Severe type 1 upgrading leprosy reaction in a renal transplant recipient: a paradoxical manifestation associated with deficiency of antigen-specific regulatory T-cells?
Abstract Background Due to its chronic subclinical course and large spectrum of manifestations, leprosy often represents a diagnostic challenge. Even with proper anti-mycobacteria treatment, leprosy follow up remains challenging: almost half of leprosy patients may develop reaction episodes. Leprosy...
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| Format: | Article |
| Language: | English |
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BMC
2017-04-01
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| Series: | BMC Infectious Diseases |
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| Online Access: | http://link.springer.com/article/10.1186/s12879-017-2406-9 |
| _version_ | 1818878767296151552 |
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| author | Ana Paula Vieira Maria Angela Bianconcini Trindade Flávio Jota de Paula Neusa Yurico Sakai-Valente Alberto José da Silva Duarte Francine Brambate Carvalhinho Lemos Gil Benard |
| author_facet | Ana Paula Vieira Maria Angela Bianconcini Trindade Flávio Jota de Paula Neusa Yurico Sakai-Valente Alberto José da Silva Duarte Francine Brambate Carvalhinho Lemos Gil Benard |
| author_sort | Ana Paula Vieira |
| collection | DOAJ |
| description | Abstract Background Due to its chronic subclinical course and large spectrum of manifestations, leprosy often represents a diagnostic challenge. Even with proper anti-mycobacteria treatment, leprosy follow up remains challenging: almost half of leprosy patients may develop reaction episodes. Leprosy is an infrequent complication of solid organ transplant recipients. This case report illustrates the challenges in diagnosing and managing leprosy and its reactional states in a transplant recipient. Case presentation A 53-year-old man presented 34 months after a successful renal transplantation a borderline-tuberculoid leprosy with signs of mild type 1 upgrading reaction (T1R). Cutaneous manifestations were atypical, and diagnosis was only made when granulomatous neuritis was found in a cutaneous biopsy. He was successfully treated with the WHO recommended multidrug therapy (MDT: rifampicin, dapsone and clofazimine). However he developed a severe T1R immediately after completion of the MDT but no signs of allograft rejection. T1R results from flare-ups of the host T-helper-1 cell-mediated immune response against Mycobacterium leprae antigens in patients with immunologically unstable, borderline forms of leprosy and has been considered an inflammatory syndrome in many aspects similar to the immune reconstitution inflammatory syndromes (IRS). The T1R was successfully treated by increasing the prednisone dose without modifying the other immunosuppressive drugs used for preventing allograft rejection. Immunological study revealed that the patient had a profound depletion of both in situ and circulating regulatory T-cells and lack of expansion of the Tregs upon M. leprae stimulation compared to T1R leprosy patients without iatrogenic immunosuppression. Conclusions Our case report highlights that leprosy, especially in the transplant setting, requires a high degree of clinical suspicion and the contribution of histopathology. It also suggests that the development of upgrading inflammatory syndromes such as T1R can occur despite the sustained immunosuppressors regimen for preventing graft rejection. Our hypothesis is that the well-known deleterious effects of these immunosuppressors on pathogen-induced regulatory T-cells contributed to the immunedysregulation and development T1R. |
| first_indexed | 2024-12-19T14:19:25Z |
| format | Article |
| id | doaj.art-36532dc58aeb46bc87f3427c86b5f4d0 |
| institution | Directory Open Access Journal |
| issn | 1471-2334 |
| language | English |
| last_indexed | 2024-12-19T14:19:25Z |
| publishDate | 2017-04-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Infectious Diseases |
| spelling | doaj.art-36532dc58aeb46bc87f3427c86b5f4d02022-12-21T20:17:51ZengBMCBMC Infectious Diseases1471-23342017-04-011711610.1186/s12879-017-2406-9Severe type 1 upgrading leprosy reaction in a renal transplant recipient: a paradoxical manifestation associated with deficiency of antigen-specific regulatory T-cells?Ana Paula Vieira0Maria Angela Bianconcini Trindade1Flávio Jota de Paula2Neusa Yurico Sakai-Valente3Alberto José da Silva Duarte4Francine Brambate Carvalhinho Lemos5Gil Benard6Laboratory of Medical Investigation Unit 56, Division of Clinical Dermatology, Medical School, University of São PauloHealth Institute, São Paulo State Health DepartmentRenal Transplantation Service, Clinics Hospital, Medical