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The Patient with Non-Group 2 Pulmonary Hypertension
Published in Andreas P. Kalogeropoulos, Hal A. Skopicki, Javed Butler, Heart Failure, 2023
Sophia Anastasia Mouratoglou, George Giannakoulas
Certain clinical conditions, e.g., connective tissue disorders (especially scleroderma), congenital heart disease, portal hypertension, and infections (human immunodeficiency virus and schistosomiasis), are strongly associated with the development of Group 1 PH or pulmonary arterial hypertension (PAH) and patients suffering from these conditions are at risk of PAH. Drugs and other substances (e.g., anorexigens, rapeseed oil adulterated by aniline (“toxic oil syndrome,” etc.) may also lead to PAH.
Differential diagnoses of systemic sclerosis
Published in Aparna Palit, Arun C. Inamadar, Systemic Sclerosis, 2019
Eosinophilia myalgia syndrome is related to consumption of L-tryptophan and is characterized by acute or subacute onset of myalgia, arthralgia, edema of the extremities, fever, respiratory symptoms, pulmonary hypertension, arrhythmias, and polyneuropathy. A multitude of cutaneous manifestations may be present; morbilliform exanthem, urticaria, livedo reticularis, and papular mucinosis. Extensive sclerodermatous changes similar to eosinophilic fasciitis are also seen. Peripheral blood eosinophilia is present. The clinical manifestations are due to the impurities in L-tryptophan. A strikingly similar syndrome following consumption of contaminated rapeseed oil was described in Spain in 1981 (the toxic oil syndrome).42,43
Pulmonary hypertension induced by drugs and toxins
Published in Philippe Camus, Edward C Rosenow, Drug-induced and Iatrogenic Respiratory Disease, 2010
Kim Bouillon, Yola Moride, Lucien Abenhaim, Marc Humbert
In May of 1981, a large foodborne epidemic occurred in central and north-western Spain. The epidemic was characterized in the initial phase by atypical pneumonia and pronounced eosinophilia in the next few days after exposure. From May to October 1981, it affected over 20 000 people. Over 300 died of this new persistent multisystemic disorder, now known as the ‘toxic oil syndrome’ (TOS), within 8 months following the onset of the outbreak. Criteria for TOS diagnosis were defined by the Spanish Clinical Commission in August 1981.121 Epidemiological investigations established that TOS was due to the consumption of adulterated food oil, which was sold principally in unlabelled 5-litre containers by travelling salesmen. This oil was in fact a mixture containing rapeseed oil originating from France. It was denatured with 2 per cent of aniline before being imported to Spain, and was theoretically for industrial use. In Spain, it was denatured and illegally refined to remove the aniline, and finally mixed with other edible oils.
Current and emerging treatment options for autoimmune hemolytic anemia
Published in Expert Review of Clinical Immunology, 2018
Wilma Barcellini, Bruno Fattizzo, Anna Zaninoni
AIHA may be primary/idiopathic or secondary to several conditions, including infections, autoimmune diseases, drugs, malignancies, particularly lymphoproliferative disorders [6]. In this regard, cold AIHA has been recently reclassified as CAD when primary (thought to be a low-grade lymphoproliferative disorder) and cold agglutinin syndrome (CAS) if secondary (CAS, as observed in aggressive lymphoma, infections, etc.). The diagnostic workup is complex and includes the evaluation of possible confounders and other causes of hemolysis (Figure 2). Of note, about 5–10% of AIHAs remain DAT negative even after performing highly sensitive tests. In these cases, the diagnosis is made in exclusion after extensive diagnostic reevaluation, and often on the basis of a response to steroids. In secondary forms, different pathogenetic mechanisms may play a prevalent role. Molecular mimicry between self- and foreign antigens is likely the main mechanism in AIHA associated to bacterial, mycoplasma, or virus infections. Polyclonal lymphocyte activation and the emergence of forbidden clones are thought to play a major role in AIHA secondary to lymphoproliferative diseases. The generation of neoantigens by drugs clearly accounts for drug-induced AIHA [7,8], and various environmental agents have also been suggested to be responsible of autoimmunity (siliconosis, Gulf War syndrome, Spanish toxic oil syndrome, and adjuvants) [5].
Clinical guide to eosinophilic fasciitis: straddling dermatology and rheumatology
Published in Expert Review of Clinical Immunology, 2022
EF is distinguished from systemic sclerosis by the absence in EF of three typical findings in systemic sclerosis – sclerodactyly, Raynaud’s phenomenon, and nailfold capillary changes, as well presence of anticentromere, anti- topoisomerase I, and anti-RNA polymerase III antibodies. In distinction, the face, hands, and feet are spared in EF [1,3]. Morphea profunda holds clinical and histological similarities with plaque-like EF [3,5]. While isomorphic morphea occurs in areas of skin friction (not so EF), the symmetrical morphea variant is distributed on the trunk and extremities alike EF and may be confused with EF. Moreover, EF and morphea may overlap. The toxic oil syndrome, caused by ingestion of adulterated rapeseed oil 1981 in Spain, was characterized by morphea-like skin lesions affecting the face, trunk, and extremities, along with myopathy, peripheral neuropathy, and arthralgia [8]. The eosinophilia–myalgia syndrome, which had epidemic proportions in 1989 was caused by ingestion of the dietary supplement L-5-hydroxytryptophan, unfortunately contaminated. Patients complained of severe myalgias. Morphea-like lesions were conspicuous. Visceral involvement was occasionally present. Blood eosinophilia was a frequent attribute [9]. Nephrogenic systemic fibrosis is a progressive multiorgan fibrosing condition developing 2 to 75 days after exposure to gadolinium-based contrast agents used for magnetic resonance imaging. Patients with advanced renal insufficiency are at higher risk. Prominent findings on physical examination are indurated papules and plaques, on the extremities, buttocks, and trunk, sparing the face [10]. Other fibrosing disorders, easily distinguished on physical examination from EF, are myxedema, scleredema, and palmar fasciitis. Pretibial myxedema is a cutaneous manifestation of Graves’ disease presenting as yellowish-brown papules, nodules and plaques on both shins; it may be refractory to control of underlying thyroid disease. Scleredema of Buschke is characterized by stiffness and hardening of the subcutaneous tissues on the upper back and posterior surface of the neck. Palmar fasciitis is a paraneoplastic syndrome manifesting as a painful swelling of the hands, caused by inflammation and sclerosis of the palmar fascia, tendon sheaths, small joints of the fingers, and flexion contractures.