School of Medicine and Health Sciences Poster Presentations
65 Year Old Woman with Hemoptysis: a Case Report/review of Literature Discussing Scleroderma,vasculitis, & Malignancy
Poster Number
240
Document Type
Poster
Publication Date
3-2016
Abstract
A 66 year-old Hispanic Caucasian lady with a 14 year history of diffuse scleroderma and bilateral stage II-B invasive breast cancer presented for evaluation of a vasculitic rash and hemoptysis. On examination vital signs were stable and cardiovascular exam was normal. Pulmonary exam revealed coarse crackles to the mid-lung fields bilaterally with bronchial breath sounds at the left apex. Abdominal exam was benign with no hepatosplenomegaly. Musculoskeletal exam revealed bilateral sclerodactyly with modified Rodnan Skin Score of 14 and contractures of the digits. There was a large punched-out ulceration over the right third metacarpophalangeal joint measuring 1x0.7x0.2 cm with desiccated tendon in the base. Multiple similar ulcers were noted over other joints, and on the legs she had a petechial rash consistent with leukocytoclastic vasculitis.
CT thorax revealed multiple lung nodules and masses, bilateral lower lobe honeycombing, bibasilar reticular changes with traction bronchiectasis, ground glass opacities in bilateral upper lobes with multiple calcified granulomata with cavitation.
Laboratory evaluation revealed normocytic anemia, ESR 23 mm/hr, CRP 14 mg/dL, c-ANCA titer of 1:640, with proteinase-3 antibody of 39.9 U/mL. Anti-nuclear antibody was positive at a titer of 1:160 homogenous pattern and additional serologic testing revealed positive RNA polymerase III antibody at 33.4 units.
Differential diagnosis included infections causing cavitating pneumonias, granulomatosis with polyangiitis and other ANCA-associated vasculitides, and primary or secondary malignant lung lesions.
The patient underwent left thoracoscopic wedge resection of the left lower lobe. Gram stain, acid fast and fungal stains with cultures were all negative. Histopathology demonstrated background fibrosis, cyst formation, and bronchial metaplasia consistent with scleroderma-associated lung disease. Additionally, areas of necrotizing granulomatous inflammation and vasculitis were seen consistent with focal vasculitis. Subjacent to the pleura atypical epithelial proliferation was seen and these cells stained diffusely positive for MAK-6, GATA-3, estrogen receptor and mammoglobulin. Taken together the findings were consistent with RNAP III-positive diffuse scleroderma with associated breast cancer, presenting with lung metastases and ANCA-associated vasculitis consistent with limited GPA.
The patient was treated with pulse-dose glucocorticoids for 3 days followed by prednisone 60 mg daily along with Rituximab for her ANCA-associated vasculitis. The breast cancer was treated with anastrazole 1 mg daily. She is currently doing well. This case emphasizes the now well-recognized co-temporal relationship between scleroderma and malignancy, and the emerging pathophysiologic understanding of the relationship between autoantigen mutations in malignant tissues and autoimmune triggering.
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65 Year Old Woman with Hemoptysis: a Case Report/review of Literature Discussing Scleroderma,vasculitis, & Malignancy
A 66 year-old Hispanic Caucasian lady with a 14 year history of diffuse scleroderma and bilateral stage II-B invasive breast cancer presented for evaluation of a vasculitic rash and hemoptysis. On examination vital signs were stable and cardiovascular exam was normal. Pulmonary exam revealed coarse crackles to the mid-lung fields bilaterally with bronchial breath sounds at the left apex. Abdominal exam was benign with no hepatosplenomegaly. Musculoskeletal exam revealed bilateral sclerodactyly with modified Rodnan Skin Score of 14 and contractures of the digits. There was a large punched-out ulceration over the right third metacarpophalangeal joint measuring 1x0.7x0.2 cm with desiccated tendon in the base. Multiple similar ulcers were noted over other joints, and on the legs she had a petechial rash consistent with leukocytoclastic vasculitis.
CT thorax revealed multiple lung nodules and masses, bilateral lower lobe honeycombing, bibasilar reticular changes with traction bronchiectasis, ground glass opacities in bilateral upper lobes with multiple calcified granulomata with cavitation.
Laboratory evaluation revealed normocytic anemia, ESR 23 mm/hr, CRP 14 mg/dL, c-ANCA titer of 1:640, with proteinase-3 antibody of 39.9 U/mL. Anti-nuclear antibody was positive at a titer of 1:160 homogenous pattern and additional serologic testing revealed positive RNA polymerase III antibody at 33.4 units.
Differential diagnosis included infections causing cavitating pneumonias, granulomatosis with polyangiitis and other ANCA-associated vasculitides, and primary or secondary malignant lung lesions.
The patient underwent left thoracoscopic wedge resection of the left lower lobe. Gram stain, acid fast and fungal stains with cultures were all negative. Histopathology demonstrated background fibrosis, cyst formation, and bronchial metaplasia consistent with scleroderma-associated lung disease. Additionally, areas of necrotizing granulomatous inflammation and vasculitis were seen consistent with focal vasculitis. Subjacent to the pleura atypical epithelial proliferation was seen and these cells stained diffusely positive for MAK-6, GATA-3, estrogen receptor and mammoglobulin. Taken together the findings were consistent with RNAP III-positive diffuse scleroderma with associated breast cancer, presenting with lung metastases and ANCA-associated vasculitis consistent with limited GPA.
The patient was treated with pulse-dose glucocorticoids for 3 days followed by prednisone 60 mg daily along with Rituximab for her ANCA-associated vasculitis. The breast cancer was treated with anastrazole 1 mg daily. She is currently doing well. This case emphasizes the now well-recognized co-temporal relationship between scleroderma and malignancy, and the emerging pathophysiologic understanding of the relationship between autoantigen mutations in malignant tissues and autoimmune triggering.
Comments
Presented at: GW Research Days 2016