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Disclosures. None. Case 1. What is going on with me?. Case 1. 74 year old male presents to his primary care physician in May complaining of shortness of breath. There was also evidence of hematuria.

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  1. Disclosures None

  2. Case 1 What is going on with me?

  3. Case 1 • 74 year old male presents to his primary care physician in May complaining of shortness of breath. There was also evidence of hematuria. • A few days later on May 6, the patient presents to the emergency department due to spontaneous epistaxis. A CXR at that visit reveals a pleural effusion. • On May 9, a CT scan of the chest confirms the pleural effusion. • On May 14, the patient undergoes a renal ultrasound that was unremarkable due to the hematuria. The patient also undergoes a right sided thoracentesis by IR with over 1400 mL fluid removed

  4. Case 1

  5. Case 1 • Pleural fluid analysis: • pH = 7.37 • Glucose = 87 • Protein = 4.4 • LDH = 530 • Cell count: • RBC = 444 • WBC = 2534 • 87% neutrophils • 13% lymphocytes • Gram stain negative • Cultures negative • Fungal cultures negative • Cytology negative

  6. Case 1 • Laboratory work-up: • Urine IEP negative for Bence-Jones proteins • Serum IEP negative for monoclonal proteins • UPEP negative • SPEP negative • C4 = 12 • C3 = 73 • Urine random protein = 91 • Urine random creatinine = 93 • ANCA negative • ANA positive

  7. Case 1 • Two weeks after the original thoracentesis performed, patient began to develop hemoptysis and a repeat chest xray was performed which showed reaccumulation of the pleural fluid on the right. Prednisone was then started.

  8. Case 1 • June 3, patient underwent a cystoscopy due to his hematuria. The cystoscopy was normal. • On June 10, patient presented back to the emergency department complaining of increased dyspnea along with continued hemoptysis despite the steroids. Now, patient was also found to be hypoxic. He was therefore admitted to the Internal Medicine service.

  9. Case 1 • In the ED patient’s hemoglobin was down to 10.8. • Creat was 1.9 • UA was positive for both blood and protein • CT scan of the chest was performed.

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  12. Case 1 • During patient’s hospitalization in June: • Bronchoscopy was performed which showed blood in the bronchial tree but no lesions seen. • A 24 hour urine was performed which showed 673 mg of protein. • Proteinase 3 was negative. • Renal Biopsy was also done and showed a focal-segmental proliferative GN. • Patient discharged on a cocktail of medicines: • Prednisone 60 mg daily • Cellcept (mycophenolate) 250 mg 4 tabs twice a day • Mepron 1500 mL daily

  13. Case 1 • About 2-3 weeks after discharge, patient’s cellcept was stopped and patient was transitioned to IV cytoxan based on final results of EM report from the renal biopsy.

  14. Case 1 • Exactly one month after discharge, on July 17, patient presented back to the hospital complaining of two-three days of cough, pleuritic chest pain, fevers and chills. • Chest xray in the ED showed a LEFT lower lobe infiltrate and left sided effusion - with no evidence of a right sided effusion. • Patient’s hemoptysis had almost completely resolved • Patient admitted for pneumonia and started on triple antibiotic coverage.

  15. Case 1 • On day #2 of patient’s hospitalization, we performed a thoracentesis on the left sided pleural effusion. • July 18 left thoracentesis results: • pH= 7.28 • Glucose = 255 • Protein = 3.0 • LDH = 1350 • Cell count: • WBC = 11,537 • 99% are neutrophils

  16. Case 1 • On July 19 the sputum culture obtained at admission reported the growth of “mold”. • Infectious disease was consulted and Vfend empirically started. • Fungitell assay was ordered and was markedly positive at >500. (normal is <80) • Eventually the pleural fluid culture also began to grow “mold”

  17. Case 1 • Patient ultimately required a repeat left sided thoracentesis two more times during his hospital stay in July. The second thoracentesis netted 900 mL of amber colored fluid and the third thoracentesis yielded 500 mL of fluid.

