Antibodies in Aseptic Meningitis of Connective Tissue Disorder: A Case Report

TL;DR

A case report describes a young woman with a headache linked to an undiagnosed connective tissue disorder, highlighting the importance of thorough evaluation for unusual symptoms.

Contribution

This case emphasizes the need for careful differential diagnosis in atypical symptoms to avoid delayed treatment in autoimmune disorders.

Findings

Headache can be an initial symptom of mixed connective tissue disorder (MCTD).

Neurological manifestations like aseptic meningitis are rare but possible in MCTD.

Delayed treatment due to misdiagnosis can lead to poor outcomes in autoimmune disorders.

Abstract

Mixed connective tissue disorder (MCTD) is the first overlap syndrome described with features of overlapping manifestations of at least two other autoimmune rheumatic conditions. It is an autoimmune disease of rarity and is strongly associated with specific antibodies to U1 small nuclear ribonucleoprotein (anti-U1-RNP). This disorder affects almost all organs of the body, and it has varied clinical presentations as it has an autoimmune and inflammatory background, causing heightened immune cell activation. They present more commonly with less fatal symptoms like joint pain, stiffness, and mucocutaneous changes. The majority present initially with Raynaud's phenomenon followed by muscular skeletal involvement and around half of them present with swallowing problems due to esophageal dysmotility. Rarely do they also present with more morbid symptoms of pulmonary hypertension and central nervous system involvement. MCTD on follow-up had a 10 percent association with neurological manifestations as reported by the National Organization for Rare Diseases (NORD), and the most reported diseases were trigeminal neuralgia and aseptic meningitis. Patients presenting with such symptoms and, when treated only with guideline-based antibiotics therapy, would delay the treatment, leading to a poorer prognosis. The following is an interesting case of a young female presenting with a headache, which was masquerading as an underlying undiagnosed connective tissue disorder. Headache is a predominant presentation that has several etiologies in autoimmune disease and meticulous differential diagnosis workup is a must. This case highlights the fact that any persistent atypical, unusual symptom needs to be always considered for further evaluation to arrive at a diagnosis and for a favorable outcome.

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