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Table 4 Characteristics and outcomes of patients with PNS, by types

From: Worsening and newly diagnosed paraneoplastic syndromes following anti-PD-1 or anti-PD-L1 immunotherapies, a descriptive study

Patient characteristics

Paraneoplastic hypertrophic osteoarthropathy (n = 6 pts)

Paraneoplastic encephalitis

(n = 6 pts)

Paraneoplastic dermatomyositis (n = 5 pts)

Patients with a pre-existing PNS

2

1

4

Patients with a newly diagnosed PNS

4

5

1

Clinical type

• Hypertrophic osteoarthropathy (n = 6 pts)

• Anti-Ma2 autoantibody encephalitis (n = 4 pts)

• Anti-neuron antibody encephalitis (n = 1 pt)

• Cortical myoclonus encephalitis (n = 1 pt)

• Seronegative dermatomyositis (n = 4 pts)

• Anti-TIF1-associated dermatomyositis (n = 1 pt)

Cancer type

 - NSCLC

4

3

2

 - Pulmonary sarcomatoid carcinoma

1

0

0

 - Renal carcinoma

1

1

0

 - Mesothelioma

0

1

0

 - Melanoma

0

1

2

 - Neuroendocrine carcinoma

0

0

1

Bone metastasis

1

0

1

General outcome for the PNS following anti-PD1 or PD-L1 immunotherapy

 - No worsening

1

0

1

 - Worsening

1

1

3

 - Newly diagnosis of a PNS

4

5

1

Highest CTCAE grade of PNS severity

 - Grade 1–2

4

0

0

 - Grade ≥ 3

2

6

5

Time interval between initiation of immunotherapy to worsening or new diagnosis of the PNS, median (range), months

1.4 (0.5–5)

2.6 (0.5–5.5)

0.7 (0.5–0.9)

Antitumor response at the time of worsening or newly diagnosis of a PNS

 - CR

0

0

1

 - PR

0

4

1

 - SD

5

2

0

 - PD

1

0

0

 - Not evaluated

0

0

2

Impact of paraneoplastic syndrome on immunotherapy, n

 - temporary discontinuation

3

0

1

 - permanent discontinuation

0

6

3

 - no discontinuation

3

0

1

Paraneoplastic syndrome treatment

 First-line treatment

All 5 patients received first-line treatment:

- Steroids (n = 3): complete resolution in one patient, partial resolution in two patients

- NSAIDs (n = 2): complete resolution in one patient, partial resolution in one patient

All 6 patients received first-line treatment:

- Steroids (n = 5): partial resolution in four patients, no resolution in one patient.

- Steroids plus immunoglobulins (n = 1): partial resolution in one patient three patients

All 4 patients received first-line treatment:

- Steroids (n = 2): partial resolution in two patients

- Steroids plus immunoglobulins (n = 2): partial resolution in one patient, no resolution in one patient.

 Second-line treatments (if required)

Second-line treatment was required in one patient:

- Methotrexate: partial resolution in one patient.

Second-line treatment was required in five patients:

- Immunoglobulins (n = 2) partial response in two patients

- Cyclophosphamide (n = 1): partial resolution in one patient

- Rituximab (n = 1): partial resolution in one patient

- Plasma exchange (n = 1): partial resolution in one patient

Second-line treatment was required in four patients:

- Methotrexate (n = 3): partial resolution in three patients

- Plasma exchange plus methotrexate (n = 1): partial resolution in one patient.

 Persistent of PNS symptoms with a CTCAE grade > 1 at last follow-up, n (%)

1 (20)

5 (83)

3 (75)

 PNS related death, n (%)

0

3 (50)

0

  1. CR Complete response, CTCAE Common Terminology Criteria for Adverse Events, NA Not available, NSAID Non-steroidal anti-inflammatory drug, NSCLC Non-small-cell lung carcinoma, PD Progressive disease, PNS Paraneoplastic syndrome, PR Partial response, Pt patient, SD Stable disease