Autoimmunity and postural orthostatic tachycardia syndrome: Implications in diagnosis and management

Albert Aboseif; J. Daniel Bireley; Yuebing Li; David Polston; Justin R. Abbatemarco

doi:10.3949/ccjm.90a.22093

Article Figures & Data

Tables

View popup

TABLE 1

Diagnostic criteria for postural orthostatic tachycardia syndrome

All of the following criteria are necessary:

A sustained increase in heart rate by ≥ 30 beats per minute within 10 minutes of standing or head-up tilt in adults (or ≥ 40 beats per minute for patients ages 12–19) without orthostatic hypotension (a fall in systolic blood pressure ≥ 20 mm Hg or diastolic blood pressure ≥ 10 mm Hg)

Associated symptoms of orthostatic intolerance that are worse with standing (light-headedness, fatigue, palpitations, tremulousness, blurred vision, syncope) and improve with recumbency

Symptom duration of at least 3 months

Absence of other conditions to explain sinus tachycardia (prolonged bed rest, medications, hyperthyroidism, anorexia nervosa, anemia, pain, fever, infection, dehydration)

Based on information in reference 1, 2, 4, and 5

View popup

TABLE 2

Clinical presentation and associated symptoms in postural orthostatic tachycardia syndrome

Cardiovascular symptoms	Orthostatic intolerance, orthostatic tachycardia, palpitations, dizziness, lightheadedness, presyncope, syncope, exercise intolerance, dyspnea, chest pain, acrocyanosis, Raynaud phenomenon, venous pooling, limb edema

Noncardiovascular symptoms
General	Deconditioning, fatigue, heat intolerance, fever

Nervous system	Headache, migraine, cognitive impairment, “brain fog,” difficulty concentrating, tremulousness, photophobia, phonophobia, blurred vision, neuropathic pain, sleep disorder, involuntary movements

Musculoskeletal	Muscle fatigue, weakness, pain

Gastrointestinal	Nausea, bloating, dysmotility, gastroparesis, diarrhea, constipation, pain, weight loss, irritable bowel syndrome

Respiratory	Shortness of breath, hyperventilation, bronchial asthma

Urogenital	Bladder dysfunction, polyuria, nocturia, urgency, frequency

Skin	Rash, erythema, petechiae, telangiectasias, diaphoresis, flushing, pallor, dry eyes, dry mouth, sudomotor dysregulation (hyperhidrosis, hypohidrosis, anhidrosis)

Psychiatric	Anxiety, depression, panic attacks, suicidal ideation, somatic hypervigilance, catastrophizing personality

Based on information in reference 1.

View popup
TABLE 3
Commonly reported symptoms in patients with postural orthostatic tachycardia syndrome
Lightheadedness or dizziness 78%–87%
Palpitations 75%–92%
Fatigue 48%–90%
Headache 28%–87%
Breathing difficulty 28%–64%
Tremulousness 38%–49%
Based on information in references 1, 5, 6, and 7.

View popup

TABLE 4

Autonomic review of systems

System	Symptoms
Sudomotor	Hyperhidrosis, hypohidrosis, anhidrosis, heat intolerance
Secretomotor	Dry eyes, dry mouth (sicca symptoms)
Cardiovascular	Postural lightheadedness or palpitations, presyncope, syncope
Gastrointestinal	Nausea, bloating, early satiety, gastroparesis, dysphagia, constipation, diarrhea
Genitourinary	Urinary urgency or retention, nocturia, incontinence, impotence, dyspareunia

Based on information in reference 5.

View popup

TABLE 5

Red-flag features suggesting an alternative diagnosis

Onset	Acute to subacute onset over days to weeks
Widespread autonomic involvement	Involvement of the parasympathetic, sympathetic, and enteric nervous systems, affecting multiple organ systems
Functional decline	Loss of functional independence and increased disability level due to progression of symptoms
Signs and symptoms	Pupillary dysfunction, hyperhidrosis or anhidrosis, urinary retention, sexual dysfunction, and gastrointestinal dysmotility
Relevant personal or family history	Personal or family history of autoimmune conditions or malignancy
Central nervous system dysfunction	Clinical features of cortical, brainstem, cerebellum, or spinal cord dysfunction
Endocrinopathy	Amenorrhea, syndrome of inappropriate antidiuretic hormone secretion, adrenal insufficiency, panhypopituitarism