ABSTRACT
HISTORY: A 27-year-old woman from Cameroon was admitted because of arthralgia, myalgia and severe thrombocytopenia (20,000/ micro l). She had been suffering from weakness, recurrent febrile episodes, generalized lymphadenopathy and pancytopenia for 2 years. Having a typical autoantibody constellation and fulfilling four (pleurisy, autoimmune-hemolytic anemia, antinuclear antibodies (ANA), anti-Sm antibodies) of the American College of Rheumatology (ACR) classification criteria, systemic lupus erythematosus (SLE) had been diagnosed at another hospital. Treatment with corticosteroids and azathioprine did not improve her condition. INVESTIGATIONS: Abnormal laboratory findings were pancytopenia, elevated markers of inflammation and extreme hypergammaglobulinemia (70 %) with polyclonal IgM (73 g/l). Antinuclear antibodies (ANA), anti-Sm-, anti-Scl 70-, anti-U1-RNP-, anti-histo-, anti-leukocyte- and IgM anticardiolipin antibodies were detected. A bone marrow biopsy showed polyclonal B-cell and plasma cell infiltrates. Examination of peripheral blood smears disclosed trypanosoma brucei infection. DIAGNOSIS, TREATMENT AND COURSE: After the diagnosis of stage 2 West African trypanosomiasis (sleeping sickness) specific treatment was initiated leading to subsequent remission of the disease. CONCLUSION: This case report underlines the importance of a thorough differential diagnosis in cases of suspected autoimmune disease. Induction of autoantibodies during infectious diseases may be misleading. The use of the ACR criteria for SLE must be restricted to the classification of proven connective tissue diseases.
Subject(s)
Autoantibodies/blood , Lupus Erythematosus, Systemic/diagnosis , Trypanosoma brucei brucei/isolation & purification , Trypanosomiasis, African/diagnosis , Adult , Animals , Autoantibodies/biosynthesis , Blood Protein Electrophoresis , Cameroon/ethnology , Diagnosis, Differential , Female , Hepatomegaly/diagnostic imaging , Humans , Immunoglobulin G/blood , Immunoglobulin M/blood , Lupus Erythematosus, Systemic/immunology , Pericardial Effusion/diagnostic imaging , Splenomegaly/diagnostic imaging , Trypanosoma brucei brucei/immunology , Trypanosomiasis, African/immunology , UltrasonographyABSTRACT
A 54-year old Wegener's granulomatosis patient with PR3-ANCA at diagnosis 2 years ago was admitted with a pulmonary relapse and new subglottic stenosis preceded by pulmonary infections. The patient presented with bactericidal/permeability increasing protein (BPI)-ANCA in ELISA whereas at the same time PR3-ANCA had disappeared. Bronchoalveolar lavage revealed pulmonary infection with Gram-negative bacteria. After antibiotic treatment, immunosuppression was started with cyclophosphamide and infliximab due to refractory disease. Remission was induced and BPI-ANCA disappeared. A bacterial growth inhibition assay with BPI and the patient's IgG purified during the actual pulmonary relapse showed inhibition of the antimicrobial activity of BPI in vitro, in contrast to IgG from sera taken 2 years before and after remission was induced. The patient's BPI-ANCA recognised the bioactive N-terminal portion of BPI. Thus a possible mechanism is demonstrated for how BPI-ANCA may contribute to a pro-inflammatory setting during the development of a pulmonary relapse in the absence of PR3-ANCA by impeding bacterial clearance.
