A 25-year-old male presented with blood stained expectoration of about 300 ml/day since 2 days and chest pain since one week with no similar complaints in past. He had history of shortness of breath and wheeze during childhood with no significant past and family history. On examination patient had tachypnea & tachycardia and saturation (SPO2) of 96% on room air. On auscultation left upper lobe crackles were heard. Laboratory findings were Haemoglobin 13.6 gm/dl, total leucocyte count of 15.500 cells mm3, ESR 44 mm/hr, Serum IgE 800IU/ml, sputum AFB stain and gene expert were negative. Liver function test, urine routine, renal function tests were within normal limits. Chest radiograph PA view (Fig. 1) revealed non homogenous opacity left upper and mid-zone. CECT chest (Fig. 2A and B) was suggestive of heterogeneously enhancing lesion measuring 6.4 x 6.8 x 5.6cm with internal areas of necrosis and surrounding ground glass attenuation in apico-posterior segment of left upper lobe with narrowing and partial occlusion of left apicoposterior segmental bronchus with few enlarged intrapulmonary lymphnodes. Videobroncoscope showed soft tissue endoluminal lesion completely occluding left upper lobe segments which bleeds on biopsy (Fig. 3). Bronchoscopic lavage, brushings and biopsy were done from left upper lobe lesion. Subsequently CT guided percutaneous biopsy was also performed from left upper lobe lesion.
Pending the reports patient was discharged. He got re-admitted after 5 days with persistent haemoptysis of about 100-200 ml, worsening of breathlessness and hypoxia with saturation of 88% on room air. His chest radiograph showed significant worsening of left upper lobe lesion. Patient was started on oxygen through nasal canula @ 2 L per minute, haemostatic agents & parenteral antibiotics.
Bronchial lavage was negative for AFB stain by Ziehl-Neelsen stain and on Gene Xpert, Mycobacterium TB was not detected. Bronchial lavage was also negative for malignant cells on cytological analysis with no growth on aerobic culture. On histopathology (Fig. 4A and B) endobronchial biopsy was suggestive of capillaritis with leucocytoclasis and infiltration by both acute and chronic inflammatory cells and occasional multinucleated giant cells. Histopathology of both bronchoscopic and CT guided biopsy showed granulomatous inflammation with multinucleated giant cells and microscopic haemorrhage suggestive of vasculitis. Further laboratory findings revealed a strongly positive value by enzyme-linked immunosorbent for antibodies anti PR3 autoantibodies (9.9IU/ml) and negative for MPO (<0.2IU/Ml).
A diagnosis of Granulomatosis with polyangiitis was made on basis of clinico-histopathological and serological evidence. Our patient was classified as having limited disease on basis of fulfilment of modified American College of Rheumatology criteria for the classification of GPA. Following the diagnosis, patient was started on intravenous methylprednisolone 1000mg in 250ml NS over 2 hours 3 days. Rituximab was given intravenously as four doses of 375 mg/m2 at weekly intervals (plus prednisolone 100mg on the day of infusion). Chest x-ray (Fig. 5) after one week of therapy showed significant clearance of left upper zone lesion along with clinical improvement with amelioration of breathlessness and haemoptysis.
Chest CT showed heterogeneously enhancing lesion measuring 6.4 x 6.8 x 5.6cm with internal areas of necrosis and surrounding ground glass attenuation in apico-posterior segment of left upper lobe with partial occlusion of left apicoposterior segmental bronchus.