Table 3.

Case reports on the use of Janus kinase inhibitors for the treatment of VEXAS syndrome.

#	Study Year	Sex Age	Signs and symptoms + UBA1 mutation	Comorbidities	Previous drugs	JAK-I + Concomitant drugs	Outcome Adverse effects (AEs)
1	Kao et al. [32] 2022	Male 50	Fever, fatigue, anorexia, pulmonary disease, mild splenomegaly + Positive	PAN, cytopenia, EBV-HLH	Dapsone, colchicine, MMF, MTX	Ruxolitinib 15 mg BD + Anakinra, PRZ, RTX	PR AEs: None
2	Loschi et al. [33] 2022	Male 60	Skin lesion + Positive	Macrocytic regenerative anemia	HCQ, thalidomide	Baricitinib then Ruxolitinib + MTX, IFX, GCs, anakinra, UST, CSA	Baricitinib: NR Ruxolitinib: PR HSCT + Ruxolitinib: CR AEs: none
3	Ronsin et al. [34] 2022	Male 72	Skin lesions, low Hb and platelet, high serum creatinine, proteinuria, hematuria, leukocyturia + Positive	AKI, AIN, CAD, LCV	PRZ, anakinra	Ruxolitinib + PRZ	PR AEs: none
4	Austestad et al. [35] 2023	Male 60	Night sweats, weight, skin lesions, pain in lower extremities + Positive	ET, PTE	PRZ, MTX	Ruxolitinib 10 mg/day + PRZ, anagrelide, anakinra	PR AEs: none
5	Bindoli et al. [36] 2023	Male 65	Fever, pulmonary disease, asthenia, DVT, tenosynovitis, chondritis, macrocytic anemia, elevated inflammatory markers + Positive	Prostatectomy, LCV, DVT, MDS	M-PRZ	Filgotinib 200 mg/day + M-PRZ	PR AEs: none
6	Fahmy et al. [37] 2023	Male 66	Skin involvement + Positive	NA	Topical halobetasol, topical tacrolimus, oral doxycycline, oral minocycline, oral HCQ, oral PRZ	Tofacitinib 5 mg BD increased to 10 mg BD	CR AEs: none
7	Mohammed et al. [38] 2023	Male 73	Skin involvement, fever, night sweats, HBM + Positive	HTN	M-PRZ	Baricitinib 2 mg/day + PRZ	CR AEs: none
8	Beecher et al. [39] 2024	Male 68	Recurrent dacryoadenitis, angioedema-like lesions, jaw aches, rash, elevated laboratory markers, splenomegaly + Positive	IAP, pulmonary diseases, MDS	PRZ, MTX	Tofacitinib 5 mg BD + Azathioprine, MMF	PR AEs: None
9	Langlois et al. [40] 2024	Male 80	Fever, generalized weakness, drowsiness, weight loss, skin rash, ear chondritis, elevated CRP, macrocytic anemia, confusion, headaches, cerebellar ataxia + Positive	Ischemic stroke, CNS vasculitis	PRZ	Ruxolitinib 15 mg BD +M-PRZ, Tocilizumab	PR AEs: none
10	Wang et al. [41] 2024	Male 66	Skin involvement, macrocytic anemia, fever, general weakness, night sweats + Positive	MDS, RGD	MTX, HCQ	Ruxolitinib 10 mg BD + PRZ	PR AEs: none
11	Al-Nusair et al. [15] 2025	Male 64	Persistent anemia, weight loss, fatigue, erythematous circumferential papules, low-grade fevers, myalgia, cough, episcleritis, chondritis + Positive	NA	PRZ	Ruxolitinib 10 mg daily + PRZ, insulin???	CR AEs: none
12	Kelly et al. [42] 2025	Male 63	Lymphoid hyperplasia, splenomegaly, migratory arthralgia, multifocal PTE, DVT, pancytopenia, recurrent oral and genital ulceration + Positive	Multifocal-PG, MDS, lobular panniculitis, neutrophilic vasculitis, HTN, GERD	PRZ, pantoprazole, rivaroxaban, telmisartan, metoprolol, alendronate, vitamin D, doxycycline, sulfamethoxazole, trimethoprim, MMF, MTX, AZA, IFX, adalimumab, Tocilizumab	Ruxolitinib	NR AEs: none
13	Lechtenboehmer et al. [17] 2025	Male 83	Visual field defect, fever, night sweats, arthritis, pneumonitis, chondritis + Positive	NA	GCs	Ruxolitinib 15 mg BD + Dexamethasone, bevacizumab	PR AEs: none

Note: MMF: mycophenolate mofetil, PAN: polyarteritis nodosa, EBV-HLH: Epstein–Barr virus-related hemophagocytic lymphohistiocytosis, MTX: methotrexate, PTE: pulmonary thromboembolism, MDS: myelodysplastic syndrome, HBM: hypercellular marrow, LCV: leukocytic vasculitis, PRZ: prednisone, RTX: Rituximab, HCQ: hydroxychloroquine, IFX: Infliximab, GCs: glucocorticoids, UST: Ustekinumab, CSA: cyclosporine, M-PRZ: methylprednisolone, HTN: hypertension, AZA: azathioprine, GCs: glucocorticoids, and GERD: gastroesophageal reflux disease.

Abbreviations: AIN, acute interstitial nephritis; AKI, acute kidney injury; CAD, coronary artery disease; CNS, central nervous system; CR, complete response; CRP, C-reactive protein; ET, essential thrombosis; HSCT, hematopoietic stem cell transplantation; IAP, idiopathic autoimmune pancreatitis; NR, no response; PR, partial response; PG, Pyoderma gangrenosum; and RGD, reactive granulomatous dermatitis.