  18. Case 1 • Oh by the way……….. • HIV was negative. Testing done due to the immunosuppressed nature of the patient. • But……………….. • CMV was positive with CMV DNA positive and and CMV PCR with 54,980 copies!

  19. Case 1 • Patient eventually discharged home on July 31 • Prednisone 60 mg daily • Vfend 200 mg every 12 hours orally x 4 months minimum • Valganciclovir 450 mg twice a day for 3 weeks • Mepron 1500 mg a day

  20. Case 1 • Any guess as to the final mold culture results?

  21. Case 1 Name this organism

  22. Case 1

  23. Case 1 - Summary • May • SOB, epistaxis, and effusion seen on CXR • Work-up started including right-sided thoracentesis with exudative effusion and positive ANA • Hemoptysis begins and prednisone started. • June • Admission for hemoptysis • Renal biopsy showing GN • Home with immunosuppressive medications • July • Patient readmitted with left pleural effusion and cultures begin to grow mold. • Evidence of a CMV viremia

  24. Case 1 - Diagnosis • 74 yo male with recurrent exudative pleural effusions, hemoptysis , and renal disease with a positive ANA. • What the heck is wrong with me??

  25. Case 1 - Diagnosis • 74 yo male with recurrent exudative pleural effusions, hemoptysis , and renal disease with a positive ANA. • Systemic Lupus Erythematosus with lupus nephritis and SLE induced Diffuse Alveolar Hemorrhage • Aspergillusfumigatusinfection due to immunocompromised status along with CMV viremia.

  26. Case 1Pleural effusion • Transudative versus Exudative Pleural effusion: • Light’s criteria: • Pleural fluid to serum protein ratio >0.5 • Pleural fluid to serum LDH ratio >0.6 • Pleural fluid LDH >200 (or >2/3 upper limit of normal) • Only need one criteria to make it an exudate

  27. Case 1 Pleural effusion • Transudative pleural effusion differential diagnosis: • CHF • Cirrhosis • Nephrotic syndrome • Urothorax • Superior vena cava obstruction • Myxedema • Iatrogenic from peritoneal dialysis or a misplaced central line

  28. Case 1Pleural effusion • Exudative Pleural effusion differential diagnosis: • Parapneumonic • Malignant • TB • PE • Collagen-vascular disease • RA • Lupus • Wegener’s granulomatosis • Churg-Strauss • Pancreatitis, Boerhaave’s effusion, subphrenicabcess • Hemothorax, chylothorax • Drug reaction • Dressler’s syndrome and Meig’s syndrome

  29. Case 1Pleural effusion • Pleural effusion due to Lupus: • Pleura involved in SLE more than any other collagen-vascular disease • Effusions are in seen in 16-44% of SLE patients • Half of the effusions in SLE are bilateral • Predominance of PMNs • Glucose typically > 80 • (Rheumatoid effusion glucose is usually <25) • LDH typically < 500 • pH typically > 7.20

  30. Case 1Diffuse Alveolar Hemorrhage • DAH is an acute, sometimes life threatening event • Most patients present with dyspnea, cough, hemoptysis and new alveolar infiltrates. • A number of different causes can cause diffuse alveolar hemorrhage. • No prospective studies however have identified the most common cause.

  31. Case 1Diffuse Alveolar Hemorrhage • 3 characteristic patterns of DAH • Associated with vasculitis • Bland pulmonary hemorrhage • DAH associated with another process or condition • Cleveland Clinic Journal of Medicine April 2008 vol 74 4 258-280

  32. Case 1Diffuse Alveolar Hemorrhage • DAH related to vasculitis pathophysiology: • ‘Neutrophils infiltrate the interalveolar and peri-bronchiolar septal vessels (pulmonary interstitium), leading to anatomic disruption of the capillaries (ie, impairment of the alveolocapillary barrier) and to extravasation of red blood cells into the alveoli and interstitium.’ • ‘Neutrophil apoptosis and fragmentation, with subsequent release of the intracellular proteolytic enzymes and reactive oxygen species, beget more inflammation, intra-alveolar neutrophilic nuclear dust, fibrin and inflammatory exudate, and fibrinoid necrosis of the interstitium.’ • Cleveland Clinic Journal of Medicine April 2008 vol 74 4 258-280

