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Bloody Lungs and Broken Kidneys Banded in One Blight
Description
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A3005 - Bloody Lungs and Broken Kidneys Banded in One Blight
Author Block: C. Vahdatpour1, O. Amoran2; 1Internal Medicine, Pennsylvania Hospital, Philadelphia, PA, United States, 2Internal Medicine, Pennsylvania Hospital, philadelphia, PA, United States.
Introduction
Microscopic polyangiitis is a rare form of p-ANCA positive small vessel vasculitis. It is characterized by a variable clinical presentation, most often including pulmonary hemorrhage and/or glomerulonephritis. End-organ damage could be deterred by prompt recognition and treatment.
Methods
Case Presentation
Results
We present a case of a 74-year-old African American female with medical history significant of chronic obstructive pulmonary disease (COPD), smoker (40 pack years), chronic kidney disease stage 3A (CKD), osteoarthritis, recurrent renal calculi, gastro esophageal reflux disease (GERD); who presented with history of progressive diarrhea, weakness, hemoptysis, and left knee pain. Bilateral bibasilar end-inspiratory rales noted on auscultation of the lungs. Laboratory and imaging studies revealed hyponatremia, worsening renal function, elevated d-dimer and low pulmonary emboli probability on a ventilation-perfusion scan. Further work-up depicted a positive P-ANCA/myeloperoxidase antibody suggesting a pulmonary-renal syndrome. Renal biopsy was not recommended due to a right atrophic kidney on ultrasound. She was diagnosed with microscopic polyangiitis complicated by rapidly progressive glomerulonephritis. She was then treated with corticosteroids (IV pulse and oral maintenance which was eventually tapered off) along several months of rituximab. Subsequent outpatient follow-up showed an improvement in her pulmonary symptoms and renal function.
Symptoms associated with microscopic microangiitis are often non-specific mimic symptoms associated with other diseases. As in our case, patient had a background history of COPD and smoking history, she was worked up in another institution due to her complaint of hemoptysis. CT thorax and bronchoscopy were nonspecific. Furthermore, she had multiple urology procedures done due to recurrent renal stones complicated by CKD. High index of suspicion is needed to make an early diagnosis and initiate treatment.
Conclusion
Although multiple disease process may mimic microscopic polyangiitis, it is important to exclude other potential disease process and initiate treatment to restrict disease progression. Often health care providers will treat individual symptoms, associating them as independent comorbidities. This often delays treatment and increased the burden on both the health of the patient and health care costs. A quickly formed multidisciplinary approach involving pulmonology, nephrology, and rheumatology is generally necessary to reduce diagnostic delay.
Author Block: C. Vahdatpour1, O. Amoran2; 1Internal Medicine, Pennsylvania Hospital, Philadelphia, PA, United States, 2Internal Medicine, Pennsylvania Hospital, philadelphia, PA, United States.
Introduction
Microscopic polyangiitis is a rare form of p-ANCA positive small vessel vasculitis. It is characterized by a variable clinical presentation, most often including pulmonary hemorrhage and/or glomerulonephritis. End-organ damage could be deterred by prompt recognition and treatment.
Methods
Case Presentation
Results
We present a case of a 74-year-old African American female with medical history significant of chronic obstructive pulmonary disease (COPD), smoker (40 pack years), chronic kidney disease stage 3A (CKD), osteoarthritis, recurrent renal calculi, gastro esophageal reflux disease (GERD); who presented with history of progressive diarrhea, weakness, hemoptysis, and left knee pain. Bilateral bibasilar end-inspiratory rales noted on auscultation of the lungs. Laboratory and imaging studies revealed hyponatremia, worsening renal function, elevated d-dimer and low pulmonary emboli probability on a ventilation-perfusion scan. Further work-up depicted a positive P-ANCA/myeloperoxidase antibody suggesting a pulmonary-renal syndrome. Renal biopsy was not recommended due to a right atrophic kidney on ultrasound. She was diagnosed with microscopic polyangiitis complicated by rapidly progressive glomerulonephritis. She was then treated with corticosteroids (IV pulse and oral maintenance which was eventually tapered off) along several months of rituximab. Subsequent outpatient follow-up showed an improvement in her pulmonary symptoms and renal function.
Symptoms associated with microscopic microangiitis are often non-specific mimic symptoms associated with other diseases. As in our case, patient had a background history of COPD and smoking history, she was worked up in another institution due to her complaint of hemoptysis. CT thorax and bronchoscopy were nonspecific. Furthermore, she had multiple urology procedures done due to recurrent renal stones complicated by CKD. High index of suspicion is needed to make an early diagnosis and initiate treatment.
Conclusion
Although multiple disease process may mimic microscopic polyangiitis, it is important to exclude other potential disease process and initiate treatment to restrict disease progression. Often health care providers will treat individual symptoms, associating them as independent comorbidities. This often delays treatment and increased the burden on both the health of the patient and health care costs. A quickly formed multidisciplinary approach involving pulmonology, nephrology, and rheumatology is generally necessary to reduce diagnostic delay.
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