A Rare Case of Anti-GBM Negative Goodpasture Disease Presenting with Dilated Cardiomyopathy and Perforating Dermatoses.

Goodpasture Syndrome with a rare incidence of one case per million accounts for 20% of RPGN cases. It occurs due to antibodies against the NC1 domain of the alpha3 chain of type IV collagen. It usually presents with glomerulonephritis & pulmonary hemorrhage. But 35% may present with glomerulonephritis alone. Circulating anti-GBM antibodies may not be present always. Herein we present a 24-year-old female from our IPGMER & SSKM Hospital, Kolkata with complaints of 30 days fever, 25 days of erythematous Skin lesions, 7 days of cola colored frothy urine, 3 days of anasarca & Oliguria, 3 days of shortness of breath & cough. Chest-X-ray revealed Pulmonary vascular congestion. Yet no history of pulmonary hemorrhage. ABG revealed Type 1 respiratory failure. ST-T changes in ECG & Dilated left Atrium and ventricle with severe Global Hypokinesia & EF 44% in Echocardiography. Very high Troponin-I (247.62). Plenty of Dysmorphic RBC & RBC Cast in Urine study. Urea 62 Creatinine 2.6 & 2.8gm protein per 24-hour urine. Evidence of Cardiomyopathy & RPGN prompted us to start Pulse methylprednisolone. Hemodialysis was also initiated, which improved dyspnea & anasarca. ANA dsDNA ANCA MPO PR3 antiGBM came negative repeatedly. Renal Biopsy revealed NECROTISING CRESCENTIC GLOMERULONEPHRITIS WITH LINEAR Ig-G Staining consistent with Goodpasture Syndrome. The patient underwent 9 rounds of Plasmapheresis and subsequently being followed up on monthly daycare basis cyclophosphamide immunosuppression (EUVASCYCLOPS protocol) and Oral Prednisolone regimen. ESRD was successfully averted. Erythematous Skin lesions Biopsy confirmed perforating dermatoses. Discussion: Possibly cardiomyopathy could have resulted from oliguria due to RPGN in this case Conclusion: To our knowledge, this is a unique and rare case of co-existence of DCM with anti-GBM negative Renal involvement only Goodpasture disease along with perforating dermatoses.