To the Editor: We discuss a 13-y-old girl with dry cough and fever (1 y); and abdominal and chest pain (2 mo). She was diagnosed as pleural effusion clinico-radiologically. After 2 wk she presented with swelling around the eyes, abdominal pain and fast breathing (4 d). On examination she was afebrile, HR-78/min, RR-32/min, BP-100/60 mmHg, pallor, pedal edema and reddish necrotic crusted lesions (pinna, heel and hands). Air entry was reduced (left side) with unremarkable other systemic examination. Past history revealed swelling and pain in multiple large/small joints with restriction of movement (1 y) and episodic ulcerations over skin (6 mo). In view persistent multisystemic involvement a differential diagnosis of disseminated tuberculosis, MIS-C, connective tissue disorder and vasculitis was considered. Investigation revealed anemia (Hb: 6.4 g/dl, microcytic/ hypochromic), raised CRP (21 mg/dl), blood urea (71 mg/dl), S. creatinine (1.3 mg/dl) and high COVID antibody (2678 BAU/ml). Urine examination showed hematuria (dysmorphic RBC), nephrotic range proteinuria (Up: Uc ratio: 7.6). During hospitalization, she developed intestinal obstructions, renal failure; hypertension, seizure and hemiplegia with aphasia. Non contrast CT head showed calcified granuloma and ill -defined hypodensities (fronto-parietal). Chest and abdominal tomography showed ground glass opacities, thrombosis (right LL segmental pulmonary artery), pulmonary infarct, mild ascites, hepatomegaly and thickened distal ileum. CT pulmonary angiogram confirmed pulmonary thrombus. Bronchoscopy revealed pulmonary hemorrhage and serum was positive for perinuclear anti-cytoplasmic antibody (40 IU/ml). Renal biopsy concluded anti-glomerular basement membrane disease (anti GBM disease) though serum anti GBM antibody were negative.

We conclude that dual positive antineutrophilic cytoplasmic antibody (ANCA) and Goodpasture’s syndrome is rarely reported with skin and neurological involvement [1,2,3]. The strength of our report is strong diagnosis (use of indirect immunofluorescence assay, biopsy) but is limited by unavailability of other methods for detection of circulating antibodies [4]. We conclude prompt recognition and early referral in children with pulmonary renal syndromes with atypical features.