RAPIDLY FAILING KIDNEYS Dr Paul Johny 2 nd yr DNB Medicine Resident
Mr Z 67yrs old Occupation : Retired officer from electricity board
Chief complaints : Fever : 5 days Right lower limb swelling and pain :5days Left ear pain and discharge : 3 days
Past History Being treated as Airborne contact dermatitis since 8 years on topical emolients and topical steroids No H/o Diabetes / Systemic Hypertension / CAD / Pulmonary Tuberculosis /Bronchial Asthma
General Examination : Dry scaly patches over exposed parts of the body, swelling over right lower limb extending above right knee joint with eroded patches over the legs. Tenderness and local rise of temperature present over right leg. Vitals Pulse :116/mt BP :110/70mmhg Spo2 : 97% room air
SYSTEM EXAMINATION FINDINGS RESPIRATORY SYSTEM Normal CARDIOVASCULAR SYSTEM Normal GASTROINTESTINAL SYSTEM Normal CENTRAL NERVOUS SYSTEM Normal EAR EXAMINATION (LEFT) Purulent Discharge + Central Perforation of TM +
CLINICAL DIAGNOSIS? RIGHT LOWER LIMB CELLULITIS ASOM (LEFT EAR)? AIRBORNE CONTACT DERMATITIS
INVESTIGATIONS INVESTIGATION Hb PLT VALUES 9.3 g/dl 153000/µL INVESTIGATION Creat BUN VALUES 3.8mg/dl 51mg/dl TC PCT 16000 /µl 45 mg/l Potassium Calcium 4.0meq/dl 9.0mg/dl CRP ESR 157mg/l 70mm/hr Uric acid 11.4mg/dl Note : Baseline creatinine (0.8mg/dl)
LIVER FUNCTION TESTS URINE ROUTINE INVESTIGATION VALUES INVESTIGATION VALUES Total protein Albumin Globulin ALP 9.1gm/dl 2.6gm/dl 6.5gm/dl 60 IU/L PROTEIN 2+ PUS CELLS 10-12/hpf RBC 8-10/hpf
Chest X-ray
Possibilities??? Sepsis (multiple sources)?multiple myeloma Anaemia Acute kidney injury
Blood culture - Streptococcus pyogenes Urine culture - Proteus Wound swab culture - Pseudomonas,Proteus
ANAEMIA EVALUATION Peripheral Smear suggestive of normocytic normochromic anaemia. Iron profile suggestive of anaemia of chronic disease Vitamin B12 was low
ACUTE KIDNEY INJURY EVALUATION USG abdomen and KUB Bilateral grade 1 renal Parenchymal Changes Bilateral simple renal cortical cyts. Urine protein creat ratio :1.64
MULTIPLE MYELOMA WORK UP Serum Protein Electrophoresis Hypoalbuminemia with elevation in alpha 1 globulin fraction and beta gamma fusion suggestive of inflammatory/cirrhotic pattern
Inspite of appropriate antibiotics as per culture reports and hydration Creatinine worsened Rapidly Progressing Renal Failure
RAPIDLY PROGRESSING RENAL FAILURE??CAUSE?NON RESOLVING SEPSIS?VASCULITIS
Vasculitis Workup ANA - Negative C-ANCA (IF) - 2+ c3,c4 - normal
Renal Biopsy
Fever spikes persisted even with higher antibiotics and infective parameters (CRP,Procalcitonin,TC) under control. Fever spikes were considered likely due to vasculitis,steroids were added and patient got better,creatinine improved to 0.9.
TREATMENT 250 mg Methyl prednisolone once daily for 3 days Discharged with oral steroids Follow up,started on Methotrexate Presently patient is on low dose steroids and methotrexate and is in remission with no symptoms and normal creatinine.
FINAL DIAGNOSIS C ANCA POSITIVE VASCULITIS SEPSIS (MULTIPLE SOURCE) CELLULITIS RIGHT LEG UTI ASOM ANAEMIA OF CHRONIC DISEASE?AIRBORNE CONTACT DERMATITIS
Why this case... Age of presentation Sepsis and Vasculitis Unexplained fever?
ANCA Anti neutrophil cytoplasmic antibodies C-ANCA ( Cytoplasmic staining pattern) Antibodies to PR -3 What is PR- 3? PR3 is a neutral serine protease that is localized in the cytoplasmic granules of neutrophils and the lysosomes of monocytes. Binding of ANCA to PR3 and Fcγ-RII receptors expressed on the cell surface of previously primed neutrophils causes degranulation and oxidative burst of neutrophils.
Used as a screening test Indirect immunofluoroscence - more sensitive ELISA - more specific Test for ANCA in patients Chronic destructive upper airway disease, pulmonary nodules Rapidly progressive glomerulonephritis Skin vasculitis with systemic illness Mononeuritis multiplex Subglottic stenosis of the trachea and retro-orbital mass. ANCA titres are not useful to guide treatment /prognosis.
False positive ANCA Inflammatory Bowel Disease Sjogren s Syndrome Cystic fibrosis HIV /HCV infection Infective Endocarditis Idiopathic Pulmonary Fibrosis Multiple Sclerosis Behcet s Disease
TAKE HOME MESSAGE Consider antineutrophil cytoplasmic antibody (ANCA) associated vasculitis when inflammatory disease cannot be ascribed to any other disease and inflammation progresses despite antibiotics. Urine routine gives a major clue in our diagnosis and guidance in treatment. Avoid diagnostic delay to prevent end organ damage, particularly renal disease. Remission is induced by high dose glucocorticoids and either cyclophosphamide/ rituximab Patients should be counselled about the disease and need for long term followup to prevent relapse and treatment complications.