  33. Case 1Diffuse Alveolar Hemorrhage • Systemic lupus erythematosus and DAH: • Up to 11% of patients have diffuse alveolar hemorrhage at onset of SLE diagnosis (DAH is more commonly associated with SLE than any other connective tissue disorder) • Fever, arthralgia, rash • ANA positivity • Anti-dsDNA antibodies • Decreased C3 and C4 • Cleveland Clinic Journal of Medicine April 2008 vol 74 4 258-280

  34. Case 1Diffuse Alveolar Hemorrhage • DAH diagnosis: • Bronchoscopy: • “blood in the lavage specimens (with numerous erythrocytes and siderophages) establishes the diagnosis.” • Cleveland Clinic Journal of Medicine April 2008 vol 74 4 258-280

  35. Case 1Diffuse Alveolar Hemorrhage • DAH Treatment: • Immunosuppressive agents are the mainstay of therapy for diffuse alveolar hemorrhage. • Most experts recommend intravenous methylprednisolone (Solu-Medrol) (up to 500 mg every 6 hours, although lower doses seem to have similar efficacy) for 4 or 5 days, followed by a gradual taper to maintenance doses of oral steroids. • In patients with pulmonary-renal syndrome, therapy should be started as soon as possible to prevent irreversible renal failure. • Besides corticosteroids, other immunosuppressive drugs such as cyclophosphamide (Cytoxan), azathioprine (Imuran), mycophenolatemofetil (CellCept) can be used. • Cleveland Clinic Journal of Medicine April 2008 vol 74 4 258-280

  36. Case 1Lupus Nephritis • Lupus nephritis is more common in women (SLE 9:1 women) and also more common among African Americans and Hispanics • The symptoms of lupus nephritis are generally related to hypertension, proteinuria, and renal failure • Most patients with Lupus will have evidence of clinical nephritis (50%) during the course of their disease, and usually within 5 years of the diagnosis. • Lupus nephritis is histologically present on most biopsies, even if the patient does not have clinical manifestations of renal disease.

  37. Case 1Lupus Nephritis • Evaluation of lupus nephritis • Laboratory evaluation should include serum creatinine, serum abumin levels, UA, ESR, 24 hour urine for protein. • Proteinuria is the most commonly seen abnormality in Lupus patients • Elevated anti-dsDNA and depressed C3 and C4 levels are associated with active nephritis. • Clinically relevant lupus nephritis is associated with a 30% decrease in creatinine clearance, proteinuria of greater than 1000 mg/d, and renal biopsy findings indicating active lupus nephritis.

  38. Case 1Lupus Nephritis • Biopsy is critical in most patients with Lupus and evidence of renal involvement • Our patient had evidence of focal segmental proliferative GN • This is consistent with Stage III renal involvement and that means patient would benefit from immunosuppresants. • Treatment: • Steroids • Mycophenolate (cellcept) • cytoxan

  39. Case 1Aspergillosus • This is an important cause of very dangerous infections, especially in immunocompromised patients. • Prolonged neutropenic patients • HIV • Inherited immunodeficiency • Allogenic hematopoietic stem cell transplant • Lung transplant • Aspergillusfumigatus is the most common species recovered • Culture if able • Galactomannan antigen • Fungitel assay to detect 1-3-B-D-glucans

  40. Case 1Aspergillosus • Voriconazole and amphotericin B are the only two antifungals indicated for primary treatment of invasive aspergillosis • Voriconazole • Oral or IV triazole compound • Hepatically metabolized • Side effects: visual disturbances (photopsia), skin rash, hepatotoxicity, and visual hallucinations

  41. Questions?And no, I have no idea why my kids can’t take a normal picture.

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