Table 2.

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

#	Study Year Number of patients	Sex Age	Signs and symptoms + UBA1 mutation	Comorbidities	Previous drugs	JAK-I + Concomitant drugs	Outcome Adverse effects (AEs)
1	Muratore et al. [24] 2022 One	Male 66	Fever, DVT, arthritis, dyspnea, skin involvement, chondritis + Positive	MDS with multilineage dysplasia	PRZ, MTX, Azathioprine	Upadacitinib 15 mg/day + PRZ	Complete remission AEs: none
2	Salehi et al. [25] 2023 Three	Male 72	Fever, DVT, urticaria, pancytopenia + Positive	Recurrent SIRS and IEOI, macrocytic anemia, MSGU	PRZ	Tofacitinib 5 mg BD + PRZ	Complete remission AEs: none
		Male 69	Fever, pruritus, anorexia, and weight loss, chondritis, DVT, PTE, skin involvement, elevated CRP, pancytopenia, pulmonary disease + Positive	Prostate adenocarcinoma	PRZ	Tofacitinib 5 mg BD + PRZ	Partial remission AEs: Delirium, respiratory distress, raised inflammatory markers, pancytopenia
		Male 72	Cutaneous reactions, lymphadenopathy, pancreatitis, dacryoadenitis, VTE, constitutional symptoms, progressive pancytopenia + Positive	Orbital inflammation, ILD, MDS, RBC-TDA	PRZ, MTX, AZA, MMF	Tofacitinib 5 mg BD + PRZ	Partial remission AEs: none
3	Diral et al. [26] 2024 Three	Male > 60	Cytopenia, orbital pseudotumor + NA	CCUS	GCs, CSA	Ruxolitinib + GCs	Partial remission AEs: none
		Male > 60	Cytopenia, lung inflammation, cutaneous vasculitis + NA	ICUS	GCs, TCZ	Ruxolitinib + GCs	Partial remission AEs: none
		Male > 60	Cytopenia, lung inflammation, ear and nose chondritis + NA	NA	GCs	Ruxolitinib + GCs, 5-azacitidine	Partial remission AEs: none
4	Kreutzinger et al. [27] 2024 Three	Male 60	Peripheral DVT, dyspnea, muscle weakness, Raynaud-like symptoms, fever + Positive	MDS, macrocytic hyperchromic anemia	MTX, LEF, PRZ	Ruxolitinib 20 mg BD + PRZ, azacytidine	No remission AEs: dizziness, headache, fever, constipation
		Male 70	Fever, dyspnea, pulmonary involvement, recurrent sterile parotitis, DVT + Positive	ILD, MDS	PRZ, azacitidine	Ruxolitinib 20 mg BD	Partial remission AEs: Mild decrease in Hb
		Male 80	Intermittent fever, weight loss, a history of skin rashes and polyarthritis + Positive	Polyarticular CPPD	PRZ, anakinra	Ruxolitinib 20 mg BD + PRZ	Complete remission AEs: none
5	Mishra et al. [28] 2024 Two	Male 68	Tender nonpruritic rash, recurrent fever, inflammatory arthritis + Negative	EN, CMML, macrocytic anemia	Prednisone, HCQ, MTX, MMF	Upadacitinib 15 mg daily + PRZ Switched to Ruxolitinib 10 mg BD	Partial remission with Upadacitinib; complete remission with Ruxolitinib AEs: none
		Male 77	Persistent pruritus, intermittent skin rash + Negative	CIU, BP, ACD, MDS, macrocytic anemia	OMA, PRX, RTX, naltrexone, IVM, topical (AH, CSs, AFg), NB-UVB	Upadacitinib 15–30 mg daily	Complete remission AEs: Cytopenia
6	Álamo et al. [29] 2025 Two	Male 54	Fever, night sweats, weight loss, relapsing auricular and nasal chondritis, digital ischemia, septal panniculitis, widespread folliculitis, vestibular dysfunction with sensorineural hearing loss, arthritis in both ankles + Positive	NA	CSs, anakinra,	Ruxolitinib 20 mg BD	No remission AEs: none
		Male 64	Superficial venous thrombosis, bilateral auricular and nasal chondritis, polyarthritis, bilateral proptosis + Positive	NA	CSs, MTX	Ruxolitinib 15 mg daily	No remission AEs: none
7	Costa et al. [30] 2025	Male 60	Asthenia, erythematous skin lesions, arthritis, periorbital edema, fever, weight loss + Positive	Sweet's syndrome	PRZ, Tocilizumab	Upadacitinib 15 mg daily + PRZ	Partial remission AEs: pancytopenia
8	Mizes et al. [31] 2025	Male 71	Skin involvement, chondritis, elevated CRP +Positive	Macrocytic anemia	HCQ, MMF, TCZ, colchicine, MTX, dapsone	Ruxolitinib 10 mg daily + PRZ	Complete remission AEs: none

Note: MTX: methotrexate, MDS: myelodysplastic syndrome, PRZ: prednisone, MGUS: monoclonal gammopathy, MMF: mycophenolate mofetil, PTE: pulmonary thromboendarterectomy, ICUS: idiopathic and clonal cytopenia of undetermined significance, AZA: azathioprine, CPPD: calcium pyrophosphate deposition disease, CMML: chronic myelomonocytic leukemia, GCs: glucocorticoids, CSA: cyclosporine, LEF: lefulonamide, HCQ: hydroxychloroquine, OMA: omalizumab, PRX: paroxetine, IVM: ivermectin, AH: antihistaminic, CSs: corticosteroid, AFg: antifungal, NB-UVB: narrowband ultraviolet B, and RTX: Rituximab.

Abbreviations: ACD, allergic contact dermatitis; BP, bullous pemphigoid; CCUS, clonal cytopenia of undetermined significance; CIU, chronic idiopathic urticaria; CRP, C-reactive protein; DVT, deep vein thrombosis; EN, erythema nodosum; I-EOI, ischemic end-organ injury; ILD, interstitial lung disease; RBC-TDA, red blood cell transfusion-dependent anemia; and SIRS, systemic inflammatory response syndrome.