Table 2.

Autoimmune diseases (AID) complicating lymphoproliferative disorders.

AID	Lymphoproliferative Disorder	Frequency of AID	Key Findings	References
SLE	CLL	Up to 3%	Various case reports including central nervous system involvement and association with SS	[31]
	NHL	1%	6 SLE out of 612 diffuse large B cell lymphoma cases	[32,33]
	HL	0.02%	1 patient out of 519 HL cases developed SLE	[13]
	CD	0.03% to 1%	9 patients in a systematic review, more than a half had concomitant immune thrombocytopenia; SLE patients had no nervous system involvement	[34]
	LGL	Up to 12%	LGL correlated with > number of SLE exacerbations, cytopenias, and high doses of corticosteroids and immunosuppressors requirement	[35]
	ALL	Case reports	May develop simultaneously or several years after ALL treatment or HSCT	[36]
	MM	Case reports	Either preceding or following MM diagnosis	[37]
RA	CLL	0.4%	Very rare association. Various case reports exist	[31]
	NHL	Case reports to 4%	Oligo- and polyarthritis may occur, mainly associated with T-cell NHL	[32,33]
	HL	Case reports	RA patients with HL seem to have a worse outcome	[13]
	CD	Case reports	Active arthritis is rare in CD patients. Anti-IL6 treatment may be effective	[38]
	LGL	Up to 33%	Felty syndrome (RA, neutropenia, splenomegaly), may benefit from methotrexate (indicated for LGL)	[39]
	ALL	Case reports	Mainly pediatric cases; RA may challenge the differential diagnosis	[38]
	MM	Case reports	Very rare association	[37]
Other AID	CLL	2%	More frequently Hashimoto’s thyroiditis, vasculitis and SS; Case reports of AH, aVWS, and APS	[31]
	NHL	Up to 5%	More frequently SS, but also psoriasis, thyroiditis and Graves’ disease, polymyositis, systemic sclerosis, vasculitis, inflammatory bowel diseases, autoimmune hepatitis, and Addison’s disease. Case reports of AH and aVWS (mainly in MZL and MALT)	[13,32]
	HL	Up to 8.6%	Mainly thyroiditis and Graves’ disease, but also glomerulonephritis, DM type 1, seronegative spondylarthritis, mixed connective tissue disease, systemic sclerosis, and vasculitis. Case reports of catastrophic APS and IgA nephropathy	[13,32]
	CD	Case reports	TAFRO syndrome and concurrent SS; case reports of pemphigus vulgaris and glomerulonephritis	[34]
	LGL	Case reports	SS may complicate up to 25% of T-LGL cases	[39,40]
	ALL	Case reports	Myasthenia gravis; type 1 diabetes mellitus; IgA nephropathy; catastrophic APS	[36]
	MM	Case reports	aVWS and AH, and vasculitis	[41]

SLE systemic lupus erythematosus, RA rheumatoid arthritis, CLL chronic lymphocytic leukemia, NHL non-Hodgkin lymphoma, HL Hodgkin lymphoma, CD Castleman disease, LGL large granular lymphocyte, ALL acute lymphoblastic leukemia, MM multiple myeloma, SS Sjögren’s syndrome, HSCT hematopoietic stem cell transplant, aVWS acquired von Willebrand syndrome, AH acquired haemophilia, APS antiphospholipid syndrome, MZL marginal zone lymphoma, MALT mucosa associated lymphoid tissue, TAFRO thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly.