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Scleroderma
Published in Charles Theisler, Adjuvant Medical Care, 2023
Symptoms may include generalized hardening, thickening, and tightening of the skin, or localized thickening over the skin on the face and fingers with telangiecta-sis, stiffness, feeling tired, poor blood flow to the fingers or toes with cold exposure (Raynaud's phenomenon), difficulty swallowing (esophageal dysmotility), and GERD. The most common early symptoms are Raynaud's and swelling of the distal extremities with thickening of the skin on the fingers of both hands. About 90% of systemic scleroderma patients will develop GI tract involvement during the course of the disease with the most common GI manifestation being gastroesophageal reflux disease (GERD). Interstitial lung disease and renal crisis are the major complications.2 There is no cure for scleroderma and treatment is aimed at improving skin appearance and reducing tightness while limiting organ damage.
Antibody-Based Therapies
Published in David E. Thurston, Ilona Pysz, Chemistry and Pharmacology of Anticancer Drugs, 2021
The main adverse events associated with cetuximab are skin reactions including skin rash, acne, pruritus, dry skin, desquamation, hypertrichosis, and nail disorders. However, nausea, vomiting, diarrhea, abdominal pain, headache, asthenia, malaise, fever, urticaria, airway obstruction, hypotension, shock, and infusion reaction are also common. Caution is required in patients with cardiopulmonary and/or pulmonary disease, and treatment should be discontinued if interstitial lung disease develops. It should only be used in pregnancy if benefits outweigh risks, although little data on toxicity to the fetus is available.
Interstitial Lung disease In Childhood Rheumatic Disorders
Published in Lourdes R. Laraya-Cuasay, Walter T. Hughes, Interstitial Lung Diseases in Children, 2019
Acute pneumonitis is characterized by the sudden appearance of pulmonary infiltrates and pleural effusions, and occurs in the setting of active lupus. It is often accompanied by fever, cough, chest pain, and tachypnea. Chronic interstitial lung disease, however, may occur with or without symptoms. The most common symptom is dyspnea on exertion. Physical findings associated with chronic interstitial disease may include poor diaphragmatic movement and basilar rales. Cyanosis and clubbing may occur. Radiographic findings include diffuse or localized infiltrates, plate-like atelectasis, unilateral elevation of the diaphragm, and small pleural effusions. When biopsied, early lesions may show inflammatory changes. Later lesions show thickening of alveolar walls with fibrous tissue, plasma cell infiltration of interstices, histiocyte desquamation, and necrosis of alveoli and bronchioles.3,6
JAK inhibitors in rheumatology
Published in Immunological Medicine, 2023
Some other autoimmune rheumatic diseases (AIRDs) have shown promise for JAK inhibitors treatment. Owing to the mechanism of JAKis inhibiting IL-6 signaling pathway, diseases that has been suggested with IL-6 inhibition has been studied. Efficacy in large vessel vasculitis is anticipated with baricitinib and upadacitinib in phase II and phase III study respectively. Refractory giant cell arteritis patients were treated with baricitinib in an open-label phase II study. Only 1 patient out of 14 patients relapsed with other 13 patients achieving glucocorticoid free remaining in disease remission during the 52-week study [56]. Phase III clinical trials with upadacitinib for treating giant cell arteritis and Takayasu arteritis are underway. Tofacitinib demonstrated tolerability in systemic sclerosis (SSc) patients in a phase I/II trial with no increase in severe AEs [57]. A systematic literature review of observational studies reported that treatment with a JAK inhibitor resulted in significant improvement of skin lesions in 52/59 (88%) of SSc patients [58]. Additionally in this report, worsening of interstitial lung disease (ILD) was avoided in 28/29.
Rapid resolution of refractory hypoxemia and vascular spiders following liver transplantation
Published in Canadian Journal of Respiratory, Critical Care, and Sleep Medicine, 2022
Allison Love, Rachel Jen, Lindsay Van Tongeren, C. Francis Ryan
A 65-year-old man was admitted for assessment for possible lung transplantation to manage severe refractory hypoxemia, platypnea and orthodeoxia, suspected to be due to hereditary hemorrhagic telangiectasia (HHT). He had a previous diagnosis of mild unclassifiable interstitial lung disease based on findings on chest CT imaging and review at multidisciplinary interstitial lung disease rounds. He also had mild centrilobular and paraseptal emphysema. He had a 45-pack-year smoking history prior to quitting 10 years previously. Pulmonary function testing showed forced vital capacity (FVC) 5.54 L (112% predicted), forced expiratory volume in 1 second (FEV1) 4.10 L (104% predicted), post-bronchodilator FEV1/FVC 0.74 with no significant bronchodilator response, and normal flow-volume loop. Lung volumes were normal with total lung capacity of 105% predicted and residual volume of 102% predicted. Diffusing capacity of lung for carbon monoxide (DLCO) was 64% predicted.
Safety and effectiveness of ramucirumab and docetaxel: a single-arm, prospective, multicenter, non-interventional, observational, post-marketing safety study of NSCLC in Japan
Published in Expert Opinion on Drug Safety, 2022
Yucherng Chen, Soshi Nagaoka, Taeko Katayose, Nobuyuki Sekine
The proportion of patients with SAEs was 31.7% (n = 126). The most common SAEs were febrile neutropenia (n = 28, 7.0%), neutrophil count decrease (n = 18, 4.5%), neutropenia (n = 14, 3.5%), pneumonia (n = 10, 2.5%), interstitial lung disease (n = 9, 2.3%), and decreased appetite (n = 5, 1.3%). AEs that resulted in death were interstitial lung disease (two patients), pneumonia, hypoxic-ischemic encephalopathy, acute respiratory distress syndrome, and pulmonary hemorrhage (one patient each). These AEs were considered related to ramucirumab, except for the case of hypoxic-ischemic encephalopathy in which the patient had preceding grade 4 aspiration. Regarding the two cases of interstitial lung disease, one patient (aged 79 years) had complications that included idiopathic pulmonary fibrosis, chronic obstructive pulmonary disease, and emphysema. The other patient was 62 years old with a history of radiation pneumonitis, a possible factor (other than ramucirumab and docetaxel) contributing to the occurrence of interstitial lung disease that resulted in death. The patient with acute respiratory distress syndrome was 79 years old with a preceding CTCAE grade 3 hemorrhagic duodenal ulcer, which was determined to be related to ramucirumab. Finally, the patient with pulmonary hemorrhage was 50 years old with a history of atrial fibrillation and was receiving anticoagulant therapy during ramucirumab and docetaxel treatment.