Rhupus; unusual presentations.

ABSTRACT

BACKGROUND: The coexistence of systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) named Rhupus is an unusual clinical condition. Previous reports mentioned that Rhupus patients have prominent RA-associated clinical manifestations and only mild organic damage related to SLE. Progressive or life-threatening manifestations are rare in Rhupus patients. METHODS: Three patients with Rhupus are described in this article. Two of them presented antiphospholipid syndrome (APS) in addition to Rhupus. Also, we searched for similar cases in published literature. RESULTS: We present three patients with Rhupus syndrome. One of the patients has only Rhupus, the second patient has Rhupus and APS, and the third patient has Rhupus accompanied by severe Raynaud's syndrome with digital ulcers, APS, pulmonary hypertension and two malignancies. Several studies have shown that Rhupus patients have an increased prevalence of positive antiphospholipid antibodies that resembles SLE. However, the presence of these antibodies is not associated with APS. There is only one case of Rhupus with secondary APS in which the patient presented headache and papilloedema due to cerebral venous thrombosis. Secondary Raynaud's syndrome is rare in Rhupus patients, and to the best of our knowledge, only three cases of this are mentioned in literature. Secondary pulmonary hypertension and malignancies were never reported before in Rhupus patients. CONCLUSIONS: Rheumatologists should be aware of the possibility that Rhupus may be accompanied by progressive or life-threatening conditions such as APS, severe Raynaud's syndrome with digital ulcers, pulmonary hypertension, or malignancies.