- Email: [email protected]
Diffuse Alveolar Hemorrhage (DAH) in Hepatitis C Virus (HCV) Associated Cryoglobulinemia After Completion of HCV Therapy
Diffuse Lung Disease SESSION TITLE: Student/Resident Case Report Poster - Diffuse Lung Disease SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM
Diffuse Alveolar Hemorrhage (DAH) in Hepatitis C Virus (HCV) Associated Cryoglobulinemia After Completion of HCV Therapy Abhijai Singh MD Chandrakanta Chuturvedula MD Gurbir GIll MD* Reshma Abraham MD; and Pieusha Malhotra MD St Vincent Hospital, Shrewsbury, MA
DIFFUSE LUNG DISEASE
INTRODUCTION: DAH in the setting of cryoglobulinemia after HCV therapy completion is unreported in the absence of glomerular disease. CASE PRESENTATION: A 62-year-old lady with a past medical history of hypertension, coronary artery disease and hypothyroidism presented with new onset purpuric rash on bilateral lower extremities. . Review of systems was negative except for pruritus around the rash. The patient denied any new medication, sick contacts or tick exposure. On examination the patient was afebrile with normal vitals. Skin revealed palpable purpuric lesions with no significant necrosis. Laboratory workup is summarized in table 1. Skin biopsy came back positive for leukocytoclastic vasculitis. A diagnosis of type 2 mixed cryoglobulinemia (type 2 MC) was established. Patient was started on treatment for hepatitis C with Ledipasvir/Sofosbuvir combination and 12 weeks later patient achieved sustained viral response (SVR). 6 months later, she presented to the emergency department with acute hypoxemic respiratory failure. CT scan identified patch bilateral ground glass opacities. The patient was admitted to the intensive care on Bipap. She underwent an urgent bronchoscopy. Bronchoscopic alveolar lavage was positive for hemosiderin laden alveolar macrophages. A diagnosis of diffuse alveolar hemorrhage (DAH) was established. HCV RNA was documented negative during this admission. Cultures including special stains for pneumocystis were unrevealing. The patient was started on a combination of pulsed methylprednisolone and plasmapheresis and recovered after 5 sessions. DISCUSSION: DAH is a very rare phenomenon in the presence of Cryoglobulinemia with unclear prevalence. Hospital based studies have identified an incidence of 2-3 % (1,2). Disease activity usually corresponds to HCV RNA levels and virologic remission has been shown to cause cryoglobulinemia quiescence. DAH despite RNA clearance begs the question if HCV antibodies have the ability to propagate disease activity independent of SVR. Absence of glomerular disease, lack of Hypocomplementemia and negative rheumatoid factor are other unique features to our case in the setting of pulmonary involvement is also unique to our case. (1,2,3). Our patient also had a favorable outcome with plasmapheresis and steroids alone. Treatment of DAH in the setting of MC is unclear due to the uncommon nature of the diagnosis. Steroids, Rituximab and plasmapheresis have been tried in varying combinations. Our patient declined therapy with Rituximab when presented with the possibility of relapse with HCV. CONCLUSIONS: Higher awareness of pulmonary manifestations of cryoglobulinemia is critical. Reference #1: Amital H, Rubinow A, Naparstek Y. Alveolar hemorrhage in Cryoglobulinemia-an indicator of poor prognosis. Clin Exp Rheumatol 2005 Sep-Oct;23(5):616-20 Reference #2: Ferri C1, Sebastiani M, Giuggioli et al. Mixed cryoglobulinemia: demographic, clinical and serologic features and survival in 231 patients. Semin Arthritir Rheum 2004 Jun;33(6):355-74 DISCLOSURE: The following authors have nothing to disclose: Abhijai Singh, Chandrakanta Chuturvedula, Gurbir GIll, Reshma Abraham, Pieusha Malhotra No Product/Research Disclosure Information DOI:
http://dx.doi.org/10.1016/j.chest.2016.08.544
Copyright ª 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.
530A
[
150#4S CHEST OCTOBER 2016
]
Diffuse Alveolar Hemorrhage (DAH) in Hepatitis C Virus (HCV) Associated Cryoglobulinemia After Completion of HCV Therapy Abhijai Singh MD Chandrakanta Chuturvedula MD Gurbir GIll MD* Reshma Abraham MD; and Pieusha Malhotra MD St Vincent Hospital, Shrewsbury, MA
DIFFUSE LUNG DISEASE
INTRODUCTION: DAH in the setting of cryoglobulinemia after HCV therapy completion is unreported in the absence of glomerular disease. CASE PRESENTATION: A 62-year-old lady with a past medical history of hypertension, coronary artery disease and hypothyroidism presented with new onset purpuric rash on bilateral lower extremities. . Review of systems was negative except for pruritus around the rash. The patient denied any new medication, sick contacts or tick exposure. On examination the patient was afebrile with normal vitals. Skin revealed palpable purpuric lesions with no significant necrosis. Laboratory workup is summarized in table 1. Skin biopsy came back positive for leukocytoclastic vasculitis. A diagnosis of type 2 mixed cryoglobulinemia (type 2 MC) was established. Patient was started on treatment for hepatitis C with Ledipasvir/Sofosbuvir combination and 12 weeks later patient achieved sustained viral response (SVR). 6 months later, she presented to the emergency department with acute hypoxemic respiratory failure. CT scan identified patch bilateral ground glass opacities. The patient was admitted to the intensive care on Bipap. She underwent an urgent bronchoscopy. Bronchoscopic alveolar lavage was positive for hemosiderin laden alveolar macrophages. A diagnosis of diffuse alveolar hemorrhage (DAH) was established. HCV RNA was documented negative during this admission. Cultures including special stains for pneumocystis were unrevealing. The patient was started on a combination of pulsed methylprednisolone and plasmapheresis and recovered after 5 sessions. DISCUSSION: DAH is a very rare phenomenon in the presence of Cryoglobulinemia with unclear prevalence. Hospital based studies have identified an incidence of 2-3 % (1,2). Disease activity usually corresponds to HCV RNA levels and virologic remission has been shown to cause cryoglobulinemia quiescence. DAH despite RNA clearance begs the question if HCV antibodies have the ability to propagate disease activity independent of SVR. Absence of glomerular disease, lack of Hypocomplementemia and negative rheumatoid factor are other unique features to our case in the setting of pulmonary involvement is also unique to our case. (1,2,3). Our patient also had a favorable outcome with plasmapheresis and steroids alone. Treatment of DAH in the setting of MC is unclear due to the uncommon nature of the diagnosis. Steroids, Rituximab and plasmapheresis have been tried in varying combinations. Our patient declined therapy with Rituximab when presented with the possibility of relapse with HCV. CONCLUSIONS: Higher awareness of pulmonary manifestations of cryoglobulinemia is critical. Reference #1: Amital H, Rubinow A, Naparstek Y. Alveolar hemorrhage in Cryoglobulinemia-an indicator of poor prognosis. Clin Exp Rheumatol 2005 Sep-Oct;23(5):616-20 Reference #2: Ferri C1, Sebastiani M, Giuggioli et al. Mixed cryoglobulinemia: demographic, clinical and serologic features and survival in 231 patients. Semin Arthritir Rheum 2004 Jun;33(6):355-74 DISCLOSURE: The following authors have nothing to disclose: Abhijai Singh, Chandrakanta Chuturvedula, Gurbir GIll, Reshma Abraham, Pieusha Malhotra No Product/Research Disclosure Information DOI:
http://dx.doi.org/10.1016/j.chest.2016.08.544
Copyright ª 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.
530A
[
150#4S CHEST OCTOBER 2016
]