Glucocorticoid-Responsive Cold Agglutinin Disease in a Patient with Rheumatoid Arthritis

Table 1

Characteristics of the patients who had CAD associated with connective tissue diseases.

Case number	Diagnosis	Age, sex	Raynaud’s phenomenon or acrocyanosis	Splenomegaly	CA titer	Monoclonal IgM	Hb (g/dL)	Treatment
1	SSc	60, F	Yes	Yes	1 : 256^†	No	6.1	Pulse + mPSL (60 mg)
2	SS	78, F	Yes	n.d.	1 : 64	n.d.	5.0	PSL (10 mg), dopamine, pyridoxal, PGE1, and systemic warming
3	SLE	55, F	n.d.	n.d.	1 : 512	n.d.	7.9	Pulse with PSL (30 mg) relapse: PSL (40 mg), DFPP, CyA, and RTX
4	SLE	27, F	No	No	1 : 7000	No	6.7	Prednisone (40 mg) and plaquenil
5	SLE	34, F	No	Yes	1 : 4096	n.d.	6.0	PSL (60 mg)
6	RA	52, F	n.d.	n.d.	1 : 512	No	9.1	PSL (1 mg/kg), CyA, and RTX
7	RA	89, F	Yes	No	1 : 320	No	31%^‡	Hydroxychloroquine
8	PMR	60, M	Yes	n.d.	1 : 256^†	Yes	8.0	Avoiding cold
Present case	RA	57, M	No	No	1 : 2048	No	6.2	PSL (1 mg/kg)

CA, cold agglutinin; CAD, cold agglutinin disease; CyA, cyclosporin; DFPP, double-filtration plasmapheresis; Hb, hemoglobin; Ht, hematocrit; mPSL, methylprednisolone; n.d., not described; PGE₁, prostaglandin E₁; PMR, polymyalgia rheumatica; PSL, prednisolone; RA, rheumatoid arthritis; RTX, rituximab; SLE, systemic lupus erythematosus; SS, Sjögren’s syndrome; SSc, systemic sclerosis. ^†The titer was measured at room temperature. ^‡Hematocrit.