The prevalence of monospecific anti-PM75 and anti-PM100 antibodies in this large SSc cohort was low. Disease features associated with anti-PM/Scl antibodies may depend on particular and possibly multiple antigen specificities. However, due to the small samples, these results need to be interpreted with caution. International collaborations are key to understanding the clinical correlates of uncommon serological profiles in SSc.
Rheumatoid meningitis is a rare complication of rheumatoid arthritis (RA). It is associated with substantial morbidity and mortality. The condition may present in a variety of ways and is therefore diagnostically challenging. Uncertainty still exists regarding the optimal treatment strategy. Herein, we describe the case of a 74-year-old man with a history of well-controlled seropositive RA on low-dose prednisone, hydroxychloroquine, and methotrexate. The patient presented with a several-month history of multiple prolonged episodes of expressive aphasia, right hemiparesis, and encephalopathy. Although no epileptiform activity was recorded on repeated electroencephalography, the symptoms fully resolved following treatment with antiepileptic drugs. He subsequently developed acute asymmetrical parkinsonism of the right hemibody. Magnetic resonance imaging revealed subtle enhancement of the leptomeninges over the left frontoparietal convexity. Cerebrospinal fluid analysis revealed a mild lymphocytic pleocytosis and elevated proteins. Histopathologic analysis of a meningeal biopsy revealed nodular rheumatoid meningitis. The patient was treated with corticosteroids and cyclophosphamide, following which he incompletely recovered. This is the first description of rheumatoid meningitis manifesting with acute parkinsonism and protracted non-convulsive seizures. A summary of cases reported since 2005, including data on pathology, therapy and outcomes, along with a discussion on the efficacy of different treatment strategies are provided.
L yell disease, commonly referred to as toxic epidermal necrolysis (TEN), is a rare, lifethreatening condition probably related to drug hypersensitivity; the mortality rate is approximately 30%.1 The disease is characterized by an initial pruritic, erythematous rash followed by the appearance of bullae, which subsequently lead to ulceration and sloughing of the underlying epidermal base similar to physiological damages caused by burns. Moreover, TEN causes crusting, desquamation, and ulceration of the mucous membranes in areas such as the oral, ocular, and genital mucosa.2 Bleeding from internal mucous membranes, especially those in the gastrointestinal and respiratory tracts, can progress to hypovolemia and lead to death. TEN is often accompanied by ocular manifestations ranging from acute conjunctivitis to corneal abrasion and, in the most severe cases, corneal ulceration.3 These changes can lead to permanent loss of vision. Feature A patient with severe toxic epidermal necrolysis underwent 2 cycles of therapeutic plasma exchange and received specialized wound care for widespread skin damage of more than 80% of his body surface area. Extensive involvement of mucous membranes, including the conjunctivas and the oropharyngeal cavity, and damage of his genitourinary organs required meticulous wound care. Daily care of injuries of tissues affected only in the most severe cases of toxic epidermal necrolysis was provided by an experienced intensive care unit nursing team. A meticulous supportive therapy regimen was a major contributing factor to this patient's remission.
Supportive Therapy(Critical Care Nurse.
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