Improving Early Diagnosis of Paraneoplastic Rheumatic Diseases to Improve Outcomes

The most effective therapy for paraneoplastic rheumatic disease is the timely detection and treatment of the underlying malignancy.

Early recognition leading to timely diagnosis is essential to improve outcomes in patients with paraneoplasias, according to German researchers. Bernard Manger, MD, professor of medicine and rheumatology at the University Hospital Erlangen, Germany, studied the importance of timely diagnosis and concluded that rheumatologists must work closely with oncologists to identify those paraneoplasias that involve pathways now treatable by new targeted therapies.

“It is important to diagnose [these paraneoplasias] in time because this can have an enormous impact on life span and quality of life for the patient. The key is knowing the clinical appearance of each paraneoplastic syndrome in order to recognize it on those rare occasions. In addition, any unusual presentation of a polyarthritis or myositis, especially [if it is] unresponsive to standard therapies such as steroids, should trigger doubts and lead to consideration about a possible underlying malignant process,” said Dr Manger.

Dr Manger relates that individual paraneoplastic syndromes, such as remitting seronegative symmetrical synovitis with pitting edema syndrome, palmar fasciitis, and polyarthritis, are rare. He states that he has seen only approximately 10 cases of hypertrophic osteoarthropathy and 2 cases of palmar fasciitis during his 30 years in clinical practice.

In an article in the journal Nature Reviews Rheumatology, Dr Manger and his colleague Georg Schett reported that, in recent years, some of the underlying mechanisms involved in paraneoplastic syndromes have been identified. Identification and understanding of these mechanisms provides insight on the physiologic and pathophysiologic roles of mediators such as vascular endothelial growth factor and fibroblast growth factor 23.1

The early diagnosis of paraneoplastic rheumatic diseases, such as hypertrophic osteoarthropathy, paraneoplastic polyarthritis, cancer-associated myositis, and tumor-induced osteomalacia, is vital. Despite the rarity of these conditions, rheumatologists must be aware of their distinct clinical patterns.

“The rheumatologist is frequently the first specialist contacted by a person with paraneoplastic musculoskeletal symptoms and is the one who usually establishes the diagnosis. After that, therapy of any underlying malignant disease, such as surgery, chemotherapy, or radiotherapy, has to be coordinated by an oncologist,” Dr Manger told Rheumatology Advisor. 

 “We certainly have learned a lot about pathophysiologic details of paraneoplastic syndromes in rheumatology. However, no new therapeutic approaches have yet arisen from this knowledge. The most effective therapy for this group of diseases still is and probably will remain in the future the timely detection of an underlying malignancy at a stage when complete removal is still possible.”