Tumor syndrome, hepatic and hematological features revealing undiagnosed early rheumatoid arthritis

Background: Extra-articular manifestations in rheumatoid arthritis (RA) are rare and generally occur in an advanced stage of the disease. We report a case of an unusual onset of early RA. Case presentation: A 21 year old male complains of polyathritis involving small and large joints, associated with fever, asthenia, anorexia and weight loss. The temperature was up to 38.8°. There was a left cervical adenopathies, hepato-splenomegaly and there were synovitis in all joints sparing distal interphalangial joints. The erythrocyte sedimentation rate was accelerated; the C-reactive protein was up to 222 mg/l. The number of white blood cells were 9000/mm3 with monocytosis, lymphocytosis and thrombocytosis at 986000/mm3 . There was cholestasis with cytolysis. Rheumatoid factor (RF) and anti-cyclic citrullinated peptide antibodies (ACCP) were positive. The hands X-ray didn’t show any RA specific signs. The chest X-Ray showed a left pleurisy. The CT scan showed multiple axillar and mediastinal lymphadenopathies, hepato-splenomegaly with multiple coelio-mesenteric and retroperitoneal lymph nodes. The pleural biopsy as well as the biopsy of the adenopathy and the salivary glands didn’t show any specific abnormalities or signs of lymphoma. The hepatic biopsy revealed an inflammatory lymphohistiocytic infiltrate with rare foci of necrosis and a dilatation of the hepatic sinusoids. The diagnosis of RA was suspected and confirmed by the MRI of the hands. Conclusion: RA may have an unusual onset such as hematologic, hepatic, pleuropulmonary features or tumor syndrome, but it is rarely reported. The diagnosis in this case is difficult