A Clinical Study to Evaluate Ianalumab in Participants With Diffuse Cutaneous Systemic Sclerosis

Key Inclusion Criteria:

- Male and female participants >= 18 and =< 70 years (at the time of the screening
visit).

- Diagnosis of systemic sclerosis, as defined by the 2013 American College of
Rheumatology/ European League Against Rheumatism (ACR/EULAR) classification criteria
for SSc (van den Hoogen et al 2013) and meet the dcSSc subset classification
according to LeRoy (LeRoy 1988)

- Disease duration of =< 60 months (defined as time from the first non-Raynaud
phenomenon manifestation, e.g., puffy hands, scleroderma, digital ulcers,
arthralgia, dyspnea)

- mRSS units of >= 15 and =< 45 at the time of the screening visit

- Active disease that meets at least one of the following criteria at screening:

- Disease duration of =< 18 months defined as time from the first non-Raynaud
phenomenon manifestation

- Increase in mRSS of >= 3 units compared with the most recent assessment
performed within the previous 6 months

- Involvement of one new body area and an increase in mRSS of >= 2 units compared
with the most recent assessment performed within the previous 6 months

- Involvement of two new body areas within the previous 6 months

- Elevated acute phase reactants (ESR) >= 30 mm/hr or high-sensitivity C-reactive
protein (hsCRP) >= 6 mg/dL)

- Presence of interstitial lung disease (ILD) and ATA autoantibody positivity

- Modified EUSTAR disease activity index (mDAI) > 2.5

- Participant must be positive for at least one of the following autoantibodies:

- anti-topoisomerase I (ATA) (also known as anti-SCL-70)

- anti-RNA polymerase III (anti-RNAP3)

- anti-nuclear antibody (ANA) (≥ 1:80) Participants who are positive only for ANA
(while being negative for both ATA /anti-RNAP3) will be limited to 30% of the
overall randomized study population.

Key Exclusion Criteria:

- Rheumatic disease other than dcSSc, including limited cutaneous disease (lcSSc) or
sine scleroderma at the screening visit. Secondary Sjogren's disease and scleroderma
myopathy are not exclusionary.

- Positive anti-centromere antibody (ACA+) without positive ATA or anti-RNAP3
autoantibody result at the screening visit

- Previous improvement (decrease) in mRSS > 10 units

- Pulmonary disease with FVC ≤ 50% of predicted or diffusing capacity of the lung for
carbon monoxide (DLCO, corrected for hemoglobin) ≤ 40% of predicted at the screening
visit

- WHO Functional Class 3 or higher assessment for pulmonary arterial hypertension
(PAH, as defined on right heart catheterization), receiving IV therapy for PAH or
evidence of other moderately severe pulmonary disease

- Participants treated with cyclophosphamide within 12 weeks prior to Baseline.

- Prior use of a B-cell depleting therapy other than ianalumab (e.g., rituximab, other
anti-CD20 mAb, anti-CD22 mAb, or anti-CD52 mAb) administered within 36 weeks prior
to randomization, or as long as B cell count is less than the lower limit of normal
or baseline value prior to receipt of B cell-depleting therapy (whichever is lower)

- Treatment with biologic agents, such as intravenous immunoglobulin or monoclonal
antibodies, including marketed drugs, within 12 weeks or 5 half-lives (whichever is
longer) prior to baseline visit, unless explicitly allowed in inclusion criteria

- Treatment with any investigational agent within ≤ 4 weeks (or 5 half-lives of the
investigational drug, whichever is longer) of the baseline visit

- Use of anti-fibrotic agents including colchicine, D-penicillamine, pirfenidone, or
tyrosine kinase inhibitors (e.g., nintedanib, nilotinib, imatinib, dasatinib) in the
4 weeks prior to baseline visit.

- Previous treatment with chlorambucil, bone marrow transplantation or total lymphoid
irradiation.

Other protocol-defined inclusion/exclusion criteria may apply.