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Diseases of the Aorta
Published in Mary N. Sheppard, Practical Cardiovascular Pathology, 2022
About one-third of patients with untreated primary syphilis will develop tertiary disease, among whom the majority will have cardiovascular manifestations. Syphilitic aortitis tends to affect a discrete segment of the aorta; a band just above the aortic valve is the most common site. Syphilitic aneurysm spares the sinus portion and diffusely involves the tubular portion of ascending aorta, beginning at the sinotubular junction. At this site, it causes aortic regurgitation and may be associated with coronary ostial stenosis. Discrete bands of aortitis can be recognized by the wrinkled tree-bark scarring of the intima (Fig. 8.35). Patients develop localized saccular aneurysms which can compress or rupture into the right bronchus, the superior vena cava or the right pulmonary artery. Erosion into the back of the sternum was common in the last century and specimens illustrating this can be found in many pathology museums. Syphilitic descending thoracic aneurysms compress or rupture into the oesophagus or trachea. Syphilitic abdominal aneurysms are very rare, but popliteal aneurysms are a classic site described by John Hunter in the 18th century. Cardiovascular syphilis has not entirely disappeared. In patients with dilated ascending aortas, with or without aortic regurgitation, a serological test for syphilis is recommended.
Syphilis
Published in Scott M. Jackson, Skin Disease and the History of Dermatology, 2023
The last phase of syphilis is called tertiary syphilis, which tends to occur many years after the onset of the original infection. Tertiary syphilis is much less common today as the available treatments and early testing prevent the progression of the infection to this stage. The three major features of tertiary syphilis are gummatous lesions, cardiovascular syphilis, and neurosyphilis. Gummatous lesions are chronic infectious infiltrations of an organ, such as the skin or liver, which resemble a tumor-like growth; on the skin, the lesions appear as an ulcerated tumor or nodule, or if the nose is affected, it can lead to destruction of the nose. Cardiovascular syphilis involves inflammation of the aorta, the arteries around the heart, or the valves of the heart; it can be complicated by aortic aneurysm, heart attack, or heart failure. Cardiovascular complications are the most common cause of death in syphilis. In neurosyphilis, the brain, as well as the spinal cord and peripheral nerves, is affected by the infection. Brain involvement can present with stroke-like symptomatology or psychiatric disease, including personality changes, dementia, psychosis, mania, delirium, and depression. Neurosyphilis, formerly known as “generalized paresis of the insane,” is caused by chronic meningoencephalitis and resultant atrophy of the brain. Spinal cord involvement, termed “tabes dorsalis,” presents with weakness, diminished reflexes, paresthesias, locomotor ataxia, episodic intense pain, urinary incontinence, deafness, eye pain, and visual impairment. Many of these tertiary neurologic features are irreversible and can be misdiagnosed as Alzheimer's disease, multiple sclerosis, or schizophrenia unless the astute clinician remembers that syphilis is the “Great Imitator” and orders the blood test for syphilis.
Ocular Syphilis as a Cause of Chronic Postoperative Uveitis Followed by a Localized Ocular Jarisch–Herxheimer-like Reaction
Published in Ocular Immunology and Inflammation, 2023
Parsha Forouzan, David Fell, Freddie R. Jones
According to the most recent report from the Centers for Disease Control and Prevention (CDC) in 2019, syphilis is on the rise with both acquired and congenital forms increasing 75% and 280%, respectively, since 2015,1 which is likely an underestimation given the omission of latent infections. Syphilis adopts variable clinical presentations, can be difficult to detect yet remains transmissible during its latent phase, and can have severe neurologic and ocular consequences if left untreated. Acquired syphilis is typically transmitted through direct sexual contact with infectious lesions and can be present in one of the four different stages. Primary syphilis develops within a few weeks of infectious contact and classically manifests as a painless chancre. Secondary syphilis presents weeks to months later after resolution of the chancre and hematogenous spread of spirochetes, usually resulting in cutaneous and lymph node involvement and less often liver, kidney, joint, and ocular involvement. After resolution of symptoms, untreated syphilis enters a latent stage. In the early latent stage within the first one to two years of initial infection, 25% of the infected persons may exhibit recurrent symptoms and can potentially transmit their infection.2 The late latent stage of untreated syphilis can last decades with most remaining asymptomatic, but 15–40% of the infected persons progress to tertiary syphilis.3 Tertiary syphilis is manifested by gummatous syphilis (15%), cardiovascular syphilis (10%), and neurosyphilis (6.5%).4
Ocular Syphilis: An Update
Published in Ocular Immunology and Inflammation, 2019
Parthopratim Dutta Majumder, Elizabeth J. Chen, Janika Shah, Dawn Ching Wen Ho, Jyotirmay Biswas, Leo See Yin, Vishali Gupta, Carlos Pavesio, Rupesh Agrawal
The natural history of syphilis is complex and variable, spanning three progressive clinical stages with chronological overlap.13 Primary syphilis is characterized by a painless chancre occurring at the site of inoculation that resolves spontaneously within 2 to 8 weeks. The incubation period ranges from 3 days to 3 months, with a median of 3 weeks. Secondary syphilis occurs 2 to 12 weeks after inoculation, presenting with systemic symptoms (fever, malaise, lymphadenopathy and mucocutaneous lesions) coinciding with maximal treponemal bacteremia. Tertiary syphilis occurs when gummas affect any organ. It is subdivided into benign tertiary syphilis, cardiovascular syphilis, and neurosyphilis. Ocular syphilis typically does not manifest in the primary stage, except as chancres of eyelid and conjunctiva. The secondary stage may have ocular involvement such as keratitis, iris nodules, iridocyclitis, episcleritis, and scleritis. Latent syphilis is defined as positive serological tests with no clinical evidence of infection. However, in the first year after the initial infection, infectious mucocutaneous lesions may recur. This is known as early latent syphilis. Late latent phase occurs after 1 year of infection, during which infectious relapses are rare. Late in the secondary stage, chorioretinitis and vitritis may develop. However, it is still more frequent in the late, latent, and tertiary stages.14,15
Clinical Manifestations and Cerebrospinal Fluid Status in Ocular Syphilis
Published in Ocular Immunology and Inflammation, 2019
Steven Lapere, Hamzah Mustak, Jonel Steffen
The CDC revised its case definition of syphilis in 2014, dividing the classification into congenital syphilis, primary, secondary, latent and late (benign and cardiovascular) syphilis.9 The term ‘neurosyphilis’ has been removed from the ‘late syphilis’ classification as it can occur at any stage of the disease. Ocular syphilis can be associated with neurosyphilis10 and has a nonspecific presentation which can include uveitis (anterior, intermediate, posterior or panuveitis), scleritis and optic neuritis.2 Human immune virus (HIV) coinfection is an important consideration and several studies have looked at the difference in presentation and outcome between HIV-positive and HIV-negative patients.11–15 The role of lumbar puncture in the diagnosis of ocular syphilis remains unclear. The CDC states that ocular syphilis should be treated as neurosyphilis, even if CSF examination is normal.10 Non-treponemal tests on cerebrospinal fluid are highly specific but insensitive and treponemal tests are highly sensitive but nonspecific.10