Coevality of Systemic Lupus Erythematosus With Sickle Cell Trait: A Not So Uncommon Entity

Dhriti Sundar Das,Debananda Sahoo

doi:10.7759/cureus.10119

Abstract

The coexistence of systemic lupus erythematosus (SLE) with sickle cell trait is quite sparingly reported in literature. Here, we narrate the case of a 17-year-old girl from Eastern India with sickle cell trait who presented with acute lupus pneumonitis. The challenges to the final diagnosis of SLE with sickle cell trait were because of the often lesser degree of clinical suspicion at the outset. In this report, we discuss this not so uncommon combination of conditions and review related literature. This girl, who was a known case of sickle cell trait, presented with fever, cough, shortness of breath with subsequent rashes, oral ulceration, high erythrocyte sedimentation rate (ESR) and proteinuria. After ruling out infective causes, she was found to be antinuclear antibody (ANA) positive and with stage 4 lupus nephritis. Emphasis should be given to the presence of autoimmune conditions in patients with sickle hemoglobinopathies, including sickle cell trait wherein atypical or systemic involvement may occur. Such association holds more importance as sickle hemoglobinopathies is one of the major hemoglobinopathies reported in this part of the country.

Highlights

Sickle hemoglobinopathies, a group of commonly encountered genetic disorders that include sickle cell trait, sickle cell disease, and sickle Beta−Thalassemia, are prevalent in this eastern part of India [1,2]
We report a case of known sickle cell trait whose clinical features and subsequent presentations lead to the diagnosis of lupus
Sputum was negative for acid-fast bacilli (AFB) and cartridge-based nucleic acid amplification test (CBNAAT)

Summary

Introduction

A group of commonly encountered genetic disorders that include sickle cell trait, sickle cell disease, and sickle Beta−Thalassemia, are prevalent in this eastern part of India [1,2]. We report a case of known sickle cell trait whose clinical features and subsequent presentations lead to the diagnosis of lupus. A 17-year-old girl presented with low-grade fever, not associated with chills and rigor, cough with expectoration for two weeks, non-radiating chest pain, and breathlessness of three days duration She was a known case of sickle cell trait. Further evaluation revealed high erythrocyte sedimentation rate (ESR; 151 mm at the end of the first hour) with anemia, rashes over both feet, bilateral cervical lymphadenopathy, painless oral ulcer and amenorrhea. Her initial total leucocyte counts and renal function test were normal.

Discussion

Conclusions

Disclosures

Findings

Balgir RS