Immune Reconstitution Reactions in Human Immunodeficiency Virus–Negative Patients

Scharschmidt, Tiffany C.; Amerson, Erin; Rosenberg, Oren S.; Jacobs, Richard A.; McCalmont, Timothy H.; Shinkai, Kanade

doi:10.1001/2013.jamadermatol.503

Cited by 17 publications

(11 citation statements)

References 25 publications

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“…Secondly, iatrogenic immunosuppression can arise in dermatology with the use of topical, injectable, or oral steroids and calcineurin inhibitors. IRIS most commonly follows immune recovery in HIV patients after starting highly active antiretroviral therapy; however, it can be seen in HIV-negative patients and has been documented in various case reports of pregnancy, stem cell and solid organ transplant recipients, and neutropenic patients [12]. In our case, the patient was treated with multiple courses of immunosuppressants and with each taper the eruption flared suggesting this observation may have represented a type of immune reconstitution reaction.…”

Section: Now the Question Arises What Allowed For Proliferation Of Dmentioning

confidence: 69%

Discrete and Coalescing Pustules Masking Severe Recalcitrant Rosacea due to Demodex

Wilson¹,

Ali²,

Mousdicas³

et al. 2015

J Clin Exp Dermatol Res

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We describe a clinical case concerning a 36 year old man with a recalcitrant dermatosis involving the face and neck to demonstrate how multiple pathogenic mechanisms may ultimately prohibit disease resolution. This patient's disease persisted despite multiple standard treatments for the leading differential diagnoses early in the disease course including: topical/systemic corticosteroids for an initially suspected facial dermatitis followed by minocycline and oral ivermectin for granulomatous rosacea with high Demodex burden. These failed therapies prompted the use of oral prednisone and topical pimecrolimus that resulted in some improvement but worsening flares if therapy was discontinued. The leading differential shifted toward rosacea fulminans or an unusual manifestation of immune reconstitution inflammatory syndrome (IRIS) in the setting of possible HIV or iatrogenic immunosuppression. An extensive diagnostic workup was completed and showed isolated IgM deficiency (49 mg/dl, normal range 60 to 300 mg/dl), low levels of 25 hydroxyvitamin D (15 pg/mL, normal range 18 to 64 pg/mL), and low ascorbic acid (0.3 mg/dl, normal range 0.6 to 2.0 mg/dl). The rash finally resolved following a tapering course of cyclosporine and vitamin repletion through supplements and dietary alteration. Our case is one with multiple confounding variables that may have contributed to the recalcitrant nature of this dermatosis: (1) presence of Demodex; (2) iatrogenic immunosuppression due to prolonged systemic and topical steroid use; and (3) vitamin deficiency. It is unclear exactly what role each of these factors played but the purpose of our case is to illustrate these variables can be encountered in regular practice and that sometimes the physician must explore and correct all potential vectors of pathogenesis in order to successfully treat recalcitrant dermatoses.

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Section: Now the Question Arises What Allowed For Proliferation Of Dmentioning

confidence: 69%

Discrete and Coalescing Pustules Masking Severe Recalcitrant Rosacea due to Demodex

Wilson¹,

Ali²,

Mousdicas³

et al. 2015

J Clin Exp Dermatol Res

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“…Features consistent with an infectious-driven process included active pre-transplant infection, poor immune reconstitution, and viable bacilli [ 18 ]. Nevertheless, distinguishing a predominant infectious process from an inflammatory reaction is challenging [ 45 ], since both mechanisms co-exist in the same patient during immune reconstitution. Gantzer et al [ 18 ] demonstrated a switch from a diffuse histiocytic granuloma-negative infiltration with positive AFB to a CD3-rich, AF negative, granulomatous inflammation, as immunity was restored.…”

