Seronegative Lupus- A Wolf in Sheep’s Skin

Abstract

Systemic Lupus Erythematosus (SLE) is a chronic inflammatory multisystem autoimmune disease. Most of the SLE cases are seropositive, but rare (5%) seronegative SLE cases can also present with complications. Hepatic involvement SLE is multifactorial like hepatotoxic drugs, steatohepatitis, viral hepatitis and Autoimmune Hepatitis (AIH). However, the differentiation between lupus-related hepatitis and AIH remains a challenge to the clinician because of many similar features. It is very difficult to differentiate whether hepatitis is due to autoimmune involvement or purely lupus related on the basis of clinical and biochemical parameters. The patient must fulfill ACR criteria for SLE and International Autoimmune Hepatitis Group (IAIHG) criteria for AIH. Histological diagnosis is considered to be definative in differentiating SLE-related hepatitis and AIH. The cardiac manifestations of SLE are multiple with pericardial disease being the most common. While pericardial effusion is rarely haemodynamically significant, the occurrence of subsequent constrictive pericarditis is even less frequent with only occasional reports in the literature. Authors described a case of a 17-year-old female with Antinuclear Antibody (ANA) negative active SLE (seronegative) with AIH and constrictive pericarditis. The patient responded well to the conservative management initially but later developed drug induced myelosuppression and bilateral pneumonia and succumbed.