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REBIN TITUSClinical Case ConferencePresenting history 71-year-old Caucasian female with past medical history notable for hypertension and arthritis, transferred from Roswell secondary to acute renal failure She was of her usual state of health up until 4 months ago, when she developed swelling of her hands and feet. She reports that she was gardening one day 4 months ago and the next day she woke up with joint pain, swelling of the bilateral upper and lower extremities especially the hands, wrist and ankles.Presenting history Since then, she has been admitted to the hospital numerous times for the same complaints Patient still complains of pain and swelling in multiple joints on day of presentation and states that her swelling waxes and wanes Since April, she has had an extensive workup, and was being followed by an internist, nephrologist and rheumatologist. She also reports some difficulty breathing, along with difficulty swallowing. Past Medical History Hypertension. Polyarthritis. Diverticulosis with history of diverticulitisOver past 4 months: Acute renal failure. Anemia of unknown etiology. Dysphagia. Dyspnea Worsening hypertension.Past Surgical History Bilateral knee replacements. Right shoulder surgery. Colon resection. History (contd.) Social History: Patient smoked pack per day for 20+ years, quit 4 years ago, has not started back. Patient is a social drinker, states she drinks 3-4 alcoholic beverages a week. No use of illicit drugs. Lives in Roswell Family History: Positive for lymphoma in father. Medications Alendronate Amlodipine Epoetin Metoprolol Morphine Omeprazole Zofran Prednisone 15 mg Ambien. PE Vitals: Temp: 95.6; BP 162/90; HR 83, RR 16; O2 sat 93% on room air. Gen appearance: Comfortable, in no distress HEENT: PERRLA, normal conjunctivae, moist MM, eyes Neck: Supple, no lymphadenopathy Lungs: CTAB CVS: S1, S2, RRR, no M/R/G Abd: Soft, BS +, NT/ND Ext: No C/C trace edema around ankles Mildly edematous joints of both hands with ulnar deviation, slighlty tender with taut skinPE (contd.Labs (8/2) CBC : WBC 12.5 , H/H 10.3 and 30.8, platelets 126K. BMP: Sodium 136, potassium 4.3, chloride 104, C02 21, glucose 95, BUN 79, creatinine 4.3, calcium 8.3. LFT: ALT 28, AST 31. Total bilirubin 0.9. Iron studies: Iron level 53. TIBC 233. Transferrin 23. Ferritin level 1,727. TSH 2.87. Free T4 1.29. Spot Urine with protein 123, creat 47. UA positive for moderate blood, sp gravity 1.009Other labs from Roswell ASO negative. RF negative. ANA negative. Ds-DNA pending. RNP pending. Scleroderma antibody negative along with reports from the nephrologist of p-ANCA and c-ANCA negative. Creatinine on 6-15-09 at 0.8 with subsequent rise approximately 2.5 to 3.0 without return to baseline. Admission day # 2,3,4 Doing well, good urine output, no complaints Continued on home meds Creatinine continuing to trend upwards: 4.5, 4.6, 4.9, BUN in the 70-80s BP also trending upAdmission day # 5 Continues to do well Producing good urine output BP uncontrolled, systolics in the 180-200s Creatinine now up to 5.5, BUN 95, GFR 10 Biopsy scheduledAdmission day # 6, 7 Biopsy results suspicious for thrombotic microangiopathy, suspicion for scleroderma Started on low dose captopril Low dose steroids stopped Creatinine continues to rise, rapidly progressing, now up to 6.7, BUN 116, GFR 8 BP continues to be uncontrolled Pt now is tired and fatigued, urine output decreasedBiopsy Thrombotic microangiopathy The histologic appearance is consistent either with malignant hypertension, scleroderma renal crisis, hemolytic uremic syndrome or cancer chemotherapy Moderate increase of mesangial matrix. The interlobular arteries show moderate narrowing. Minimal lymphocytic infiltration is seen in the interstitial tissue. Less than 10% of renal parenchyma is lost by tubular loss and atrophy and interstitial fibrosis. IgA, IgG, IgM, C1q, C3, C4 and albumin are negative. No immunoglobulins, complements, albumin or fibrinogen seen along the tubular basement membrane or blood vessel wall.Marked thickening of vessel wall with narrowed lumenThrombi in capillary loops and arterioleThrombi in capillary loops and arterioleLoss of parenchyma by interstitial fibrosis and tubular atrophyAdmission day # 7,8 Increasing doses of captopril, upto 50 mg tid BP some better 150-190s systolics Now being treated as a scleroderma renal crisis Creatinine continues to rise 7.4, 8.2; BUN 115, 117, Admission day # 9,10 Creatinine 9, BUN 117, K 5.4, with some altered mentation, also some nausea, low appetite Decision made to dialyze secondary to uremic symptoms Tunnel catheter placed BP now under good ctrlAdmission day # 11,12 Patient tolerated dialysis well, improved Creatinine down to 5.9, BUN 60, other lytes normal BP well controlled on captopril Induction for dialysisAdmission day #13, 14, 15 Continues hemodialysis daily Creatinine down to 5.1, 5.5 Tunnel catheter placed Set up for routine hemodialysis in Roswell Discharged homeScleroderma renal crisis Abrupt onset of moderate to severe hypertension Urine sediment that is normal or reveals only mild proteinuria with few cells or casts Progressive renal failure Severe and life-threatening Scleroderma renal crisis Can develop in approximately 10 to 20 percent of patients with the diffuse cutaneous form of systemic sclerosis and much less frequently in limited cutaneous systemic sclerosis. Despite the widespread use of ACE inhibitors for the treatment of scleroderma renal crisis, morbidity and mortality remain high.Prevalence Approximately one-half of scleroderma patients show some evidence of renal involvement, such as proteinuria, a mild elevation in the creatinine concentration, and/or hypertension Scleroderma renal crisis (SRC) develops in up to 20 percent of patients with diffuse cutaneous systemic sclerosis, although its incidence appears to be declining Risk factors Diffuse skin involvement Glucocorticoid use Presence of certain autoantibodies like anti-RNA polymerase antibodies .Clinical Features Occurs within the first five years of the onset of the disease. In one series, renal crisis occurred at a median duration of 7.5 months from the onset of the disease. In some cases, SRC is the initial manifestation of systemic sclerosis.Clinical features Acute renal failure, usually in the absence of previous kidney disease. Abrupt onset of moderate to marked hypertension, often accompanied by manifestations of malignant hypertension, such as hypertensiv