Hoarseness and Shortness of Breath: Is Your RA the Cause?
When discussing of the symptoms of rheumatoid arthritis, we often talk about pain, stiffness, swelling, and disability. We don’t often talk about vocal quality or ability to breathe freely, but RA can affect the larynx and small joints of the head and neck, including temporomandibular joint (TMJ), the cricoarytenoid joint (CAJ), and the cricothyroid joint (CTJ).
According to a new literature review in the journal Autoimmune Diseases, the prevalence of laryngeal symptoms of RA has risen from up to 31% of RA patients in 1960 (Lawry, 1984) to 75% by the end of the 20th century (Hamdan, 2013). At least a portion of this significant increase is likely due to increased awareness and better clinical diagnosis.
Symptoms of larynx involvement caused by RA include odynophagia (painful swallowing), foreign body sensation, dysphagia (difficulty swallowing), sore throat, lump sensation in the throat, change in voice quality (e.g. hoarseness, breathiness, vocal fatigue), referred otalgia (earache), and respiratory symptoms (e.g. shortness of breath, decreased exercise tolerance, stridor, dyspnea, respiratory distress). In rare cases, patients with RA may also present with symptoms of croup. Patients may also be asymptomatic.
Dysphonia, or change in voice quality, may be the most noticeable symptom caused by changes in the larynx caused by RA. The change in voice quality may vary from mild roughness to complete aphonia (inability to produce a sound). Based on the GRBAS scale - where G stands for grading, R for roughness, B for breathiness, A for asthenia, and S for straining - 35% of patients with RA have grades 2 and 3 (Amernik, 2007).
In a study of 77 patients with RA with average disease duration of 9.4 years, the most frequent complaints were foreign body sensation in 51%, hoarseness in 47%, and voice weakness in 29% of the cases (Amernik, 2007). In acute phases, patients may complain of burning, foreign body sensation in the throat, and difficulty in swallowing. In chronic cases, the cricoarytenoid joint (CAJ) can become fixed and airway symptoms that arise may require an emergency tracheotomy.
When inflammation is moderate and only one laryngeal joint is involved (CAJ or CTJ), patients may experience minimal airway symptoms, with occasional or mildly persistent respiratory discomfort, shortness of breath, and decrease in exercise tolerance. If both vocal cords are immobile, patients may experience breathiness, vocal fatigue, inability to sustain or produce sound. If the vocal cords are fixed in the midline, the arthritis may endanger the patient with dyspnea (difficulty swallowing) and choking.
Examination with an endoscope to view the larynx may reveal inflammation, mucosal edema, engorgement (hyperemia), myositis, impaired mobility or fixation of the vocal folds, inflammation of the epiglottis, rheumatic nodules, or Bamboo nodes. However, in the early stage of the disease, laryngeal examination may be normal.
Inflammatory masses or rheumatoid nodules in the larynx and pharynx are similar to rheumatoid nodules that can develop elsewhere in the body. The nodules can be submucosal and/or subcutaneous masses. Diagnosis of the nodule can be done by excising the lesion or by performing a fine-needle aspiration. Bamboo nodes are cystic yellowish deposits which are arranged transversally in the vocal folds. These lesions are seen more often in patients with active disease and in females with history of gastroesophageal reflux disease (GERD).
Electromyography (EMG) is a useful test to distinguish between CAJ fixation and paralysis secondary to recurrent laryngeal nerve injury. High-resolution CT (computerized tomography) scan can detect CAJ arthritis in the early stages. Scans may reveal CAJ prominence, increased density of the joint, narrowing of the joint space, ankylosis, and vocal fold thickening.
Medical treatment for laryngeal manifestation of RA includes use of steroids or nonsteroidal anti-inflammatory drugs (NSAIDs) to avoid formation of nodules or fibrosis. Steroids may be given systemically or locally injected into the joint. Bamboo nodes may be removed surgically. Conservative treatment may begin with speech therapy. If after speech therapy patients still have trouble with changes in vocal quality, steroid injection and surgical intervention may be advised. When both vocal folds are fixed in the midline, a tracheotomy (temporary or permanent) may be needed to alleviate the obstructed airway.
As RA can affect many areas of the body, including the biomechanics of the larynx, it is important for RA patients and physicians to be aware of the significance of subtle airway symptoms and/or changes in voice quality. If your voice has become hoarse, you feel as though you have something in your throat, or your breathing becomes stressed, tell your doctor. Early diagnosis, proper treatment, and rehabilitation of laryngeal manifestations can alleviate suffering and increase safety for the RA patient.
Amernik K. Glottis morphology and perceptive-acoustic characteristics of voice and speech in patients with rheumatoid arthritis. Annales Academiae Medicae Stetinensis 2007;53(3):55–65.
Hamdan AL, Sarieddine D. Laryngeal Manifestations of Rheumatoid Arthritis. Autoimmune Diseases 2013; vol. 2013, Article ID 103081, 6 pages.
Kamanli A, Gok, U, et al. Bilateral cricoarytenoid joint involvement in rheumatoid arthritis: a case report. Rheumatology 2001;40:593-594. doi:10.1093/rheumatology/40.5.593
Lawry GV, Finerman ML, Hanafee WM. Laryngeal involvement in rheumatoid arthritis: A clinical, laryngoscopic, and computerized tomographic study. Arthritis and Rheumatism 1984;27(8):873–882.