal pain and urinary symptoms were absent. On physical examination, there were skin lesions (Figure 1), wheezing on chest auscultation and a body mass index of 27.2 kg/m 2 . Laboratory tests showed marked eosinophilia (5,700 cells/mm 3 at admission, peaking 15,520 cells/mm 3 during hospital stay), elevated erythrocyte sedimentation rate (80 mm/h) and C-reactive protein (2,6 mg/dL, NR <0,5 mg/dL). Renal function and urine analysis were normal. Skin biopsy demonstrated eosinophilic leukocitoclastic vasculitis of small vessels with fibrinoid necrosis and eosinophilic perivascular infiltrates (Figure 2). Antinuclear antibody and antineutrophil cytoplasmic antibodies (ANCA) tested negative. Serological tests for hepatitis B and C viruses and acquired human immunodeficiency virus were negative. At admission, chest radiography revealed pulmonary infiltrates.A computerized tomography (CT) scan was performed, showing bilateral alveolar infiltrates and a small indeterminate solid nodule on left lower lobe. Daily oral prednisone was started (up to 1 mg/kg) with no improvement. The patient evolved with respiratory insufficiency, with severe hypoxemia, and then intravenous (IV) methylprednisolone 1 g/day was prescribed for three days. There was a striking improvement, with normalization of eosinophil count and complete resolution of respiratory symptoms. However, the cutaneous vasculitis not only persisted, but also progressed some days after the IV glucocorticoid therapy, ...

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