An interview with rheumatologist, researcher, AS patient, and long-time member of SAA’s Medical and Scientific Advisory Board, Muhammad Asim Khan, MD
We begin this conversation around possible impacts of long-standing spondyloarthritis with none other than Dr. Khan – a noted and beloved rheumatologist, who himself has long-standing ankylosing spondylitis. Our thanks to Dr. Khan for helping us kick off this topic!
Spondylitis Plus: What cardiac related risks and potential complications should those with long-standing spondyloarthritis be aware of? What screenings are recommended for these issues? And are there any preventative measures people can take to reduce cardiac risks?”
Dr. Khan: Some patients with longstanding spondyloarthritis (SpA) may develop a slowly progressive inflammation and resultant scarring (fibrosis) at the root of the aorta where it comes off from the heart. This inflammation can extend to the adjacent initial few centimeters of the wall of the aorta and can be much better visualized by PET/CT scan. It can also involve the aortic valve (causing leaky valve – aortic valve insufficiency) and even the heart’s electric conduction system (causing electric conduction disturbances – leading to slowing of the heartbeat that may be serious enough to require a pacemaker). The worsening aortic valve insufficiency, which can easily be detected by echocardiography, can cause symptoms of heart failure, including leg or ankle swelling (edema) and shortness of breath during exercise or exertion. This requires monitoring and, in some cases, aortic valve replacement.
The risk for aortic insufficiency and electric conduction disturbances increases with the age of the patient, the duration of SpA, and is more common among those who are HLA-B27 positive, as well as among those with hip, shoulder, or peripheral (limb) joint involvement. Cardiac electric conduction disturbances occur in up to 3% of those with disease of 15 years’ duration, and in up to 9% of those with disease duration of 30 years or more. I may add that I underwent cardiac pacemaker implantation in April of this year, a week after my 77th birthday. I have suffered from ankylosing spondylitis since age 12, have had bilateral hip joint replacements, possess HLA-B27, and have named my website HLAB27.com.
Some patients have impaired relaxation of their heart muscle but with normal ability to contract to pump blood out. This however can lead to impaired cardiac function after many years. Lastly, it is worth stating that, as among the general population, high blood pressure, high cholesterol, smoking, and sedentary lifestyle all increase the risk of coronary (heart) artery disease and stroke. Controlling and minimizing these are some of the preventable measures that can reduce cardiac risks.
Spondylitis Plus: We know that some with long-standing SpA experience lung restriction / chest expansion issues and pain in the rib cage. Can we discuss this, and any other lung-related impacts of SpA?
Dr. Khan: People with axial spondyloarthritis (axSpA) can complain of pain in their rib cage that is worsened by coughing or sneezing because of inflammation of the joints where the ribs attach to the backbone. They may also complain of soreness (tenderness) of the anterior chest wall. It is commonly called costochondritis because the tenderness is most prominent over the cartilaginous parts of the ribs where they attach to the breast bones (sternum and manubrium).
People with axSpA have no symptoms of lung disease; nevertheless, they may have functional lung impairment – documented by lung function testing – because of restricted chest expansion resulting from progressive fusion of the ribs with the spine. However, this does not usually result in breathing insufficiency because of compensatory increased use of diaphragm muscles (that separate the lungs from the abdomen) used in normal breathing.
Some patients may take longer to recover from severe influenza, bronchitis, or pneumonia. COVID-19 infection can also potentially result in longer recovery times. Patients should discuss with their doctor the need for vaccines against viral infections and bacterial pneumonia.
Very rarely, there can be some changes (fibrosis) in the lung tissues, especially at the uppermost part of the lung. This may or may not cause breathing problems or other complications.
Patients may also be more prone to obstructive sleep apnea (especially if they also have forward stooping and immobile neck, and/or are markedly overweight) that may result in poor sleep and require use of breathing assistance machines (CPAP or BIPAP). Smoking tobacco cigarettes, including electronic cigarettes (vaping) should be completely avoided by everyone with SpA.
Spondylitis Plus: Can long-standing SpA, or medication used for SpA, lead to kidney involvement?
Dr. Khan: Kidney disease in SpA can occur for various reasons and present with protein leakage in the urine (sometimes associated with presence of red cells detected on routine urinalysis) with or without impairment of kidney function. NSAIDs can cause fluid retention, mostly manifested by swelling of the ankles. Their long-term use can impair the normal functioning of the kidneys and can also cause hypertension (increased blood pressure) or blunt the effect of drugs used to treat it, such as diuretics (water pills), ACE-inhibitors (such as lisinopril (Zestril)), and ACE-receptor blockers (such as losartan (Cozaar)). This makes their use risky for patients who already have kidney disease, and for the elderly population. NSAIDs can also cause acute inflammation in the kidneys, called acute interstitial nephritis, after a few days of use. This side effect seems to be under recognized.
Adverse effects on kidney function can also result from other drugs used to treat SpA. Rarely, inflammation of the kidneys (nephritis) can occur with deposition of an immunoglobulin (Ig) protein called IgA that can result in protein leakage and blood cells in the urine, with or without impairment of kidney function. Deposition in the kidneys of another protein called amyloid (amyloidosis) has now become very rare in the U.S. and most of the other developed countries due to very effective disease management and is now mainly seen in patients with poorly controlled disease.
Spondylitis Plus: Can there be neurologic involvement in long-standing SpA? And is there any relationship between peripheral neuropathy and biologic medications, such as TNF inhibitors?
Dr. Khan: Neurologic involvement may occur in axSpA patients due to fracture of the spine and resultant spinal instability. Although TNF inhibitors are generally well tolerated, there have been rare but well-documented reports of associated peripheral neuropathies, including Guillain-Barre syndrome. There is also an extremely rare and very slowly progressive complication in patients with very long-standing, severe AS. It is called cauda equina (meaning horsetail) syndrome because of the involvement of lowermost spinal nerves that slope downward as a bunch before leaving the spinal column. It is caused by fibrous entrapment and scarring of the lowermost nerve roots and can result in “saddle anesthesia” (so called because of loss of skin sensation over the parts we sit on), urinary retention and incontinence, and fecal incontinence due to decreased urinary sphincter and rectal tone. Men may develop erectile dysfunction or impotence. It may also cause some pain and weakness in the legs. Its characteristic feature is presence of enlarged sacs containing spinal fluid and erosions of the spinal canal, best seen on CT or MRI.
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Editor’s Note: We will highlight additional complications and issues associated with long-standing SpA in our fall 2021 issue of Spondylitis Plus. Keep an eye out for part two of this important topic, and if you’d like to suggest questions around long-standing SpA complications please send them to the editor at Elin@spondylitis.org.
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