Complications

Complication
Timeframe
Likelihood
short term
medium

Can occur in up to 25% of patients with FS and are a source of major morbidity.[3][7]​​

Ulcers may indicate a cutaneous vasculitis, although histologic evidence of vasculitis is rare.

Peripheral neuropathy and long-term corticosteroid use can predispose to leg ulceration or worsen existing ulcers.

Usual management of chronic leg ulceration and use of immunosuppressants to control RA can aid ulcer healing.

long term
medium

Secondary malignancy is twice as common in patients with FS.[3][32] This may be due to chronic immunostimulatory mechanisms. 

Non-Hodgkin lymphoma

long term
medium

Secondary malignancy is twice as common in patients with FS.[3][32] This may be due to chronic immunostimulatory mechanisms.

Overview of leukemia

long term
medium

Long-term use of G-CSF in FS has been associated with worsening of arthritis and development of a leukocytoclastic vasculitis.[23]

Recommendations are to use the lowest dose of G-CSF to maintain neutrophil counts >1000/microliter.

White blood cell count should be monitored twice weekly in the first 3 weeks of treatment and weekly thereafter.[25] Long-term treatment has been reported to be safe if doses are kept as low as possible.[3][4]​​

Systemic vasculitis

variable
high

Most common complication of FS, particularly of the skin and respiratory tract.[2][3]

Neutrophil count <1000/microliter is the biggest risk factor for recurrent infections.[13] Other risk factors include neuropathy, skin ulcers, high levels of immune complexes, and corticosteroid treatment.

Common infectious organisms include Staphylococcus aureus, Streptococcus species, Haemophilus influenzae, and enteric Gram-negative organisms.[7]

While neutropenia predisposes to infection, defects in neutrophil function may contribute because infections can occur despite normalization of neutrophil count.[2]

Treatment of infections is determined by antibiotic sensitivity of the infecting organism. Immunosuppressive treatment of rheumatoid arthritis (RA) and granulocyte colony-stimulating factor (G-CSF) may be used to prevent infections.

Although antibiotics are the mainstay of therapy for infections in FS, there are no data on the role of antibiotic prophylaxis in reducing incidence of infections.[17]

variable
medium

Can occur in up to 20% of patients. Related to nodular regenerative hyperplasia of the liver.[3][33]

Increased splenic and thus portal blood flow has been proposed as the cause of portal hypertension in FS.[33]

Splenectomy has been suggested as the preferred treatment.[33]

variable
medium

Bleeding esophageal varices can occur in up to 20% of patients if they develop portal hypertension.[3][33]

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