DOI: 10.1097/RHU.0b013e3182642477
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PMID: 22832303
Issn Print: 1076-1608
Publication Date: 2012/08/01
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Excerpt
We report a patient with similar features to an earlier Journal of Clinical Rheumatology report by Davis et al.,1 in which the authors described 2 patients with chronic rheumatoid arthritis (RA) who developed disseminated histoplasmosis mimicking Felty syndrome.
In December 2004, a 43-year-old woman with long-standing erosive nodular seropositive RA presented hemolytic anemia, leukopenia, and neutropenia without splenomegaly. At this point, our differential diagnosis pointed at the development of Felty syndrome because of the presence of neutropenia and hemolytic anemia. Bone marrow suppression from methotrexate was also considered and treatment with 1 mg/kg per day of prednisone in decreasing doses was initiated. In January 2005, the patient experienced episodes of epistaxis from her right nostril. A biopsy was not performed because of the patient’s refusal. In November 2005, the patient was admitted for presenting fever (temperature of 38°C) 15 days in duration. During admission, she was treated with methotrexate 10 mg/wk and chloroquine sulfate 100 mg/d. Physical examination disclosed perforation of the nasal septum and distended abdomen. She had stable chronic RA deformities without active synovitis. Laboratory tests showed hemolytic anemia (reticulocyte count, 0.056; hemoglobin, 9.2 g/dL; hematocrit, 0.29; indirect bilirubin, 1.89; lactate dehydrogenase, 324 UI/L; and positive result in the Coomb test) with a white blood cell count of 2.7 × 109/L (reference range, 3.6–10 × 109/L), an absolute neutrophil count of 1000, and erythrocyte sedimentation rate of 135 mm/hr. Levels of electrolytes, platelet count, serum creatinine, and liver-associated enzymes were all normal. Blood, urine, sputum, and stool cultures as well as throat cultures showed no development of common germs, fungi, or mycobacterium tuberculosis. Antinuclear antibody (Hep-2) titer was 1:160; levels of anti-dsDNA and anti-Ro, La, Sm, and Rnp were negative; and levels of C3-4 were normal. Ultrasound and abdominal computed tomography revealed the presence of liver and spleen enlargement and ascites. A computed tomographic scan of paranasal sinuses showed destruction of the nasal septum in the cartilaginous section. Transthoracic echocardiogram did not show abnormalities. At this moment, differential diagnosis included either development of Felty syndrome (neutropenia and splenomegaly) or sepsis from an infectious cause.
A biopsy of the nasal septum revealed yeast forms consistent with Histoplasma capsulatum. On the 14th day after admission, the patient died. The result of cultures, received subsequently, confirmed the diagnosis of histoplasmosis.
Connective tissue diseases and histoplasmosis share several clinical findings, such as fever, fatigue, chest pain, pleural effusion, diffuse pulmonary infiltrates, pericarditis, myalgias, epistaxis, arthralgias, arthritis, erythema nodosum, diffuse papules, oropharyngeal lesions, hepatosplenomegaly, lymphadenopathies, stroke syndromes, seizures, endocarditis, anemia, leukopenia, thrombocytopenia, elevations of hepatic enzymes and bilirubin, and uveítis.2–4 Opportunistic infections such as disseminated histoplasmosis can mimic other disease processes, including Felty syndrome, and are important to consider when there is a change in the clinical status of patients with rheumatic disease who are immunocompromised.
Similar to the description of Davis et al., our patient had the triad of typical Felty syndrome, high-titer seropositive RA with splenomegaly, and neutropenia. The diagnosis of this syndrome relies on excluding other potential causative factors. In our patient, the change in the clinical status was epistaxis and nasal septal perforation.
In immunocompromised patients, discriminating between rare occurrences of opportunistic infections and manifestations of autoimmune diseases is crucial. It is hence necessary to consider histoplasmosis both clinically and histopathologically.
