QJM: An International Journal of Medicine, 2015, 969–970 doi: 10.1093/qjmed/hcv080 Advance Access Publication Date: 10 April 2015 Case report
CASE REPORT
Septic arthritis and smoldering myeloma A. Silawy1, G. Lavie1,2, S. Perek1,2, D. Zisman2,3, G. Weber2,4, M. Preis2,5 and S. Cohen1,2 From the 1Division of Internal Medicine, Lady Davis Carmel Medical Center, Haifa, Israel, 2Ruth and Bruce Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel, 3Rheumatic Diseases Unit, Lady Davis Carmel Medical Center, Haifa, Israel, 4Infectious Diseases Unit, Lady Davis Carmel Medical Center, Haifa, Israel, and 5Institute of Hematology, Lady Davis Carmel Medical Center, Haifa, Israel Address correspondence to A. Silawy, Division of Internal Medicine, Lady Davis Carmel Medical Center, Haifa, Israel. email: [email protected]
Pneumococcus is a rare causative agent of septic arthritis. The diagnosis of pneumococcal septic arthritis should direct further investigation of systemic immunosuppression. Acquired immunodeficiency including lymphoproliferative disorders and myeloma should be considered in patients with invasive pneumococcal infection, especially in musculoskeletal infections.
Case report A 52-year-old woman presented with 5 days history of fever with chills and severe pain of the left wrist and shoulders. Physical examination was notable for swollen left wrist and painful right acromioclavicular joint. Laboratory evaluation revealed elevated inflammation markers [C-reactive protein 39 mg/dl (0–0.5 mg/dl)]. Auto-antibodies against antinuclear, rheumatoid factor, and Anti-cyclic citrullinated peptide were negative. Synovial fluid analysis demonstrated an elevated leukocyte count. Blood and joint fluid cultures were positive for Pneumococcus. The patient was treated with Ceftriaxone intravenously under the diagnosis of Pneumococcal oligoarthritis. Transthoracic echocardiography ruled out vegetations. Chest and abdominal CT followed by magnetic resonance imaging showed findings compatible with septic arthritis of the right acromioclavicular joint and mediastinal microabscesses with involvement of the pectoralis major and the right psoas muscle (Figure 1). Whole-body CT exam showed intact spleen and did not identify obvious solid malignancy, HIV serology was negative. Quantitative immunoglobulins level demonstrated elevated IgG levels (2700 mg/dl); and low levels of IgM and IgA (35 and
44 mg/dl, respectively) with monoclonal peak of IgG k type (2100 mg/dl). Bone marrow biopsy demonstrated clonal plasma cell infiltration of 30%. Since the patient did not have any evidence for symptomatic myeloma, a diagnosis of IgG k smoldering myeloma was made. The patient was treated with IV antibiotics for 6 weeks and was followed by serial imaging studies as an outpatient. Her clinical condition improved progressively with complete resolution of her symptoms. In the face of her low polyclonal immunoglobulins level, we evaluated the ability of her humoral system to produce specific antibodies. The patient was vaccinated with pneumococcal and tetanus–diphtheria vaccines. Indeed, both vaccinations yielded suboptimal-specific antibody titres as measured 2 months after vaccination.
Discussion Our case report describes an apparently healthy woman who presented with invasive infection—pneumococcal oligoarthritis, bacteraemia and soft tissue infection. Pneumococcus is the causative agent of only 6% of cases of septic arthritis. Approximately one-third of pneumococcal septic arthritis cases in adults are oligo/polyarticular.1–3 The major risk factors for pneumococcal arthritis in adults include rheumatoid arthritis, prosthetic joints and splenectomy. Invasive pneumococcal disease in a healthy adult should lead to further investigation for an underlying disease including primary immunodeficiencies, but also acquired immunodeficiency including—HIV, lymphoproliferative disorders and specifically multiple myeloma.1–4 Recent studies identified genetic susceptibility for invasive Pneumococcal infections in adults. Genetic variation in control of the proinflammatory transcription factor NF-kB appears to play a key role in host defence against pneumococcal disease.5
C The Author 2015. Published by Oxford University Press on behalf of the Association of Physicians. V
All rights reserved. For Permissions, please email: [email protected]
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QJM: An International Journal of Medicine, 2015, Vol. 108, No. 12
Figure 1: (a) Chest CT revealing infiltration in the right acromioclavicular joint. (b) Abdominal CT demonstrating infiltration of the right psoas muscle.
Conclusion Though the presentation of myeloma as an infectious episode is rare, this entity should be ruled out in front of an invasive pneumococcal infection without an obvious predisposing disease.
