A Teenager Diagnosed as Lymphocytic Hypophysitis Associated with Systemic Lupus Erythematosus (SLE)

Jing E Hui Wang

doi:10.4172/2155-9864.1000221

Abstract

A 15-year-old female was admitted to our hospital with intermittent fever, cough and sputum for one week, and diagnosed as pneumonia. Because of no clinic remission with antibiotic and antiviral treatment for one week and following with facial erythema, severe anemia, and neutropenia, appropriate screening laboratory examinations showed severe lower complements (C3, C4) and positives autoantibody such as ANA, ds DNA, Anti-Sm etc, Therefore, SLE was diagnosed with muti-organs involved including lungs, blood, liver, kidney, medical peritonitis, and central nervous system presented as irritable and moody. Because the patient complained of dizziness, fatigue, anorexia, hands trembling and showed a very indifferent mood, endocrinological tests were took. The results revealed very low levels of sex hormones, thyroid hormones and serum cortisol, and manifested as hypopituitarism. Magnetic resonance imaging (MRI) of pituitary region demonstrated a mild enlargement of the pituitary stalk. SLE associated with Lymphocytic hypophysitis (LYH) was suspected. And the clinical symptoms and laboratory tests were ameliorated after glucocorticoid treatment (involve twice methylprednisolone (MPIV) pulse therapy and oral 40 mg/d of MPIV) combined with cyclophosphamide therapy. Although, LYH associated with SLE have been reported, a 15-year teenager, as seen in this case, is a rare first presentation of SLE.

Highlights

Systemic Lupus Erythematosus (SLE) is a chronic multisystem autoimmune inflammatory disorder characterized by a large range of variable clinical, serological manifestations, and a relapsing-remitting course [1,2]
Lymphocytic hypophysitis (LYH) which can be classified as Lymphocytic adenohypophysitis (LAH) and lymphocytic infundibuloneurohypophysitis (LINH) is an organ-specific autoimmune disease in which the pituitary gland is enlarged secondary to inflammatory infiltration and destruction of pituitary gland [7]
We report the first youngest case that was recently diagnosed as LYH related to SLE and discuss the therapies especially methylprednisolone (MPIV) pulse treatment combined with cyclophosphamide in such disease

Summary

Introduction

SLE is a chronic multisystem autoimmune inflammatory disorder characterized by a large range of variable clinical, serological manifestations, and a relapsing-remitting course [1,2]. We report the first youngest case that was recently diagnosed as LYH related to SLE and discuss the therapies especially methylprednisolone (MPIV) pulse treatment combined with cyclophosphamide in such disease. Because the patient always complained of general malaise such as dizziness, fatigue, and anorexia, combined with her very indifferent mood and hands trembling, relevant examinations such as sex hormones, thyroid hormones and serum cortisol were tested. The cortisol continued below normal level that may associated with SLE and LYH. LYH was suspected on the strength of clinical symptoms, endocrine function, as well as pituitary MRI. The renal pathological diagnosis was diffuse glomerulonephritis [IV-G(A)] (Figure 3), combined with accelerated erythrocyte sedimentation rate and high level of serum C-reactive protein (CRP), the second MPIV pulse therapy

Discussion

Conclusion