School, University of São PauloLaboratory of Medical Investigation Unit 53, Tropical Medicine Institute, University of São PauloLaboratory of Medical Investigation Unit 56, Division of Clinical Dermatology, Medical School, University of São PauloRenal Transplantation Service, Clinics Hospital, Medical School, University of São PauloLaboratory of Medical Investigation Unit 56, Division of Clinical Dermatology, Medical School, University of São PauloAbstract Background Due to its chronic subclinical course and large spectrum of manifestations, leprosy often represents a diagnostic challenge. Even with proper anti-mycobacteria treatment, leprosy follow up remains challenging: almost half of leprosy patients may develop reaction episodes. Leprosy is an infrequent complication of solid organ transplant recipients. This case report illustrates the challenges in diagnosing and managing leprosy and its reactional states in a transplant recipient. Case presentation A 53-year-old man presented 34 months after a successful renal transplantation a borderline-tuberculoid leprosy with signs of mild type 1 upgrading reaction (T1R). Cutaneous manifestations were atypical, and diagnosis was only made when granulomatous neuritis was found in a cutaneous biopsy. He was successfully treated with the WHO recommended multidrug therapy (MDT: rifampicin, dapsone and clofazimine). However he developed a severe T1R immediately after completion of the MDT but no signs of allograft rejection. T1R results from flare-ups of the host T-helper-1 cell-mediated immune response against Mycobacterium leprae antigens in patients with immunologically unstable, borderline forms of leprosy and has been considered an inflammatory syndrome in many aspects similar to the immune reconstitution inflammatory syndromes (IRS). The T1R was successfully treated by increasing the prednisone dose without modifying the other immunosuppressive drugs used for preventing allograft rejection. Immunological study revealed that the patient had a profound depletion of both in situ and circulating regulatory T-cells and lack of expansion of the Tregs upon M. leprae stimulation compared to T1R leprosy patients without iatrogenic immunosuppression. Conclusions Our case report highlights that leprosy, especially in the transplant setting, requires a high degree of clinical suspicion and the contribution of histopathology. It also suggests that the development of upgrading inflammatory syndromes such as T1R can occur despite the sustained immunosuppressors regimen for preventing graft rejection. Our hypothesis is that the well-known deleterious effects of these immunosuppressors on pathogen-induced regulatory T-cells contributed to the immunedysregulation and development T1R.http://link.springer.com/article/10.1186/s12879-017-2406-9LeprosyType 1 reactionRenal transplantationImmunosuppressorsImmune reconstitution syndromeRegulatory T cell |
| spellingShingle | Ana Paula Vieira Maria Angela Bianconcini Trindade Flávio Jota de Paula Neusa Yurico Sakai-Valente Alberto José da Silva Duarte Francine Brambate Carvalhinho Lemos Gil Benard Severe type 1 upgrading leprosy reaction in a renal transplant recipient: a paradoxical manifestation associated with deficiency of antigen-specific regulatory T-cells? BMC Infectious Diseases Leprosy Type 1 reaction Renal transplantation Immunosuppressors Immune reconstitution syndrome Regulatory T cell |
| title | Severe type 1 upgrading leprosy reaction in a renal transplant recipient: a paradoxical manifestation associated with deficiency of antigen-specific regulatory T-cells? |
| title_full | Severe type 1 upgrading leprosy reaction in a renal transplant recipient: a paradoxical manifestation associated with deficiency of antigen-specific regulatory T-cells? |
| title_fullStr | Severe type 1 upgrading leprosy reaction in a renal transplant recipient: a paradoxical manifestation associated with deficiency of antigen-specific regulatory T-cells? |
| title_full_unstemmed | Severe type 1 upgrading leprosy reaction in a renal transplant recipient: a paradoxical manifestation associated with deficiency of antigen-specific regulatory T-cells? |
| title_short | Severe type 1 upgrading leprosy reaction in a renal transplant recipient: a paradoxical manifestation associated with deficiency of antigen-specific regulatory T-cells? |
| title_sort | severe type 1 upgrading leprosy reaction in a renal transplant recipient a paradoxical manifestation associated with deficiency of antigen specific regulatory t cells |
| topic | Leprosy Type 1 reaction Renal transplantation Immunosuppressors Immune reconstitution syndrome Regulatory T cell |
| url | http://link.springer.com/article/10.1186/s12879-017-2406-9 |
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