Conflict of interest: None declared.
References 1. Sumrall A, Muzny C, Bell J, Dreiling B. Pneumococcal septic arthritis as the initial presentation of multiple myeloma. Int J Lab Hematol 2008; 30:82–3. 2. Riachy MA. Streptococcus pneumoniae causing septic arthritis with shock and revealing multiple myeloma. BMJ Case Rep 05/2011; 2011. DOI: 10.1136/bcr.12.2010.3664. 3. Costa DB, Shin B, Cooper DL. Pneumococcemia as the presenting feature of multiple myeloma. Am J Hematol 2004; 77: 277–81. 4. Chapman SJ, Khor CC, Vannberg FO, Rautanen A, Walley A, Segal S, et al. Common NFKBIL2 polymorphisms and susceptibility to pneumococcal disease: a genetic association study. Crit Care 2010; 14:R227. 5. Renou F, Gerber A, Moiton MP, Ferrandiz D, Yvin JL. Pneumococcal septic arthritis revealing a multiple myeloma. Rev Med Intern 2007; 28:186–7. 6. Cuesta M, Bernad M, Espinosa A, Herranz P, Martin ME, Gijo´n BJ. Pneumococcal septic arthritis as the first manifestation of multiple myeloma. Clin Exp Rheumatol 1992; 10:483–4.
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There is a known association of multiple myeloma with a diverse array of infections, the mostly common found organisms are polysaccharide encapsulated bacteria, especially Pneumococcus.1,6 However, acute infection as the initial presentation of myeloma is uncommon. Pneumococcaemia, especially with musculoskeletal infection, should raise the suspicion of an underlying myeloma and should prompt further investigations.1,2,5 Patients with plasma cell dyscrasia often fail to adequately respond to pneumococcal vaccination. Therefore, patients with plasma cell dyscrasia and history of severe infection will be considered for prophylactic Immunoglobulin (IVIG) therapy.2,6 Treatment for the underlying smoldering (asymptomatic) myeloma is still a matter of controversy. Although limited data from recent trials demonstrated that high risk patients may benefit from treatment, the current recommendations are to continue to follow these patients and to treat the smoldering myeloma only in the context of clinical trial. After 6 months of follow-up, our patient continues to do well with no recurrence and no evidence of progression to symptomatic myeloma.
CASE REPORT
Septic arthritis and smoldering myeloma A. Silawy1, G. Lavie1,2, S. Perek1,2, D. Zisman2,3, G. Weber2,4, M. Preis2,5 and S. Cohen1,2 From the 1Division of Internal Medicine, Lady Davis Carmel Medical Center, Haifa, Israel, 2Ruth and Bruce Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel, 3Rheumatic Diseases Unit, Lady Davis Carmel Medical Center, Haifa, Israel, 4Infectious Diseases Unit, Lady Davis Carmel Medical Center, Haifa, Israel, and 5Institute of Hematology, Lady Davis Carmel Medical Center, Haifa, Israel Address correspondence to A. Silawy, Division of Internal Medicine, Lady Davis Carmel Medical Center, Haifa, Israel. email: [email protected]
Pneumococcus is a rare causative agent of septic arthritis. The diagnosis of pneumococcal septic arthritis should direct further investigation of systemic immunosuppression. Acquired immunodeficiency including lymphoproliferative disorders and myeloma should be considered in patients with invasive pneumococcal infection, especially in musculoskeletal infections.
Case report A 52-year-old woman presented with 5 days history of fever with chills and severe pain of the left wrist and shoulders. Physical examination was notable for swollen left wrist and painful right acromioclavicular joint. Laboratory evaluation revealed elevated inflammation markers [C-reactive protein 39 mg/dl (0–0.5 mg/dl)]. Auto-antibodies against antinuclear, rheumatoid factor, and Anti-cyclic citrullinated peptide were negative. Synovial fluid analysis demonstrated an elevated leukocyte count. Blood and joint fluid cultures were positive for Pneumococcus. The patient was treated with Ceftriaxone intravenously under the diagnosis of Pneumococcal oligoarthritis. Transthoracic echocardiography ruled out vegetations. Chest and abdominal CT followed by magnetic resonance imaging showed findings compatible with septic arthritis of the right acromioclavicular joint and mediastinal microabscesses with involvement of the pectoralis major and the right psoas muscle (Figure 1). Whole-body CT exam showed intact spleen and did not identify obvious solid malignancy, HIV serology was negative. Quantitative immunoglobulins level demonstrated elevated IgG levels (2700 mg/dl); and low levels of IgM and IgA (35 and
44 mg/dl, respectively) with monoclonal peak of IgG k type (2100 mg/dl). Bone marrow biopsy demonstrated clonal plasma cell infiltration of 30%. Since the patient did not have any evidence for symptomatic myeloma, a diagnosis of IgG k smoldering myeloma was made. The patient was treated with IV antibiotics for 6 weeks and was followed by serial imaging studies as an outpatient. Her clinical condition improved progressively with complete resolution of her symptoms. In the face of her low polyclonal immunoglobulins level, we evaluated the ability of her humoral system to produce specific antibodies. The patient was vaccinated with pneumococcal and tetanus–diphtheria vaccines. Indeed, both vaccinations yielded suboptimal-specific antibody titres as measured 2 months after vaccination.
