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Inflammatory, Hypersensitivity and Immune Lung Diseases, including Parasitic Diseases.
Published in Fred W Wright, Radiology of the Chest and Related Conditions, 2022
Many patients develop or present with Pneumocystis pneumonia (which may be lifethreatening), or other infections (mycobacterial - TB, etc - see ps. 19.16 et seq., fungi aspergillosis, nocardia, histoplasmosis, etc., Candida of the throat and oesophagus - see ps. 16.16 - 17, viruses - CMV (causing both lung infection and retinitis - leading to blindness), varicella, or protozoa - toxoplasma, etc.). Not uncommonly a mixture of infections is found (see also opportunistic pneumonias - p. 19.7). Some patients produce drug reactions e.g. Stevens-Johnson syndrome. Non-specific pneumonitis, LIP (p. 19.119) may occur particularly in children, and congestive cardiomyopathy may also be present. Infections are often atypical and chronic and a fatal encephalopathy (particularly in patients with cryptococcal infection) is not uncommon. Sinusitis may also be an important clinical problem - see reference to Chong et al. (below). Secondary infections particularly occur when the T cell CD4 level drops below 200/ cu ml. (When the virus encounters a T cell, proteins on the virus bind to both CD4 and co-receptors and the virus enters the cell and its genetic material is integrated into the T cell's DNA to produce more viral protein which is released into new viruses).
Acute erythematous rash on the trunk and limbs
Published in Richard Ashton, Barbara Leppard, Differential Diagnosis in Dermatology, 2021
Richard Ashton, Barbara Leppard
Occasionally, the skin lesions may be widespread and associated with blisters or erosions in the mouth. Stevens-Johnson syndrome is erythema multiforme with extensive mucous membrane involvement (see p. 106).
Adverse Reactions to Antibiotics in the Critical Care Unit
Published in Cheston B. Cunha, Burke A. Cunha, Infectious Diseases and Antimicrobial Stewardship in Critical Care Medicine, 2020
Diane M. Parente, Cheston B. Cunha, Michael Lorenzo
Stevens-Johnson syndrome is erythema multiforme with mucosal involvement. The most commonly implicated antibiotics are the aminopenicillins and sulfonamides. Onset is typically 1‒3 weeks. The rash can present as maculopapular and/or target lesions [30]. Stevens-Johnson syndrome can involve mucosa of the eyes, mouth, and the genitourinary tract, and up to 25% of cases may only involve the oral mucosa. Diagnosis can be confirmed by skin biopsy with immunofluorescent staining. Infections, e.g., mycoplasma, may cause Stevens-Johnson syndrome. Stevens-Johnson syndrome can evolve into toxic epidermal necrolysis, which carries a morality of 30% [30].
Lacrimal Gland Insufficiency in Aqueous Deficiency Dry Eye Disease: Recent Advances in Pathogenesis, Diagnosis, and Treatment
Published in Seminars in Ophthalmology, 2022
Acquired causes of non-Sjögren’s-type ADDE include immune-mediated conditions such as Stevens–Johnson syndrome (SJS), mucous membrane pemphigoid (MMP) and graft versus host disease (GVHD).3,17,18 Stevens-Johnson syndrome is a chronic mucocutaneous blistering disease, which is triggered by drugs or viral infections.19–22 It is said to occur as a result of dysregulated immune response, mainly pertaining to innate immunity.23,24 It is a cytokine-mediated response, which leads to the deposition of neutrophils in the conjunctiva of these patients.25 In both SJS and MMP, there is chronic inflammation of the ocular surface, which if not treated in time, leads to cicatrising conjunctivitis, ultimately causing ADDE. Graft versus host disease (GVHD) is an adverse immunological event following allogeneic hematopoietic stem cell transplantation. It includes a T cell-mediated immune response which causes infiltration and inflammation of ocular structures such as the lacrimal glands and conjunctiva, leading to ADDE.26,27 Other acquired causes of non-Sjögren’s-type ADDE include ocular chemical burns, iatrogenic causes such as kerato-refractive surgeries, long-term use of topical and systemic medications and radiation therapy for head and neck cancers. Some of these conditions, like radiation therapy, cause direct damage to the lacrimal gland and the ocular surface, while others, like topical anti-glaucoma medications, cause chronic inflammation and fibrosis of the ocular surface leading to cicatrising changes.
Drug-induced Stevens-Johnson syndrome: a disproportionality analysis from the pharmacovigilance database of the World Health Organization
Published in Expert Opinion on Drug Safety, 2022
Yi Zheng, Wenli Zhou, Xiaojing Guo, Lijie Chi, Chenxin Chen, Zhijian Guo, Jizhou Liang, Lianhui Wei, Xiao Chen, Xiaofei Ye, Jia He
Stevens-Johnson syndrome (SJS) is a rare skin condition that has been categorized as one of the most serious disorders of the skin. The initial clinical manifestations of SJS include fever, uncomfortable swallowing, eye irritation, and other systemic nonspecific symptoms [], followed by damage to various skin tissues and mucus membranes[1]. Mucosal damage is severe and extensive and can involve the mucosa of the mouth, eyes, vulva, respiratory tract, and digestive tract. Damage to the eyes, mouth, and vulva are the most common [2]. It is worth noting that long term ocular complications would affect 20–75% of survivors of SJS [3]. Damage to the mucosa of the digestive tract and respiratory tract is more serious, and can lead to serious complications such as gastrointestinal bleeding and respiratory failure. Skin damage includes papules, erythema, bullae, or other forms of skin disturbances, including a positive Nikolsky sign [4]. Skin lesions are distributed on any part of the body, except for the palms, plantars, backs of hands, and extensible sides of limbs, and often involve the trunk and face. In severe cases of SJS, 10% of the body’s skin surface area may be detached [5]. The incidence of severe exfoliative skin reactions is estimated to be 1 to 7 cases per million person-years [6]. Severe cases may be accompanied by liver damage, renal failure, respiratory failure, and other serious complications, with a mortality rate of 1–5% [7]. However, the mortality rates of the elderly and children with severe SJS are much higher.
Clinical Characteristics and Outcomes of Short-term Repeat Corneal Transplantation
Published in Ocular Immunology and Inflammation, 2022
Xiunian Chen, Ting Li, Xiaolin Qi, Weiyun Shi, Hua Gao
The second indication for keratoplasty was immune keratopathy, mainly Mooren’s ulcer and Stevens-Johnson syndrome. The incidence of Mooren’s ulcer as high as 0.03% in China,23 which may be related to the increased susceptibility to Mooren’s ulcer caused by HLADR17 or DQ2.24 Stevens-Johnson syndrome is a severe skin and mucous membrane disease that affects the whole body, including the eyes, mouth, skin, and genitalia.25 Some patients with severe condition can die of systemic infection or pulmonary problems.25 The success rate of keratoplasty in patients with Stevens-Johnson syndrome is much lower than in those with other eye diseases due to its refractory nature and tendency to recurrence. Additionally, the transplant surgery can not remove the autoantigen of patients entirely26,27 and the effect of the drugs to inhibit the recurrence remains uncertain. In China, the recurrence rates of Mooren’s ulcer were 28.28% after patients underwent LK.28 Therefore, clinicians should pay more attention to managing the autoimmune reaction in patients with immune keratopathy, such as proper administration of systemic immunosuppressants.29