Table I.
Patient demographics and disease characteristics
Patient | 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 |
---|---|---|---|---|---|---|---|---|
Age, y | 34 | 42 | 62 | 59 | 55 | 71 | 67 | 45 |
Sex | Female | Female | Female | Female | Female | Female | Female | Female |
Race | White | Hispanic/Latino | Black | White | White | White | White | White |
Overlap disease timing | 2020: SCLE/SLE 2022: CADM |
2001: ACLE/SLE 2021: CDM |
2007: DLE 2021: SCLE and CDM |
1980s: SCLE/SLE 2016: CDM |
2007: ACLE/SLE 2018: CDM |
2015: CDM 2020: SCLE |
1997: ACLE/SLE 2007: CDM |
2006: ACLE/SLE 2019: SCLE 2020: CADM |
Overlap disease triggers | N/A | DM rash after COVID-19 infection and 9 mo later, muscle symptoms after bivalent vaccine | DM after first COVID-19 vaccine | DM rash after starting adalimumab∗ and 6 y later, muscle symptoms after COVID-19 infection | N/A | SCLE after receiving RTX† | N/A | N/A |
Other concomitant autoimmune diseases | N/A | APLS, Relapsing polychondritis | Scleroderma | Rheumatoid arthritis, secondary Sjogren’s syndrome | Sjogren’s syndrome | N/A | Sjogren’s syndrome | Cutaneous vasculitis, Sjogren’s syndrome |
Clinical manifestations | ||||||||
Lupus | Erythematous papules on arms and trunk, photosensitivity, arthritis | Malar rash, serositis, arthritis, Raynaud’s phenomenon, oral ulcers | Hyperkeratotic and atrophic papules in conchal bowls, hyperpigmented macules and patches on arms, legs, and back | Photoexposed annular lesions, seizures, fatigue, low-grade fevers, polyarthritis | Photosensitive rash, alopecia, oral/nasal ulcers, arthritis, serositis, Raynaud’s phenomenon, lymphopenia | Erythematous plaques on the back, arms, and chest | Photosensitive rash, oral ulcers, fatigue, arthralgias, Raynaud’s phenomenon, anemia | Malar rash, alopecia, oral ulcers, migraine, nephritis, arthritis, erythematous scaly papules on chest, back, arms, and hairline, thrombocytopenia |
DM | Malar erythema, Gottron’s sign, itchy rash on arms | Periorbital erythema and edema, malar erythema, Gottron’s sign, dysphagia, myalgia | Erythematous V-of the neck, arms, abdomen, and back, myalgia, proximal weakness | Scaly erythema on the scalp with alopecia, erythema on ears, arms, and knees, Gottron’s sign, periungual erythema with nailfold capillary changes, myalgia, proximal weakness, ILD | Periorbital lichenification, erythema over elbows, Gottron’s sign, proximal nailfold changes, myalgias | Erythema and scale on the scalp with thinning of hair, facial erythema, dysphagia, proximal weakness | Erythema on hands, elbows, hips, and shoulders, Gottron’s sign/papules, papules on elbows, mechanic’s hands, proximal weakness | Periorbital erythema, Gottron’s sign, proximal nailfold changes |
Complete topical treatment history | Clobetasol 0.05%, Triamcinolone 0.1% | Clobetasol 0.05% | Clobetasol 0.05%, Tacrolimus 0.1%, Triamcinolone 0.1% | Betamethasone 0.05%, Clobetasol 0.05%, Pimecrolimus 1%, intralesional Triamcinolone 5 mg/mL | Clobetasol 0.05%, Tacrolimus 0.1% Triamcinolone 0.1% | Clobetasol 0.05%, Triamcinolone 0.1%, Tacrolimus 0.1% | Triamcinolone 0.1% | Pimecrolimus 1%, Tacrolimus 0.1%, Triamcinolone 0.1% |
Complete systemic treatment history | HCQ, MMF, MP, MTX | Anakinra, AZA, CTX, dapsone, HCQ, MMF, MP, MTX, QC | Apremilast, HCQ, IVIG, MMF, prednisone | Chloroquine, HCQ, IVIG, MTX, prednisone, QC, TFB‡ | BEM, HCQ, MMF, MTX, prednisone | HCQ, IVIG, MMF, prednisone | AZA, chloroquine, HCQ, MTX, prednisone, QC | CTX, HCQ, MMF, prednisone |
Systemic treatment changes at onset of overlap Disease | Continued on HCQ and MMF | Continued on HCQ and MP; Added MMF and QC | Continued HCQ; Added MMF and prednisone | Switched adalimumab to TFB; Added HCQ | Continued on HCQ, MMF and prednisone; Discontinued BEM | Continued on HCQ | Discontinued HCQ, MTX, and quinacrine; Started AZA, chloroquine, and prednisone | Continued on HCQ and MMF |
Current regimen | Triamcinolone 0.1%, HCQ 400 mg/d, MMF 2000 mg/d | Anakinra 100 mg, HCQ 400 mg/d, CTX every 2 wk, MP 32 mg/day, QC 100 mg/d | Clobetasol 0.05%, IVIG 2 g/kg monthly, MMF 3000 mg/d, prednisone 5 mg/d | Betamethasone 0.05%, Clobetasol 0.05%, Pimecrolimus 1%, IVIG 2 g/kg every 5 wk, MTX 0.6 mL/wk, TFB 11 mg/d | Clobetasol 0.05%, Tacrolimus 0.1% Triamcinolone 0.1%, HCQ 300 mg/d, MTX 20 mg/wk | Clobetasol 0.05%, Triamcinolone 0.1%, Tacrolimus 0.1%, HCQ 400 mg/d, IVIG monthly | Triamcinolone 0.1%, Chloroquine 250 mg/d for 4 d/wk, prednisone taper, MTX 20 mg/wk | Pimecrolimus 1%, Tacrolimus 0.1%, Triamcinolone 0.1%, HCQ 300 mg/d, prednisone taper, MMF 2000 mg/d |
Response to current regimen | Continues to experience arthritis and transient itchy rash on the chest and forehead | Improving facial erythema and swelling, and arthritis | Continues to experience pain in lower extremities and skin erosions | Improved muscle weakness but continues to experience erythema on the scalp/face and alopecia | Improved rashes with continued mild Gottron’s sign | Improved muscle symptoms and skin activity but continues to have mild facial erythema in the malar area and erythematous papules/plaques on the trunk | Continues to experience weakness in lower extremities and itchy rash on ears and arms | Continues to have facial erythema |
ACLE, Acute cutaneous lupus erythematosus; APLS, antiphospholipid syndrome; AZA, azathioprine; BEM, belimumab; CADM, clinically amyopathic dermatomyositis; CDM, classic dermatomyositis; CTX, cyclophosphamide; DLE, discoid lupus erythematosus; DM, dermatomyositis; HCQ, hydroxychloroquine; ILD, interstitial lung disease; IVIG, intravenous immune globulin; MMF, mycophenolate mofetil; MP, methylprednisolone; MTX, methotrexate; QC, quinacrine; RTX, rituximab; SCLE, subacute cutaneous lupus erythematosus; SLE, systemic lupus erythematosus; TFB, tofacitinib.
Adalimumab was used for treatment of rheumatoid arthritis.
Rituximab was used for treatment of brain lymphoma since 2017.
Tofacitinib replaced adalimumab in the setting of new-onset DM.