Case No. 7 :Case No. 7


History: :History: 45 years old male Recurrent fever (38.5), productive cough, blood tinged sputum, epistaxis for >1year Anti-TB drugs given on no base for cavitary lung lesions (no AFB in sputum) for 6 months Painless bilateral ankle swelling 2 m duration, anorexia, weight loss


Slide 3:Recent change in nasal feature, smell and taste Normal ear Not a smoker No bleeding per other orifices


Examination: :Examination: Cachectic, pale BP =150/100 Pulse = 102 bpm Temp = 37.7 RR =18 bpm Saddle nose deformity, normal ear No epistaxis, no LNs, no inc. JVP


Slide 5:Coarse crepitations heard bilateral. Normal cardiac examination. Bilateral ankle swelling (no tenderness or joint swelling or warmth). Neurological examination is normal except for bilateral moderate conductive hearing loss and loss of smell.


Investigations: :Investigations: Blood: ESR =120 RBCs = normocytic normochromic anemia Creatinine = 2.4 mg/dl BUN = 18 mmol/l Albumin = 2.2 g/dl


Slide 7:Urine analysis: RBCs: 30-40 /HPF WBCs: 10-25 /HPF Large albumin No casts Culture: no growth 24-hr urine protein: 1.9 g/d


Slide 8:Sputum: Pseudomonas No AFB Abdominal U/S: Bilateral moderate hydronephrosis with normal sized kidneys.


Slide 9:CXR and CT chest:


Slide 10:What condition is responsible for this patient's numerous findings?Hint: Consider noninfectious etiologies that may affect a wide array of body systems. Tuberculosis Good pasture syndrome Systemic lupus erythematosus Wegener granulomatosis


Slide 11:What condition is responsible for this patient's numerous findings?Hint: Consider noninfectious etiologies that may affect a wide array of body systems. Tuberculosis Good pasture syndrome Systemic lupus erythematosus Wegener granulomatosis


Slide 12:C-ANCA and P-ANCA were done with C-ANCA +ve result. ANA was +ve (speckled pattern) Renal biopsy was done ? focal segmental sclerosis (some pts show crescentic GN) Nasal biopsy ? crusts, sub-mucosal fibrosis and thickened vessel walls


Slide 13:Lung biopsies ? showed extensive parenchymal necrosis, with cavitations admixed with fibrinoid material, excess neutrophils, and few eosinophils involving both lung parenchyma and bronchi


Treatment: :Treatment: Prednisolone 40-60 mg/d (for 6-9 m) Cyclophosphamide 2mg/Kg/d (for 6 m) Antibiotic according to sensitivity 3weeks later: pt improved, creat.= 1.6, anemia, urine improved, CXR improved. Discharged for FU after a month,


Case No 8 :Case No 8


History: :History: 36 years old male, heavy smoker. Urticaria, chronic cough, allergic rhinitis, sinusitis, asthma for many years. Presented with cramping abdominal pain, bloating, dark watery diarrhea of 1 wk duration. Fever generalized myalgias and arthralgias.


Examination: :Examination: Temp = 38.8 BP = 150/90 Pulse = 110 bpm RR = 20 bpm SO2 = 89% on RA LL edema, diffuse maculo-papular rash on both legs Bilateral basilar crepitations heard


Investigations: :Investigations: WBCs: 30,000 with high eosinophils Thrombocytosis: 870,000 Hb = 9.7 mg/l Normal renal and liver functions ESR >120 Urine: numerous RBCs, no casts, high P/Creat (protinuria) Serum IgE is high


Slide 19:CT abdomen with contrast: LNs with thickened distal duodenum & jejunum, normal kidneys. CXR, CT: bilateral patchy pul. infiltrates and cardiomegaly. Blood culture: staph epidermidis Urine culture: contaminants


D.D: :D.D:


Slide 21:ANA, complement, RF, hepatitis markers, HIV are all –ve but ANCA +ve GI endoscopy: ulcerated duodeno-jejunal mass ? biopsy ? inflammation Skin biopsy ? vesicular dermatitis with lymph., eosinophils, neutrophils (unusual) Renal biopsy ? focal necrotizing GN, no immune-complexes Bronchoscopy ? alveolar Hge


Slide 22:The most possible diagnosis is a pauci-immune vasculitis ? Churg-Stauss syndrome


Treatment: :Treatment: Steroids +/- Cyclophosphamide are given to induce remission, maintain it and prevent relapse (evaluated by clinical picture, eosinophilia, ESR). Plasmapharesis and Ig ttt are also beneficial.


Thank you :Thank you