Discussion Our case report describes an apparently healthy woman who presented with invasive infection—pneumococcal oligoarthritis, bacteraemia and soft tissue infection. Pneumococcus is the causative agent of only 6% of cases of septic arthritis. Approximately one-third of pneumococcal septic arthritis cases in adults are oligo/polyarticular.1–3 The major risk factors for pneumococcal arthritis in adults include rheumatoid arthritis, prosthetic joints and splenectomy. Invasive pneumococcal disease in a healthy adult should lead to further investigation for an underlying disease including primary immunodeficiencies, but also acquired immunodeficiency including—HIV, lymphoproliferative disorders and specifically multiple myeloma.1–4 Recent studies identified genetic susceptibility for invasive Pneumococcal infections in adults. Genetic variation in control of the proinflammatory transcription factor NF-kB appears to play a key role in host defence against pneumococcal disease.5
C The Author 2015. Published by Oxford University Press on behalf of the Association of Physicians. V
All rights reserved. For Permissions, please email: [email protected]
969
Downloaded from by guest on March 23, 2016
Learning points for clinicians
970
|
QJM: An International Journal of Medicine, 2015, Vol. 108, No. 12
Figure 1: (a) Chest CT revealing infiltration in the right acromioclavicular joint. (b) Abdominal CT demonstrating infiltration of the right psoas muscle.
Conclusion Though the presentation of myeloma as an infectious episode is rare, this entity should be ruled out in front of an invasive pneumococcal infection without an obvious predisposing disease.
Conflict of interest: None declared.
References 1. Sumrall A, Muzny C, Bell J, Dreiling B. Pneumococcal septic arthritis as the initial presentation of multiple myeloma. Int J Lab Hematol 2008; 30:82–3. 2. Riachy MA. Streptococcus pneumoniae causing septic arthritis with shock and revealing multiple myeloma. BMJ Case Rep 05/2011; 2011. DOI: 10.1136/bcr.12.2010.3664. 3. Costa DB, Shin B, Cooper DL. Pneumococcemia as the presenting feature of multiple myeloma. Am J Hematol 2004; 77: 277–81. 4. Chapman SJ, Khor CC, Vannberg FO, Rautanen A, Walley A, Segal S, et al. Common NFKBIL2 polymorphisms and susceptibility to pneumococcal disease: a genetic association study. Crit Care 2010; 14:R227. 5. Renou F, Gerber A, Moiton MP, Ferrandiz D, Yvin JL. Pneumococcal septic arthritis revealing a multiple myeloma. Rev Med Intern 2007; 28:186–7. 6. Cuesta M, Bernad M, Espinosa A, Herranz P, Martin ME, Gijo´n BJ. Pneumococcal septic arthritis as the first manifestation of multiple myeloma. Clin Exp Rheumatol 1992; 10:483–4.
Downloaded from by guest on March 23, 2016
There is a known association of multiple myeloma with a diverse array of infections, the mostly common found organisms are polysaccharide encapsulated bacteria, especially Pneumococcus.1,6 However, acute infection as the initial presentation of myeloma is uncommon. Pneumococcaemia, especially with musculoskeletal infection, should raise the suspicion of an underlying myeloma and should prompt further investigations.1,2,5 Patients with plasma cell dyscrasia often fail to adequately respond to pneumococcal vaccination. Therefore, patients with plasma cell dyscrasia and history of severe infection will be considered for prophylactic Immunoglobulin (IVIG) therapy.2,6 Treatment for the underlying smoldering (asymptomatic) myeloma is still a matter of controversy. Although limited data from recent trials demonstrated that high risk patients may benefit from treatment, the current recommendations are to continue to follow these patients and to treat the smoldering myeloma only in the context of clinical trial. After 6 months of follow-up, our patient continues to do well with no recurrence and no evidence of progression to symptomatic myeloma.
