Case of lifelong symptoms of systemic lupus erythematosus
This article describes the case of a 56-year-old male patient diagnosed with systemic lupus erythmatosus (SLE). The patient presented with a combination of clinical manifestations including thrombocytopenic purpura (TTP), DVT and glomerulonephritis. He was suffering with symptoms of systemic lupus erythematosus (SLE).
The patient presented to the emergency department with complaints of extreme weakness, anorexia and fatigue, with a history of 2 months. Similarly, his medical history revealed an episode of thrombotic thrombocytopenia purpura (TTP) that was diagnosed 20 years ago. Doctors treated him with plasmapheresis, corticosteroids and aspirin which helped with complete recovery. However, 18 months after treatment, doctors diagnosed him with right leg deep vein thrombosis without an apparent cause. In addition, his antiphospholipid antibodies were negative.
In addition, several years prior to this current presentation, the patient had presented with similar symptoms of weakness and weight loss. He lost over 30 kgs in over a few months. Doctors advised blood tests, computed tomography (CT) and positron emission tomography/CT (PET/CT). Doctors advised an excisional biopsy of the pelvic lymph nodes and bone marrow which showed no significant findings. Immune serologies were significant for only a weakly positive antinuclear antibody (ANA).
Differential diagnosis
The differentials included a systemic infection, autoimmune lymphoproliferative syndrome (ALPS) and indolent neoplastic process. However, investigations ruled out all these diagnoses. Upon admission, the patient’s physical examination showed bilateral leg oedema and numerous lesions on the skin. Examination showed normal vital signs, however, recurrent fever spikes were evident.
The patient’s blood tests were significant for elevated inflammatory markers, high creatinine levels, hypoalbuminemia and pancytopneia. Doctors advised a CT scan which was unremarkable. However, PET/CT scan of the patient showed diffusely increased BM uptake and hypermetabolic lymphadenopathy. In addition, the patient’s clinical condition continued to deteriorate with dyspnoea, fatigue and ongoing weakness. He also complained of progressive asymmetric leg oedema and chest pain. Tests showed an extensive DVT in the right leg, new onset atrial fibrillation and elevated cardiac enzymes. In addition to this, a small pericardial effusion was also evident on the transesophageal echocardiography (TTE).
Doctors started the patient on treatment with enoxaparin, for DVT and atrial fibrillation. Doctors also suspected a bacterial infection, for which they started him on broad-spectrum antibiotic coverage. Biopsy of the skin lesions showed deposits of lupus band and lichen planus-like pathological picture.
The patient was diagnosed with systemic lupus erythematosus. Treatment included high-dose corticosteroid therapy, ramipiril, mycophenolate mofetil and hydroxychloroquine. The patient was discharged after 4 weeks of hospital stay for rheumatology and nephrology outpatient clinics. A year later, the patient is well and suffers no with no residual neurological deficits.
References
From TTP to Glomerulonephritis: A Lifetime of Lupus https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7803419/
