Background <p>Rheumatic fever is endemic in our part of the world. Arthritis is the most common clinical presentation and is characteristically migratory in nature. Rheumatoid-like arthritis in acute rheumatic fever is not described in literature. Although arthritis is typically treated with nonsteroidal anti-inflammatory drugs, the role of hydroxychloroquine remains less well studied.</p> Case presentation <p>A 24-year-old Pakistani female individual presented with symmetrical polyarthritis involving the small joints of the hands along with morning stiffness. She was diagnosed with rheumatic heart disease in 2011, when she initially presented with arthritis and mild mitral regurgitation. On current evaluation, her echocardiography showed worsening heart disease, with severe mitral regurgitation, moderate aortic regurgitation, moderate tricuspid regurgitation, severe pulmonary hypertension, and good biventricular systolic function. Her antistreptolysin O titer and C-reactive protein levels were raised. She was started on nonsteroidal anti-inflammatory drugs but showed no response.The patient was referred to a rheumatologist and managed with immunosuppressive drugs, including hydroxychloroquine. Her arthritis settled, the aortic regurgitation regressed, and she was discharged in stable condition following mitral valve replacement. Features favoring rheumatic arthritis in this case included the presence of rheumatic heart disease, elevated antistreptolysin O titer, and response to benzathine penicillin. Features suggestive of rheumatoid arthritis included poor response to nonsteroidal anti-inflammatory drugs and the pattern of joint involvement (symmetrical polyarthritis of the small joints of the hands with morning stiffness). Group A streptococcal infection can trigger interleukin-1 beta-mediated granulocyte-monocyte colony-stimulating factor and interferon gamma + CD4 T cell expansion, mechanisms implicated in autoimmune disorders such as rheumatoid arthritis and systemic lupus erythematosus. Similarly, granulocyte-monocyte colony-stimulating factor plays an important role in the pathogenesis of rheumatic heart disease following acute rheumatic fever. Hydroxychloroquine suppresses granulocyte-monocyte colony-stimulating factor and may therefore be effective in these patients.</p> Conclusion <p>Immunosuppressive drugs may be beneficial in treating these patients, as they behave exactly the same way as patients of other autoimmune disorders. <i>Streptococcus</i> acts as a trigger for the activation of the immune system. The clinical picture can vary depending upon the immune response activated.</p>