Received Dec 24, 2025
| Type | Name | Manufacturer | Dose | Lot | Route / Site |
|---|---|---|---|---|---|
| MEN | MENINGOCOCCAL (NO BRAND NAME) | UNKNOWN MANUFACTURER | UNK | — | OT |
Rapidly progressive interstitial lung disease; Worsening dyspnea; irregular scarring; patchy ground-glass opacities; progression in her ground-glass opacities with septal thickening; respiratory failure progressed; <p>SESSION TITLE: SESSION TYPE: Case Report PRESENTED ON: 10/20/2025 12:30 pm - 01:15 pm INTRODUCTION: Rapidly progressive interstitial lung disease (RP-ILD) is a life-threatening complication of autoimmune conditions. Among the rare yet notable culprits is clinically amyopathic dermatomyositis (CADM), particularly in the presence of anti-melanoma differentiation-associated 5 (MDA5) antibodies. This condition is marked by aggressive pulmonary decline despite the absence of significant myopathy, making early recognition challenging. The lack of a hallmark muscular phenotype combined with the disease's rapid progression, often results in delayed diagnosis, contributing to its poor prognosis. This rapid pulmonary decline dominates the clinical course, underscoring the urgency of early recognition and intervention in this vulnerable population. We present two cases of anti-MDA5 positive disease illustrating the rapid clinical decline in the absence of overt rheumatologic signs and the futility of immunosuppressive therapies when initiated late in the disease course. CASE PRESENTATION: Patient A was a 61-year-old male with a history of xylene exposure a month before presenting with dry cough and bilateral lower lobe infiltrates on chest X-ray, initially treated as pneumonia with no improvement. Chest computed tomography (CT) showed confluent lower lobe patchy bilateral infiltrates and an infectious work-up was negative. Due to worsening respiratory decline, he was placed on venovenous extracorporeal membrane oxygenation. A work-up for interstitial lung disease showed positive anti-MDA5 antibodies. Despite using steroids, cyclophosphamide, tacrolimus, intravenous immunoglobulins, and broad-spectrum antimicrobials patient's respiratory failure rapidly progressed resulting in his death.Patient B was a 44-year-old female on rituximab for neuromyelitis optica spectrum disorder. Weeks after receiving the meningococcal vaccine she experienced worsening dyspnea. Chest CT demonstrated scattered irregular scarring, architectural distortion, and patchy ground-glass opacities. Despite initial treatment with fluticasone-salmeterol and oral prednisone her dyspnea worsened, and a repeat chest CT showed progression in her ground-glass opacities with septal thickening. Despite broad-spectrum antibiotics and high-dose steroids, her respiratory status deteriorated requiring intubation. Autoimmune work-up was positive for anti-MDA5 antibodies. She was started on mycophenolate mofetil, but her respiratory failure progressed, leading to her death. DISCUSSION: Anti-MDA5 positive CADM-associated RP-ILD is associated with high mortality, as demonstrated in two fatal cases with environmental (xylene) or iatrogenic (post-vaccination) triggers. without any rheumatologic features. Despite aggressive immunosuppression and without exhibiting significant rheumatologic symptoms, both patients progressed to respiratory failure. Conversely, a recent study reported zero mortality on all cases with combined tacrolimus, cyclophosphamide, and high-dose steroids, highlighting that early anti-MDA5 antibody identification and prompt treatment initiation are critical to optimizing outcomes. Furthermore, the lack of standardization in treatment approaches is a significant issue. Despite the success rates associated with the combined use of tacrolimus, cyclophosphamide and steroids, some case reports reported the use of mycophenolate mofetil as a single agent, which has not yielded comparable outcomes. CONCLUSIONS: Given the prognosis and rapid clinical decline in patients with positive anti-MDA5 antibodies, initial testing with a full ILD panel for new onset interstitial lung disease should be considered. Early detection of this antibody will trigger aggressive treatment early in the course of the disease and could improve patient outcomes. In order to help guide management, additional studies are required given the sparsity of this condition. REFERENCE. DISCLOSURES: No relevant relationships by author No relevant relationships by author No relevant relationships by author No relevant relationships by author No relevant relationships by author No relevant relationships by author No relevant relationships by author Initial information received on 15-Dec-2025 via other healthcare professional regarding an unsolicited valid serious case issued from a Conference abstract This case involves a 44 year old female patient (patient B) who experienced rapidly progressive interstitial lung disease (interstitial lung disease) and respiratory failure progressed (respiratory failure) while receiving Meningococcal Vaccine (latency: unspecified). The patient's past medical treatment(s), vaccination(s) and family history were not provided. The patient was on rituximab for neuromyelitis optica spectrum disorder. On an unknown date, the patient received a dose of suspect Meningococcal Vaccine produced by unknown manufacturer via unknown route in unknown administration site for prophylactic vaccination. On an unknown date, weeks after receiving the meningococcal vaccine she experienced worsening dyspnea (dyspnoea). Chest Computed tomography (CT) demonstrated scattered irregular scarring (scar), architectural distortion, and patchy ground-glass opacities (lung opacity). Despite initial treatment with fluticasone-salmeterol and oral prednisone her dyspnea worsened, and a repeat chest CT showed progression in her ground-glass opacities with septal thickening (Pulmonary septal thickening). Despite broad-spectrum antibiotics and high-dose steroids, her respiratory status deteriorated requiring intubation. Autoimmune work-up was positive for anti-MDA5 antibodies. She was started on mycophenolate mofetil, but her respiratory failure progressed, leading to her death. Rapidly progressive interstitial lung disease (RP-ILD) is a life-threatening complication of autoimmune conditions. Among the rare yet notable culprits is clinically amyopathic dermatomyositis (CADM), particularly in the presence of anti-melanoma differentiation-associated 5 (MDA5) antibodies. This condition is marked by aggressive pulmonary decline despite the absence of significant myopathy, making early recognition challenging. The lack of a hallmark muscular phenotype combined with the disease's rapid progression, often results in delayed diagnosis, contributing to its poor prognosis. This rapid pulmonary decline dominates the clinical course, underscoring the urgency of early recognition and intervention in this vulnerable population. Author presented two cases of anti-MDA5 positive disease illustrating the rapid clinical decline in the absence of overt rheumatologic signs and the futility of immunosuppressive therapies when initiated late in the disease course. Anti-MDA5 positive CADM-associated RP-ILD is associated with high mortality, as demonstrated in two fatal cases with environmental (xylene) or iatrogenic (post-vaccination) triggers. without any rheumatologic features. Despite aggressive immunosuppression and without exhibiting significant rheumatologic symptoms, both patients progressed to respiratory failure. Conversely, a recent study reported zero mortality on all cases with combined tacrolimus, cyclophosphamide, and high-dose steroids, highlighting that early anti-MDA5 antibody identification and prompt treatment initiation are critical to optimizing outcomes. Furthermore, the lack of standardization in treatment approaches is a significant issue. Despite the success rates associated with the combined use of tacrolimus, cyclophosphamide and steroids, some case reports reported the use of mycophenolate mofetil as a single agent, which has not yielded comparable outcomes. It is unknown if an autopsy was done. The cause of death was reported as Respiratory failure. Information regarding batch number and expiration date corresponding to the one at time of event occurrence was requested. Seriousness Criteria: Medically significant for both events, Death for Respiratory failure and life threatening for other event Action taken: Not applicable Outcome: Not recovered for interstitial lung disease and fatal for other event Corrective treatment: fluticasone-salmeterol, prednisone, broad-spectrum antibiotics, high-dose steroids, intubation, mycophenolate mofetil for interstitial lung disease and not reported for respiratory failure. Reporter causality: related for both events; Sender's Comments: Sanofi company comment dated 23-Dec-2025: This case involves a 44-year-old female patient with neuromyelitis optica spectrum disorder receiving rituximab therapy who experienced rapidly progressive interstitial lung disease and respiratory failure following meningococcal vaccine administration. The causal role of the company suspect vaccine cannot be assessed based on the temporal relationship, as the event occurred weeks after vaccination without specification of exact latency period. The patient's underlying autoimmune condition requiring rituximab immunosuppression, positive anti-MDA5 antibodies indicating clinically amyopathic dermatomyositis, and the well-established association between anti-MDA5 positive disease and rapidly progressive interstitial lung disease with high mortality represent significant confounding factors more likely to explain pulmonary deterioration than vaccine-related adverse reaction. The progression despite aggressive immunosuppressive therapy including high-dose steroids and mycophenolate mofetil, ultimately leading to death, is consistent with natural history of anti-MDA5 positive rapidly progressive interstitial lung disease rather than vaccine-induced pathology. Further information regarding exact time to onset, vaccine manufacturer and batch details, concurrent infections or exposures, previous vaccination history, baseline pulmonary function, serial imaging timeline, rituximab dosing relative to vaccination, bronchoalveolar lavage results, lung biopsy findings, exclusion of infectious etiologies, and autopsy results are needed to fully assess this case. Based upon the reported information, the role of the individual suspect vaccine cannot be assessed in isolation given the patient's severe underlying autoimmune disease and characteristic clinical course of anti-MDA5 positive rapidly progressive interstitial lung disease.; Reported Cause(s) of Death: respiratory failure progressed
Neuromyelitis optica spectrum disorder
RITUXIMAB
Test Name: Autoimmune work-up; Test Result: Positive ; Result Unstructured Data: for anti-MDA5 antibodies; Test Name: chest CT; Result Unstructured Data: demonstrated scattered irregular scarring, architectural distortion, and patchy ground-glass opacities.; Test Name: chest CT; Result Unstructured Data: showed progression in her ground-glass opacities with septal thickening; Comments: repeat