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بسم الله الرحمن الرحيم. Total Body Necrosis. In late 2004, a 23 year old woman who complained of polyarthralgia, mouth ulcers, and alopecia, was admitted to another hospital where she was diagnosed SLE And class IV nehritis. (ANA) & (ds ANA) were positive.
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In late 2004, a 23 year old woman who complained of polyarthralgia, mouth ulcers, and alopecia, was admitted to another hospital where she was diagnosed SLE And class IV nehritis. (ANA) & (ds ANA) were positive. .
At that time renal function was normal, liver function was normal except that albumin was low (12gm/L)
Pt. was treated with methyl prednisolne (1g IV) for three days followed by oral prednisolone (60mg) in a tapering dose.
The total duration of treatment was unknown. Since 2004, the woman had frequent relapses of disease activity. • She was treated as described above with pulse steroid followed by a tapering Dose of prednisolone.
In march 2006, the woman was admitted to KAUH with dyspnea, lower – limb edema, decreased urine output, and polyarthragia.
On Clinical Examination • The following values were recorded: • Temperature (37.1oC) • Blood pressure (149/90mmHg) • Heart rate (84 beats/min) • elevated jugular venous pressure (JVP) • Body weight (90kg) and lower limb edema. The chest examination bilateral basal crepitation . The remainder of the exam was unremarkable.
LAB DATA • Creatinine 402 mmo/L • Po4 3.3 mmol/L • Calcium 2.4 mmol/L • Alkaline phospatase 36 I.U./L • Albumin 19 g • Repeat renal biopsy : diffuse proliferative GN with crescent (class lV)
She was treated with: • Pulse methyl prednisolone 1gm i.v.for 3 days • Then oral prednisolone 60 mg to be tapered after 1 month • Mycophenolate mofetil • H.D. • Phosphate binders
The Patient Responded • Began to produce urine in the range of 1.5-2 litter/day. The CaPO4 homeostasis was well- maintained and she came out of dialysis for 1/12 almost symptom free and with no pulmonary edema or lower – limb edema.
On 1st April 2008, the woman was discharged with creatinine (330mmol/L) and PO4 1.4mmo1/L to be assessed once each week. On 2 July 2006, she was admitted to KAUH with skin lesions that started at the glutei region. The lesions were diagnosed at the other hospital as glutei abscises.
The lesions were drained at the other hospital therefore, the precise characteristics of the lesions were not clear. On re-admission to our hospital, the lesions typically were violaceous, painful, plaque-like, and involved in the dermis and subcutaneous fat on back, buttocks, thighs, and breast. Subsequently the lesions progressed to ischemic/necrotic ulcers. The patient denied a history of fever or trauma.
On Clinical Examination • The following values were recoded body temperature (37oC) • Blood pressure (140/80mmHg) jugular venous pressure was not elevated and there was no lower limb edema.
Laboratory Tests • Showed creatinine 763mmol • Alkaine phosphatase 149IU/L • Calcium 1.66 mmo1/L • Phosphate 4.24mmo1/L • Calcium phosphate 7.0 mmo1/L • Parathyroid hormone 38 • ANA 1:320 g/L • dsDNA 52iu/ml C3 0.87g/l, C4 0.33g/L • CRP 130mg/L • Normal protein C, protein S,anticardiolipin and cryglobinemia.
Differential Diagnosis • Cholesterol Embolization. • Warfarin Necrosis. • Cryoglobulinemia • Vasulitis • Nephrogenic Systemic Fibrosis • Hyperoxaluria
Histology Examination • Revealed that dermis and epidermis show necrosis and fibrosis. Epidermis, subcutaneous fat and blood vessels with marked calcification
The patient was treated with daily dialysis • And oral prednisolone (0.5mt/1kg/1day And phospate binder • Pain killers (opiates) • Wound care • Antibiotics • Vitamin K+ Albumin
After all treatment patient transfer to ICU with septic shock. • Repeated PTH was 5.3, Ca 2.2mmol/l • PO4 1.4mmol/l • She patient died due to sepsis.
Calciphylaxis • Calciphylaxis is a small vessel vasculopathy involving mural calcification with intimal proliferation, fibrosis and thrombosis.
Risk Factors for the Developmennt Calciphylaxis • The role of Obesity. • Ca, Po4 and Ca x Po4 product. • The role of warfarin. • The role protein C and/or Protein S Deficiency. • Fetuin – A Glycoprotein and Matrix gla protein.
The role of protein malnutrition. • The role of PTH. • The role of Vitamin D Analogs.
The Diagnosis of Calciphylaxis • Physical Examination. • Exclusion of other Vascular Disease. • Tissue Biopsy. • Measurements of Transcutaneous oxygen saturation. • Bone Scans. • Xeroradiography.
An aggressive program of wound care and adequate pain control. • Avoidance of local tissue trauma, including subcutaneous injections. • Among dialysis patients, normalize serum PTH levels (intact PTH should be between 150 to 300 pg/ml) • Parathyroidectomy.
SUMMARY • Preventive Strategies • Reassess the Dialysis Prescription. • Improve serum calcium and phosphorus levels • Reassess the use of warfarin. • Consider Parathyroidectomy.
