Case Presentation of a Patient with Systemic Lupus Erythematous, A Tour through the More Unusual Presentationss

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We presentthe case of a 23 year old female with Systemic Lupus Erethematosus [SLE] from diagnosis followed for 7 years. She presented unusually, with acute autoimmune hepatitis that has not recurred. Subsequently she developed significant haematological involvement with complications of profound thrombocytopenia and neutropenia. Ultimately she developed a rare malignancy-namely CD8+ epidermotropic T-cell lymphoma. We discuss the complications of SLE and the development of the cutaneous T cell lymphoma.

A twentythree year old female with no past medical history was referred to the Rheumatology clinic in 2009 following an acute illness managed by the Hepatologists. She presented with jaundice and diarrhoea. She had no risk factors for acute hepatitis and was not taking any hepatotoxic medication. Her liver enzymes were significantly elevated, suggestive of acute hepatic injury. Her bloods showed Bilirubin 300umol/L (0-20), ALT 1500IU/L (10-35), ALP 150IU/L (35-104 INR 1.5 and Albumin was 35g/L (34-50). Further investigations demonstrated a strongly positive anti-nuclear antibody (titre >1:5120). Anti-smooth muscle and anti-mitochondrial antibodies were negative. IgG was elevated at 23.18g/L (7.0-16.0 g/L). HIV and Hepatitis B&C serology was negative for previous infection. Subsequently a liver biopsy demonstrated evidence of acute hepatitis, with associated portal and lobular inflammation. She was treated with a tapering dose of oral prednisolone in combination with Azathioprine 75mg daily. Shortly thereafter she developed a macular rash, arthralgia and alopecia with an associated thrombocytopenia and lymphopenia.Further investigations revealed positive antibodies to Ro and double stranded DNA 208IU/ml (0-50). C3 was low at 0.77g/L (0.90- 1.80). The ESR was elevated at 66mm/hr. The anti-phospholipid screen was negative with the exception of an IgM anti-cardiolipin antibody. A diagnosis of Systemic Lupus Erythematosus (SLE) was made with associated lupus hepatitis.

She responded well to the initial treatment for several months with evidence of both clinical and serological remission. In early 2010 she presented again with mucosal bleeding and a petechial rash. Profound pancytopenia was noted with a platelet count of 0 (150-400 x10⁹/l), haemoglobin 54 (115-155 g/l) with positive direct antiglobulin test. This flare was complicated by a retinal haemorrhage resulting in central vision loss. She underwent a diagnostic Bone Marrow Aspirate and Trephine (BMAT) which demonstrated red cell aplasia with normal megakaryocytic suggesting periphera destruction. She was treated with intravenous methyl prednisolone, Cyclophosphamide and Rituximab for lupus-related pancyopenia. After several weeks her blood counts normalized. Over the next four years she remained relatively well although she required annual Rituximab infusions predominantly for mild flares comprising of arthritis, rash and constitutional symptoms.

In late December 2014, she developed serositis manifesting as pleuritic chest pain (pleurisy). An echocardiogram demonstrated associated pericarditis with a small pericardial effusion noted. She was again treated with high dose intravenous corticosteroids and Rituximab and made a good clinical recovery.