Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0034069 (pulmonary fibrosis)
 7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An autopsy case of a 70-year-old woman with sarcoidosis followed up for 16 years is reported. She was admitted for examination of bilateral hilar lymphadenopathy on chest X-ray film and uveitis in 1971. Sarcoid granuloma was revealed by Daniels lymph node biopsy. After steroid therapy, bilateral hilar lymphadenopathy and uveitis disappeared. However, small nodular shadows appeared in bilateral lower lung fields on chest X-ray film in 1973. Gradually small nodular shadows increased and reticular shadows appeared. In 1980, chest X-ray film revealed fibrotic change in the bilateral lung fields and giant bulla in the left upper lung field. She developed acute pneumonia and died of acute progression of chronic respiratory failure in 1987. Autopsy revealed sarcoid granulomas and honeycomb lesions in bilateral lungs and also silicotic nodules near the sarcoid granuloma in the upper lobes. This case suggests that inhaled mineral particles may play an important role in the development of pulmonary fibrosis in sarcoidosis.
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PMID:[A long-term follow-up case of sarcoidosis with fibrosis and silicotic nodules in the lung]. 206 60

Thirty-eight patients with advanced Hodgkin disease were treated with a combination of cyclophosphamide, vinblastine, procarbazine, and prednisone (CVPP) from 1970 to 1973 and followed prospectively. Long-term results after a median follow-up of 14 years are reported. Seventeen of the 28 complete responders (61%) survived more than 10 years from the initiation of chemotherapy. At the current time, 12 of the 28 patients (43%) are continuously disease-free 12.8 to 15.3 years after completing induction chemotherapy. Two additional patients are alive in third and fifth remissions. All relapses occurred within 5.5 years of completing induction chemotherapy. Late complications included sterility, aseptic osteonecrosis, severe pulmonary fibrosis, and chronic uveitis. Four of the complete responders (14%) developed second neoplasms, including acute myelogenous leukemia, non-Hodgkin lymphoma and small cell carcinoma of the lung. All second malignancies were fatal and developed 5-13 years after initiation of induction chemotherapy. Our data confirm that cure is possible with alternative regimens to MOPP (nitrogen mustard, vincristine, procarbazine, and prednisone).
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PMID:Chemotherapy with cyclophosphamide, vinblastine, procarbazine, and prednisone (CVPP) for Hodgkin disease: fourteen-year follow-up results. 341 72

Sarcoidosis is a multisystem granulomatous disorder that most often affects the lungs and may cause significant morbidity. Sarcoidosis can manifest as neurological disease, uveitis, blindness, end-stage pulmonary fibrosis, pulmonary hypertension, dysrhythmias, cardiomyopathy, hypercalcemia, and renal failure. Sarcoidosis persists as chronic disease in approximately one-third of those affected. Clinical pitfalls and misconceptions about the course of disease place this population at risk for delayed or inadequate care. While noncaseating granulomas are the histopathological hallmark of sarcoidosis, they also are nonspecific. No pathognomonic diagnostic test exists for sarcoidosis, so the diagnosis remains one of exclusion. While the etiology of sarcoidosis is still unknown, recent insights into its immunopathogenesis have moved investigators closer to finding more effective treatments. Corticosteroids remain the standard of care when treatment is indicated, despite their adverse effect profile. Clinical investigations of novel drugs and biological agents targeting mechanisms involving CD4 type 1 helper T cells may provide more effective, better tolerated therapies.
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PMID:Sarcoidosis: clinical presentation, immunopathogenesis, and therapeutics. 2154 Apr 17

Clinical outcomes for patients with advanced melanoma have improved significantly with the introduction of immune checkpoint inhibitors. These agents have distinct adverse effects with the potential for heightened host immune responses manifesting as an autoimmune reaction in any organ. We report a unique case who developed pembrolizumab induced arthritis, ocular hypotony with vision loss and pulmonary interstitial fibrosis. A 57-year old gentleman with advanced melanoma was treated with pembrolizumab and attained complete response with no evidence of disease on functional imaging. Treatment was well-tolerated with the only side effect being arthritis controlled with low dose steroids. Following a work related blunt trauma to the right eye, the patient developed bilateral visual impairment secondary to ocular hypotony. The ocular hypotony failed to respond to high-dose glucocorticoid and multiple surgeries. Intraoperatively, ciliary body atrophy was found. Pembrolizumab was ceased after the eye trauma and he remained in complete remission from melanoma. After a further 10 months, the patient developed symptomatic pulmonary fibrosis. There was moderate symptomatic improvement with nintedanib, an antifibrotic agent. This case describes two rare and unique adverse effects. Ocular adverse effects are extremely uncommon and this is the first case to report immune checkpoint inhibitor related ocular hypotony without uveitis to the best of our knowledge. Similarly, the incidence of severe pneumonitis is reported to be low, however limited data is available regarding pulmonary interstitial fibrosis. The occurrence of multiple adverse effects in this case including one occurring several months after cessation of treatment highlights the need for vigilance by clinicians who manage patients treated with immune checkpoint inhibitors. Further research is necessary with regards to rare adverse effects of immune checkpoint inhibitors and the relation of these to treatment administration.
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PMID:Pembrolizumab Induced Ocular Hypotony With Near Complete Vision Loss, Interstitial Pulmonary Fibrosis and Arthritis. 3160 34