Section: Discussionmentioning

confidence: 99%

“…While there is not enough data to establish Omenn syndrome as a risk factor for post-HSCT BCG-related complications, it is possible that the switch from a Th2 milieu in Omenn syndrome [ 46 ], known to inhibit mycobacterial immune response [ 38 ], to a Th1 milieu in the post-HSCT period increased the risk of developing IRIS. Certainly, a shift from a Th2 to Th1/Th17 predominance is known to occur in multiple settings associated with IRIS [ 44 , 45 , 47 ].…”

Section: Discussionmentioning

confidence: 99%

Bacillus Calmette–Guerin (BCG) Vaccine-associated Complications in Immunodeficient Patients Following Stem Cell Transplantation

et al. 2020

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Purpose Bacillus Calmette–Guerin (BCG) is a live attenuated vaccine with the potential of causing severe iatrogenic complications in patients with primary immunodeficiency diseases (PID) before and after hematopoietic stem cell transplantation (HSCT). We aim to investigate risk factors of post-HSCT BCG-related complications in PID patients. Methods A retrospective analysis of pediatric PID patients who had received the BCG vaccine and underwent HSCT at Hadassah-Hebrew University Medical Center, between 2007 and 2019. Results We found 15/36 (41.67%) patients who developed post-HSCT BCG-related complications. The most significant risk factor for developing BCG-related complications was T cell deficiency (47.6% of the non-complicated vs 83.3% of the BCGitis and 100% of the BCGosis groups had T cell lymphopenia, p = 0.013). None of the chronic granulomatous patients developed BCG-related manifestation post-transplant. Among T cell–deficient patients, lower NK (127 vs 698 cells/μl, p = 0.04) cell counts and NK-SCID were risk factors for ongoing post-HSCT BCGosis, as was pretransplant disseminated BCGosis (33.3% of patients with BCGosis vs none of the non-BCGosis patients, p = 0.04). Immune reconstitution inflammatory syndrome (IRIS) was observed in 3/5 patients with Omenn syndrome. Prophylactic antimycobacterial treatment was not proven effective. Conclusion BCG vaccination can cause significant morbidity and mortality in the post-transplant T cell–deficient patient, especially in the presence of pre-transplant disease. Taking a detailed medical history prior to administering, the BCG vaccine is crucial for prevention of this complication.

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“…3 The main mechanism in the pathogenesis of IRIS following HSCT is rapid restoration of T-cell numbers, and it is believed that Th1-and Th17-positive lymphocytes with proinflammatory cytokines predominate in mycobacterial IRIS. 4,5 Abu Arja et al reviewed 19 reported cases of BCG infection that had been reported in post-transplant patients with PID. [6][7][8][9][10][11][12][13][14][15] Three of them had no prior evidence of infection, yet developed either local or disseminated infection following HSCT as in our case.…”

Section: Discussionmentioning

confidence: 99%

Successful treatment of BCG‐related immune reconstitution inflammatory syndrome following ex vivo T‐cell–depleted haploidentical hematopoietic stem cell transplantation: A case report

Erbey

Dur

Atay

et al. 2019

Pediatric Transplantation

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IRIS is a phenomenon describing localized inflammatory reactions at BCG vaccination site and development of lymphadenopathy as immune system recovers. It is a rare entity in children following haploidentical HSCT. We represent the successful treatment of a case with fluctuating lymphadenopathy due to BCG vaccine during immune reconstitution period following ex vivo T‐cell–depleted haploidentical HSCT.

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Immune Reconstitution Reactions in Human Immunodeficiency Virus–Negative Patients

Cited by 17 publications

References 25 publications

Discrete and Coalescing Pustules Masking Severe Recalcitrant Rosacea due to Demodex

Discrete and Coalescing Pustules Masking Severe Recalcitrant Rosacea due to Demodex

Bacillus Calmette–Guerin (BCG) Vaccine-associated Complications in Immunodeficient Patients Following Stem Cell Transplantation

Successful treatment of BCG‐related immune reconstitution inflammatory syndrome following ex vivo T‐cell–depleted haploidentical hematopoietic stem cell transplantation: A case report

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